Brain Studies

A diagnosis of a brain or spinal cord tumor brings uncertainty and worry to you and your friends and family.  It’s easy to become overwhelmed by a new world of tests, technology, and treatments that you may know little or nothing about.

This handbook will give you a better understanding of brain and spinal cord tumors, their treatment options, and the latest research to find safer, more effective ways to diagnose and treat them.  You can take the best care of yourself by learning about your diagnosis and discussing it with your doctors.

Brain and spinal cord tumors are found in the tissue inside the skull or the bony spinal column, which makes up the central nervous system (CNS). A tumor is a mass of cells that forms a new growth or is present at birth (congenital).  Tumors occur when genes that regulate cell growth become damaged or mutated, allowing cells to grow and divide out of control.  Tumors can form anywhere in the body.

Depending on the type, a growing tumor can kill healthy cells or disrupt their function.   It can move or press on sensitive tissue and block the flow of blood and other fluid, causing pain and inflammation.  A tumor can also block the normal flow of electricity in the brain or nerve signaling to and from the brain.  Some tumors cause no trouble at all.

There are more than 120 types of brain and spinal cord tumors.  Some are named by the type of cell in which they start (such as glioma) or location (such as meningioma, which form in the lining of the brain and spinal cord).  See the Appendix at the end of this guide for a listing of some CNS tumors and tumor-related conditions and the Glossary for specific terms and their meanings.

The following overview explains how the CNS works and what happens when a tumor is present.

The brain has three major parts:

• brain stem—This lowest part of the brain (above the neck) connects to the spinal cord and relays information between the brain and the body using bundles of long nerves.  It controls basic life-sustaining functions, including blood pressure, heartbeat, breathing, consciousness, swallowing, and body temperature.
• cerebrum—This largest and outermost part of the brain processes information from our senses to tell the body how to respond.  It controls functions including movement, touch, judgment, learning, speech, emotions, and thinking.
• cerebellum—Located at the lower rear of the brain, above the brain stem, the cerebellum controls balance, helps maintain equilibrium, and coordinates such complex muscle movements as walking and talking.

The brain’s two halves, or hemispheres, use nerve cells (neurons) to speak with each other.  Each side of the cerebrum controls movement and function on the other side of the body.  In addition, each hemisphere has four sections, called lobes, which handle different neurological functions.

The frontal lobes manage voluntary movement, such as writing, and let us set and prioritize goals.  A frontal lobe tumor can cause changes in personality, intellect, reasoning, and behavior; affect coordination and walking, and cause speech loss.  The temporal lobes are linked to perception, memory, and understanding sounds and words.  A tumor here might cause speech and hearing problems, blackouts, seizures, or sensations such as a feeling of fear.  The parietal lobes let us simultaneously receive and understand sensations such as pressure and pain.  A parietal lobe tumor might cause difficulty understanding or speaking words, problems with coordination, seizures, and numbness or weakness on one side of the body.  The occipital lobes receive and process light and visual images, and detect motion.  An occipital lobe tumor can affect the field of vision, usually on one side of the face, and how we understand written words.

Three layers of protective tissue (called the meninges) cover the brain—the thick dura mater (outer layer), the arachnoid (middle), and the pia mater (innermost to the brain).

Brain tumors in infants and adults tend to be located in the cerebrum.  Brain tumors in children ages 1-12 years are more commonly found in the cerebellum.

The spinal cord—an extension of the brain—lies protected inside the bony spinal column.  It contains bundles of nerves that carry messages between the brain and other parts of the body, such as instructions from the brain to move an arm or information from the skin that signals pain.

A tumor that forms on or near the spinal cord can disrupt communication between the brain and the nerves or restrict the cord's supply of blood.  Because the spinal column is narrow, a tumor here—unlike a brain tumor—can cause symptoms on both sides of the body at the same time.

Most spinal cord tumors form below the neck.   Symptoms generally strike body areas at the same level or at a level below that of the tumor.  For example, a tumor midway along the spinal cord (in the thoracic spine) can cause pain that spreads over the chest and gets worse when the individual coughs, sneezes, or lies down.  A tumor that grows in the cervical spine can cause pain that seems to come from the neck or arms, and a tumor that grows in the lower, lumbar spine can trigger back or leg pain.

The three major groups of spinal cord tumor describe where they are found.  Extradural tumors grow between the inner surface of the spinal canal and the tough dura mater.  Tumors inside the dura (intradural tumors) are further divided into those outside the spinal cord (extramedullary tumors) and those inside the spinal cord (intramedullary tumors).  Other descriptors for spinal cord tumors are intrinsic, meaning the tumor forms inside the spinal cord; and extrinsic, where the tumor forms outside of and presses on the cord as it grows.

No matter where they are located in the body, tumors are classified as benign or malignant.

Benign tumors are slow growing, non-cancerous cell masses that have a defined edge and do not spread to other parts of the body.  Cells in the tumor are similar to normal cells.  Often these tumors can be removed surgically and usually do not recur.

Malignant, or cancerous, tumors have cells that look different from normal cells.  They can quickly invade surrounding tissue and often have edges that are hard to define, which makes it difficult to remove the entire tumor surgically.

Primary tumors of the CNS are growths that begin in the brain or spinal cord.   They can be either malignant or benign and are identified by the types of cells they contain, their location, or both.  Most primary CNS tumors occur in adults.

Metastatic, or secondary, tumors in the CNS are caused by cancer cells that break away from the primary tumor that developed in a non-CNS part of the body.  These tumors are named after the type of cancer that causes them.   Metastastic tumors (also called metastases) to the brain occur in about one-fourth of all cancers that develop in other parts of the body, such as cancer of the lung, breast, or kidneys; or melanoma, a form of skin cancer.  They are more common than primary tumors and occur more often in adults than in children.

Metastatic spine tumors usually form within the bony covering of the spinal column but may also invade the spinal canal from the chest or abdomen.

While cancers elsewhere in the body can easily cause tumors inside the brain and spinal cord, CNS tumors rarely spread outside the nervous system.

Researchers really don't know why primary brain and spinal cord tumors develop.  Possible causes under investigation include viruses, defective genes, exposure to certain chemicals and other hazardous materials, and immune system disorders.  Although smoking, alcohol consumption, and certain dietary habits are associated with some types of cancers, they have not been linked to primary CNS tumors.

In a small number of individuals, CNS tumors may result from specific genetic diseases, such as neurofibromatosis and tuberous sclerosis, or exposure to radiation. Non-ionizing radiation (radio waves) from mobile phone use does not increase the risk of developing a brain tumor.¹

Brain and spinal cord tumors are not contagious or, at this time, preventable.

Anyone can develop a primary CNS tumor, although the risk is very small.  Having one or more of the known risk factors does not guarantee that someone will develop a tumor.  Brain tumors occur more often in males than in females and are most common in middle-aged to older persons.  They also tend to occur more often in children under age 9 than in other children, and some tumors tend to run in families.  Most brain tumors in children are primary tumors.

Other risk factors for developing a primary CNS tumor include race (Caucasians are more likely to develop a CNS tumor than other races) and occupation.  Workers in jobs that require repeated contact with ionizing radiation or certain chemicals, including those used to manufacture building supplies or plastics and textiles, have a greater chance of developing a brain tumor.

More than 359,000 persons in the United States are estimated to be living with a diagnosis of primary brain or central nervous system tumor.²    More than 195,000 Americans are diagnosed with a brain tumor each year.³  Brain tumors are the most common form of solid tumor in children.

Spinal cord tumors are less common than brain tumors.  Although they affect people of all ages, spinal cord tumors are most common in young and middle-aged adults.  Nearly 3,200 central nervous system tumors are diagnosed each year in children under age 20.⁴

The generally accepted scale for grading CNS tumors was approved by the World Health Organization in 1993.  Grading is based on the tumor’s cellular makeup and location.  Tumors may also be classified as low-grade (slowly growing) or high-grade (rapidly growing).  Some tumors change grades as they progress, usually to a higher grade, and can become a different type of tumor.  The tumor is graded by a pathologist following a biopsy or during surgery.

Grade I tumors grow slowly and generally do not spread to other parts of the brain.  It is often possible to surgically remove an entire grade I benign tumor, but this type of tumor may be monitored periodically, without further treatment.

Grade II tumors also grow slowly, sometimes into surrounding tissue, and can become a higher-grade tumor.  Treatment varies according to tumor location and may require chemotherapy, radiation, or surgery followed by close observation.

Grade III tumors are malignant and can spread quickly into other CNS tissue.  Tumor cells will look different than those in surrounding tissue.  Aggressive treatment, often using a combination of chemotherapy, radiation, and/or surgery, is required.

Grade IV tumors invade nearby tissue very quickly and are difficult to treat.  The cancerous tissue will look very different from surrounding tissue.  Aggressive treatment is required.

Brain and spinal cord tumors cause many diverse symptoms, which can make detection tricky.  Symptoms depend on tumor type, location, size, and rate of growth.  Certain symptoms are quite specific because they result from damage to particular areas of the brain.  Symptoms generally develop slowly and worsen as the tumor grows.

The most obvious sign of a brain tumor in infants is a rapidly widening head or bulging crown.

Common symptoms of a brain tumor in adults include headaches, seizures, problems with balance or coordination, loss of muscle control, hydrocephalus, and changes in personality or behavior.

Headaches are the most common symptom of a brain tumor.  Headaches may progressively worsen, become more frequent or constant, and recur, often at irregular intervals.  Headache pain may worsen when coughing, changing posture, or straining and may be severe upon waking.

Seizures can occur, with symptoms that may include convulsions, loss of consciousness, or loss of bladder control.  Seizures that first start in adulthood (in someone who has not been in an accident or who had an illness that causes seizures) are a key warning sign of brain tumors.

Nausea and vomiting may be more severe in the morning and may accompany headaches.

Vision or hearing problems can include blurred or double vision, partial or total loss of vision or hearing, ringing or buzzing sounds, and abnormal eye movements.

Personality, behavior, and cognitive changes can include psychotic episodes and problems with speech, language, thinking, and memory.

Motor problems can include weakness or paralysis, lack of coordination, or gradual loss of sensation or movement in an arm or leg.  A sudden, marked change in handwriting may be a sign of a tumor.

Balance problems can include dizziness, trouble with walking, clumsiness, or loss of the normal control of equilibrium.

Hydrocephalus and increased intracranial pressure are caused when a tumor blocks the flow of the cerebrospinal fluid (CSF) that surrounds the brain and spinal cord.  Symptoms may include headaches, nausea, and vomiting.

Other symptoms may include endocrine disorders or abnormal hormonal regulation, difficulty swallowing, facial paralysis and sagging eyelid, fatigue, weakened sense of smell, or disrupted sleep and sleep pattern changes.

Common symptoms of a spinal cord tumor include pain, numbness or sensory changes, and motor problems and loss of muscle control.

Pain can feel as if it is coming from various parts of the body.  Back pain may extend to the hips, legs, feet, and arms.  This pain is often constant and may be severe.  It is often progressive and can have a burning or aching quality.

Numbness or sensory changes can include decreased skin sensitivity to temperature and progressive numbness or a loss of sensation, particularly in the legs.

Motor problems and loss of muscle control can include muscle weakness, spasticity (in which the muscles stay stiffly contracted), and impaired bladder and/or bowel control.  If untreated, symptoms may worsen to include muscle wasting, decreased muscle strength, an abnormal walking rhythm known as ataxia, and paralysis.

Symptoms may spread over various parts of the body when one or more tumors extend over several sections of the spinal cord.

A doctor, usually a neurologist, oncologist, or neuro-oncologist, can confirm a diagnosis of a brain or spinal cord tumor based on a patient’s symptoms, personal and family medical history, and results of a physical exam and specialized tests and techniques.

A neurological exam—the first test—assesses movement and sensory skills, hearing and speech, reflexes, vision, coordination and balance, mental status, and changes in mood or behavior, among other abilities.   Some tests require a specialist to perform and analyze results.

Diagnostic Imaging

The doctor will order one or more imaging techniques that show extremely detailed views of body structures, including tissues, organs, bones, and nerves.  If there is a tumor, diagnostic imaging will confirm the diagnosis and help doctors determine the tumor's type, detect swelling and other associated conditions, and, over time, check the results of treatment.

See the NINDS publication, “Neurological Diagnostic Tests and Procedures,” for a more complete description of the following tests:

Computed Tomography (CT) uses x-rays and a computer to produce fast, detailed cross-sectional images or “slices” of organs, bones, and tissues, including a tumor.  It is also good for detecting the buildup of calcium, which causes tissue to harden and can develop into a tumor.

Magnetic Resonance Imaging (MRI) uses a computer, radio waves, and a strong magnetic field to produce two-dimensional slices or a detailed three-dimensional model of tissue being scanned.  MRI takes longer to perform than does a CT but is more sensitive and gives better pictures of tumors located near bone. 

Both CT and MRI scans for tumor are usually performed before and after administration of a "contrast" agent (such as a dye) that is given into a vein.  Many tumors become much brighter on the scan taken after the contrast is given.

Functional MRI (fMRI) creates images of areas of the brain with specific functions such as movement and language.   It can assess brain damage from head injury or degenerative disorders, identify and monitor other neurological disorders such as stroke, and show the distance between specific brain functions and tumors in particular areas of the brain.

Magnetic Resonance Spectroscopy (MRS) gives doctors a chemical snapshot of tissues being studied.  It uses the MRI scanner's magnetic field and radio waves to measure and analyze the chemical make-up of the tissue sample.

Positron Emission Tomography (PET) provides computer-generated two- and three-dimensional scans of the brain’s chemical activity and cellular function.  PET traces and measures the brain’s use of glucose (sugar, used by the brain for energy) that is attached to small amounts of radioactivity and injected into the bloodstream.  Since malignant tissue uses more glucose than normal tissue, it usually shows up on the scan as brighter than surrounding tissue.  

Single Photon Emission Computed Tomography (SPECT) studies blood flow to tissue.  Certain tumors grow new blood vessels to increase their supply of blood and nutrients.  A radioactive isotope is injected intravenously and traced as it travels into the skull.  A sophisticated computer processes and stacks the data into a detailed three-dimensional image of activity within the brain.

Angiography (or arteriogram) can distinguish certain types of tumors that have a characteristic pattern of blood vessels and blood flow.  A dye that deflects x-rays is injected into a major blood vessel and a series of x-rays is taken as the dye flows to the brain.  In many situations angiography has been replaced by non-invasive tests such as CT and MRI.

Laboratory Tests

Testing blood, urine, and other substances can provide clues about the tumor and monitor levels of therapeutic drugs.

Additional tests may include an electroencephalogram, or EEG, which monitors brain activity through the skull (tumors can disrupt the normal flow of brain wave activity and cause seizures); CSF analysis, in which a small amount of the cerebrospinal fluid is removed by a special needle and examined for abnormal cells or unusual levels of various compounds that suggest a brain or spinal cord tumor; and magnetoencephalography (MEG), which studies brain function by measuring the magnetic field generated by nerve cells in the brain.   CSF fluid analysis should be performed with extreme caution on individuals with very large brain tumors.

Diagnosing the distinct type of brain tumor is often difficult.  Individuals should consider asking their primary care physician or oncologist for a second opinion, particularly from a neuro-oncologist or neurosurgeon, as there may be new information available and some tumors can change grade or recur.  Even a second opinion that confirms the original diagnosis can help people better prepare for their care and treatment.

A specialized team of doctors advises and assists individuals throughout treatment and rehabilitation.  These doctors may include:

A neurologist is a specialist in nervous system disorders.

A medical oncologist is a specialist in cancer.

A neuro-oncologist is a neurologist who specializes in nervous system tumors.

A neuroradiologist is a doctor trained in reading diagnostic imaging results who specializes in the CNS.

A pathologist is a clinical physician who diagnoses diseases of tissues or cells using a variety of laboratory tests.

A neurosurgeon is a brain or spinal cord surgeon.

A radiation oncologist is a doctor who specializes in using radiation to treat individuals with cancer.

This team will recommend a treatment plan based on the tumor's location, type, size and aggressiveness, as well as on the individual’s medical history, age, and general health.

Initial treatment for a CNS tumor may involve a variety of drugs, including anticonvulsants to treat seizures, pain medications, steroids or other anti-inflammatory drugs to reduce swelling and improve blood flow, antidepressants to treat anxiety or ease depression that might occur following a tumor diagnosis, and drugs to fight nausea caused by various treatments.

Malignant tumors require some form of treatment, while some small benign tumors may need only periodic monitoring.  The three standard treatment options for malignant CNS tumors are neurosurgery, radiation therapy, and chemotherapy.  Some patients may receive a combination of treatments.

Surgery is usually the first step in treating an accessible tumor—one that can be removed without unacceptable risk of neurological damage.  Surgery is aimed at removing all or as much tumor as possible (called resecting or excising) and can often slow worsening of neurological function.

An inaccessible or inoperable tumor is one that cannot be removed surgically because of the risk of severe nervous system damage during the operation.  These tumors are frequently located deep within the brain or near vital structures such as the brain stem.

A biopsy is usually performed to help doctors determine how to treat a tumor.  A brain biopsy involves surgically removing a small part of the skull to sample the tumor tissue.  Biopsies can sometimes be performed by a needle inserted through a small hole.  A small piece of tissue remains in the hollow needle when it is removed from the body.  A pathologist will stain and examine the tissue for certain changes that signal cancer and grade it to reflect the degree of malignancy.

If the sample is cancerous, the surgeons will remove as much of the tumor as possible.  For some primary brain tumors it is not possible to surgically remove all malignant cells.  Malignant brain tumors commonly recur from cells that have spread from the original tumor mass into the surrounding brain tissue.  In contrast, many benign tumors and secondary metastatic tumors can be completely removed surgically.

In some cases, a surgeon may need to insert a shunt into the skull to drain any dangerous buildup of CSF caused by the tumor.  A shunt is a flexible plastic tube that is used to divert the flow of CSF from the central nervous system to another part of the body, where it can be absorbed as part of the normal circulatory process. 

Fortunately, research has led to advances in neurosurgery that make it possible for doctors to completely remove many tumors that were previously thought to be inoperable.  These new techniques and tools let neurosurgeons operate within the tight, vulnerable confines of the CNS.  Some tools used in the operating room include a surgical microscope, the endoscope (a small viewing tube attached to a video camera), and miniature precision instruments that allow surgery to be performed through a small incision in the brain or spine.

Intraoperative MRI uses a special type of MRI to provide a real-time evaluation of the surgery.   Constantly updated images that are provided during surgery let doctors see how much tumor material has been removed.  Intraoperative MRI can also help doctors choose the best surgical approaches and monitor any complications during surgery.

Navigation equipment used in computer-guided, or stereotactic, neurosurgery gives doctors a precise, three-dimensional map of an individual’s spine or brain as the operation progresses.  A computer uses pre-operative diagnostic images of the individual to reduce the risk of damage to surrounding tissue.

Intraoperative nerve monitoring tests such as evoked potentials use real-time recordings of nerve cell activity to determine the role of specific nerves and monitor brain activity as the surgery progresses.  Small electrodes are used to stimulate a nerve and measure its electrical response (or evoked potential).  Some surgeries may be done while the individual is awake under monitored anesthesia care, rather than under general anesthesia.  This allows doctors to monitor the individual’s speech and motor functions as a tumor is being removed.

A possible side effect of surgery is swelling around the site of the tumor, and can be treated with steroids.  Bleeding into the tumor site or infection are other serious risks of brain surgery.

In the cae of metastatic tumors, doctors usually treat the original cancer.  However, if there are only one or two metastases to the brain or if a metastatic tumor causes serious disability or pain, doctors may recommend surgery—even if the original cancer has not been controlled.

Surgery may be the beginning and end of your treatment if the biopsy shows a benign tumor.  If the tumor is malignant, doctors often recommend additional treatment, including radiation and chemotherapy, or one of several experimental treatments.

Radiation therapy usually involves repeated doses of x-rays or other forms of radiation to kill cancer cells or keep them from multiplying.  When successful, this therapy shrinks the tumor mass but does not actually remove it.  Radiation therapy can be used to treat surgically inaccessible tumors or tumor cells that may remain following surgery.

Depending on tumor type and stage, radiation treatment might be delivered externally, using focused beams of energy or charged particles that are directed at the tumor, or internally, using a surgically implanted device.  The stronger the radiation, the deeper it can penetrate to the target site.  Healthy cells may also be damaged by radiation therapy but most are able to repair themselves, while the damaged tumor cells cannot.

A radiation oncologist will explain the therapy and how much radiation is needed.  Treatment often begins a week after surgery and may continue for several weeks.  Depending on tumor type and location, individuals may be able to receive a modified form of therapy to lessen damage to healthy cells and improve the overall treatment.

Externally-delivered radiation therapy poses no risk of radioactivity to the patient or family and friends.  Types of external radiation therapy include:

Whole brain radiation is generally used to shrink multiple cancerous tumors, rather than to target individual tumors.  It may be given as the sole form of treatment or in advance of other forms of radiation therapy and microsurgery.  Whole brain radiation is generally used for metastatic tumors and rarely for primary tumors.

Conventional external beam radiation aims a uniform dose of high-energy radiation at the tumor and surrounding tissue from outside the body.  It is used to treat large tumors or those that may have spread into surrounding tissue.

Three-dimensional conformal radiotherapy (3D-CRT) uses diagnostic imaging to prepare an accurate, computer-generated three-dimensional image of the tumor and surrounding tissue.  The computer then coordinates and sends multiple beams of radiation to the tumor’s exact shape, sparing nearby organs and surrounding tissue.

Intensity modulated radiation therapy (IMRT) is similar to 3D-CRT but varies the intensity of the hundreds of individual radiation beams to deliver more precise doses to the tumor or to specific areas within it.  IRMT can provide a highly effective dose of radiation to the tumor, with less exposure to surrounding tissue.

Hyperfractionation involves giving two or more smaller amounts of radiation a day instead of a larger, single dose.  It can deliver more radiation to certain tumors and reduce damage to normal cells.

Radiosurgery is usually a one-time treatment involving a large amount of sharply focused radiation that is aimed at the brain tumor.  Stereotactic radiosurgery uses computer imaging to direct precisely focused radiation into the tumor from multiple angles.  It does not actually cut into the person but, like other forms of radiation therapy, harms a tumor cell’s ability to grow and divide.  Stereotactic radiosurgery is commonly used to treat surgically inaccessible tumors.  It also may be used at the end of conventional radiation treatment.  Two common stereotactic radiosurgery procedures are:

• Linear-accelerated radiosurgery (LINAC) uses radar-like technology to prepare and fire a single beam of high-energy x-rays into the tumor.  Also called high linear-energy transfer radiation, LINAC forms the beam to match the tumor’s shape, avoiding surrounding tissue.  A special machine that rotates around the head then fires a uniform dose of radiation into the tumor.
• Gamma knife® radiosurgery focuses more than 200 beams of gamma radiation into one intersecting beam that is fired into the tumor.  It also uses computer imaging to prepare a model of the tumor.  This single treatment takes between one and four hours and is often recommended for inaccessible or hard to treat tumors.

Both procedures may be given on an outpatient basis but an overnight stay in the hospital is often recommended.

Proton beam therapy directs a beam of high-energy protons directly at the tumor site, leaving surrounding healthy tissue and organs intact.  It is best used to treat tumors that are solid and have not spread to other parts of the body.  Proton beam therapy can be used as a stand-alone treatment or in combination with chemotherapy or as follow-up to surgery.

Internal tumor radiation therapy, also called brachytherapy or interstitial radiation therapy, involves placing a small amount of radioactive material into or near the tumor (or its cavity, if the tumor has been surgically removed).  In most cases, the radiation is inserted at the time of surgery or using imaging and a catheter.  The radiation may be left in place for several days and, if more than one treatment is needed, the doctor may leave the catheter in place for a longer period.

Individuals may need to be hospitalized for a few days following this procedure as the radiation may extend outside their body and could possibly harm others.  The radiation becomes less active each day until it is safe for individuals who have been treated to be around others.

Side effects of radiation therapy vary from person to person and are usually temporary.  They typically begin about two weeks after treatment starts and may include fatigue, nausea, vomiting, reddened or sore skin in the area receiving treatment, headache, hearing loss, problems with sleep, and hair loss (although the hair usually grows back once the treatment has stopped).  Radiation therapy in young children, particularly those children age three or younger, can cause problems with learning, processing information, thinking, and growing.

Chemotherapy uses powerful drugs to kill cancer cells or stop them from growing or dividing.  These drugs are usually given by pill or injection and travel through the body to the brain, or can be inserted surgically using dissolvable wafers that have been soaked in a chemotherapeutic drug.   These wafers slowly release a high concentrate of the drug to kill any remaining malignant cells.  Chemotherapy may also be given to kill cancer cells in the spinal column.

Chemotherapy is given in cycles to more effectively harm cancer cells and give normal cells time to recover from any damage.  The oncologist will base the treatment on the type of cancer, drug(s) to be used, the frequency of administration, and the number of cycles needed.

Individuals might receive chemotherapy to shrink the tumor before surgery (called neo-adjuvant therapy), in combination with radiation therapy, or after radiation treatment (called adjuvant therapy) to destroy any remaining cancer cells.  Metronomic therapy involves giving continuous low-dose chemotherapy to block mechanisms that stimulate the growth of new blood vessels needed to feed the tumor.  Chemotherapy is also used to treat CNS lymphoma and inaccessible tumors or tumors that do not respond to radiation therapy.

Not all tumors are vulnerable to the same anticancer drugs, so a person’s treatment may include a combination of drugs.  Common CNS chemotherapeutics include temozolomide, carmustine (also called BCNU), lomustine, tamoxifen, carboplatin, methotrexate, procarbazine, and vincristine.  Individuals should be sure to discuss all options with their medical team.

Side effects of chemotherapy may include hair loss, nausea, digestive problems, reduced bone marrow production, and fatigue.  The treatment can also harm normal cells that are growing or dividing at the same time, but these cells usually recover and problems stop once the treatment has ended.

Alternative and complementary approaches may help tumor patients better cope with their diagnosis and treatment.  Some of these therapies, however, may be harmful if used during or after cancer treatment and should be discussed with a doctor beforehand.  Common approaches include nutritional and herbal supplements, vitamins, special diet, and mental or physical techniques to reduce stress.

Each person is different.  Prognosis depends greatly on prompt diagnosis and treatment, the individual’s age and general health, whether the tumor is malignant or benign, tumor size and location, tumor grade, and response to therapy.  An individual whose entire tumor has been removed successfully may recover completely.  Generally, prognosis is poorer in very young children and in older individuals.  Rehabilitation and counseling can help patients and family members better cope with the disorder and improve quality of life.

Continued monitoring and long-term follow-up is advised as many tumors resist treatment and tend to recur.

Normal tissue and nerves that may have been damaged or traumatized by the tumor or its treatment will need time to heal.  Some post-treatment symptoms will disappear over time.  Physical therapy can help people regain motor skills, muscle strength, and balance.  Some individuals may need to relearn how to swallow or speak if the brain’s cognitive areas have been affected.  Occupational therapy can teach people new ways to perform tasks.   Supportive care can help people manage any pain and other symptoms.

Scientists continue to investigate ways to better understand, diagnose, and treat CNS tumors.  Several of today’s treatment regimens were experimental therapies only a decade ago.  Current clinical studies of genetic risk factors, environmental causes, and molecular mechanisms of cancers may translate into tomorrow’s treatment of, or perhaps cure for, these tumors.

Much of this work is supported by the National Institutes of Health (NIH), through the collaborative efforts of its National Institute of Neurological Disorders and Stroke (NINDS) and National Cancer Institute (NCI), as well as other agencies within the Federal government, nonprofit groups, pharmaceutical companies, and private institutions.   Some of this research is conducted through the collaborative neuroscience and cancer research community at the NIH or through research grants to academic centers throughout the United States .

The jointly sponsored NCI-NINDS Neuro-Oncology Branch coordinates research to develop and test the effectiveness and safety of novel therapies for people with CNS tumors.  These experimental treatment options may include new drugs, combination therapy, gene therapy, biologic immuno-agents, surgery, and radiation.  Information about these trials, and trials involving other disorders, can be accessed at the Federal government’s database of clinical trials, http://clinicaltrials.gov.

Part of this research involves creating a comprehensive public database of clinical, molecular, and genetic information on brain tumors to help researchers and clinicians identify and evaluate molecular targets in brain cancer.   The joint NCI-NINDS Repository for Molecular Brain Neoplasia Data (REMBRANDT) will hold genetic and molecular analysis of samples from brain tumor patients in NCI-sponsored clinical trials in what is slated to become the largest clinical/genetic corollary study ever conducted on brain tumors.   It will also store a wide array of molecular and genetic data regarding all types of brain tumors.   Understanding the biology behind these tumors will provide clues to new therapeutic approaches.

Researchers are also developing mouse models that mimic human CNS cancers, which may speed discovery of potential treatments in humans. 

Beyond their efforts to develop needed resources, scientists at the NIH and at universities across the US are are exploring a variety of approaches to treat CNS tumors.  These experimental approaches include boosting the immune system to better fight tumor cells, developing therapies that target the tumor cell while sparing normal cells, making improvements in radiation therapy, disabling the tumor using genes attached to viruses, and defining biomarkers that may predict the response of a CNS tumor to a particular treatment.

Biological therapy involves enhancing the body’s overall immune response to recognize and fight cancer cells.  The immune system is designed to attack foreign substances in the body; since cancer cells aren’t foreign, they usually do not generate much of an immune response.  Researchers are using different methods to provoke a strong immune response to tumor cells.  Such proteins as interleukin and interferon, both of which are immune system “messengers” called cytokines, and other substances can stimulate and restore the body’s natural response against proteins on the surfaces of cancer cells and thus slow tumor growth.  Other therapy uses viruses, T-cells (a major component of immune system function), and other substances to increase immune response and target the tumor cells.  Scientists are also experimenting with genetically altered T-cells from the patient’s own body, which are cultured with an antigen and injected directly into the brain following surgery.  Biological therapies to fight CNS tumors include vaccines, gene therapy, antibody therapy, and tumor growth factors.

Antibodies are proteins that are normally produced by the body to ward off bacteria and viruses.   Monoclonal antibodies are multiple copies of a single antibody that act as a homing device to fit one—and only one—type of antigen (a protein on the surface of the tumor cell that stimulates the immune system response).  Scientists at the NINDS and elsewhere are linking these antibodies to immunotoxins that seek out tumor cells with a matching antigen, bind to these tumor cells, and deliver their toxin, with minimal damage to surrounding normal cells.  Additional approaches include attaching a radioactive substance to the antibodies, which act as targeting agents to deliver radiation to the tumor cell.

Gene therapy (also called gene transfer) aims to deliver a suicide gene to the tumor cell.  It can also be used to boost the immune system.  A gene whose activity can be influenced to kill the cell is integrated into a virus that can cross the blood-brain barrier (an elaborate network of fine blood vessels and cells that filters the blood reaching the CNS) and travel to the tumor.  Delivery methods under investigation include viruses and stem cells.  Gene therapy may become an important add-on therapy for individuals who do not respond well to other treatments.

Scientists are testing the effectiveness of vaccine therapy for a variety of CNS tumors.  Vaccine therapy strengthens the immune response by inserting an antigen that the body will attack.  Some vaccines target a specific antigen, while others, using the whole tumor cell to make the vaccine, hope to target multiple antigens which the tumor may express.  Research has shown the vaccine can be genetically engineered with tumor antigens and injected into the body to induce an immune response, which increases the attack on antigens expressed on the surface of the tumor cell.  Experimental CNS tumor vaccines include those designed to stimulate an immune response to a particular protein or antigen, and dendritic cell vaccines (in which blood cells are taken from the body, processed in a laboratory and given back to the patient to break up the tumor cell outer protein into smaller pieces, which increases targeting by immune cells).  

Findings from NIH-sponsored research on adult CNS tumor development and treatment suggest the immune system and, potentially, infection with the cytomegalovirus may play a critical role in certain tumor-type risk and prognosis.  Repeated stimulation of the immune system to counter this herpes virus may serve as a tumor promotion factor.  Scientists hope to identify immune factors and develop targeted immunotherapies for this disease.

Targeted therapy uses different molecules to reduce tumor gene expression and suppress uncontrolled growth by killing or reducing the production of tumor cells.  Of particular interest to scientists is the development of tailored therapeutics—involving a combination of targeted agents—to treat tumors based on tehir genetic make-up.  Specifically, molecularly targeted drugs seek out the molecular and cellular changes that convert normal cells into cancer.  Many targeted cancer therapies are being tested in animals for use alone or in combination with other cancer treatments, such as chemotherapy.  Researchers hope to discover tumor-specific cellular or molecular pathways that can be used to increase drug delivery and to find ways to identify patients who may benefit from combined therapy.  Targeted therapies include oncogenes, growth factors, and molecules aimed at blocking gene activity.

Growth factors often govern normal cell growth.  Growth factors are released from either the cell or the surrounding tissue.  Cancer cells can either secrete these growth factors or respond to them, enabling   them to divide out of control.  A number of tumor growth factors have been identified, including those that trigger nerve tissue growth and stimulate blood vessels to grow (a process called angiogenesis).  Researchers continue to identify and develop agents that can block these factors. 

Anti-angiogenetic compounds block blood vessel growth and the flow of nutrients and oxygen to the tumor, and may also hamper cell signaling and stop tumor cells from spreading elsewhere in the body.  Clinical trials have shown that anti-angiogenetics can improve the outcome for glioblastoma multiforme tumors that recur following initial treatment with radiation and chemotherapy.  More than a dozen such compounds have been identified, including bevacizumab, interferon, and endostatin.  Researchers are studying the combination of anti-angiogenics and radiation or chemotherapy in treating newly diagnosed brain tumors.  Other research is testing angiogenic inhibitors that have had positive results in adults on children with primary CNS tumors and in patients with tumors that haven't responded well to other tumor treatment.

Oncogenes are transformed genes that are involved in cell growth and cause normal cells to divide uncontrolled and become malignant, either through mutation or over-expression.  Researchers have identified specific events that lead to this mutation and are developing diagnostic and therapeutic screening tools. 

Certain enzymes that are involved in cell division or the copying of genetic information may be over-produced in cancer cells, causing gene mutations and uncontrolled cell growth.  Several different types of kinase inhibitors—proteins that block growth-signaling enzymes without harming normal cells—have been identified and approved for cancer treatment.  Scientists are testing protein-level kinase inhibitors to see of they make CNS tumors more sensitive to chemotherapy.  Clinical trials for brain tumors are studying the safety and effectiveness of kinase inhibitors in children with CNS tumors and in other patients with recurring or hard to treat tumors.

Anti-sense oligonucleotide technology uses short fragments of nucleic acid molecules to block gene expression in specific cells and prevent tumor growth.  Unlike gene therapy, anti-sense oligonucleotide technology does not replace or substitute genetic material but inhibits the expression of select targets.  This technology (also known as RNA interference, siRNA, or shRNA) may help scientists to identify additional genes involved in tumor formation and improve drug delivery with fewer side effects.

Of particular interest are microRNAs (or miRNAs)—naturally occurring molecules that regulate gene expression and are involved in the formation and development of tumor stem cells.  Researchers are using miRNAs to switch off tumor stem cell activity.   Researchers are studying miRNAs as a possible diagnostic and therapeutic strategy for brain tumors and other forms of cancer.

Biomarkers are molecules or others substances in the blood or tissue that can be used to diagnose or monitor a particular disorder, among other functions.  As cells become cancerous, they can release unique proteins and other molecules into the body which scientists use to speed diagnosis and treatment.  Some CNS tumor biomarkers have been found, such as the epithelial growth factor receptor (EGRF) gene.  Researchers continue to search for additional clinical biomarkers of CNS tumors, to better assess risk from environmental toxins and other possible causes, and monitor and predict the outcome of CNS tumor treatment.  NINDS-funded research has recently shown that mutation analysis of EGFR can be used to predict which tumors are most likely to respond to a specific class of cancer-fighting drugs.   Identifying biomarkers may also lead to the development of new models of disease and novel therapies for tumor treatment.

Radiation therapy research incluces testing several new anticancer drugs, either independently or in combination with other drugs.  Researchers are also testing different drugs in combination with other therapies and are investigating combined therapies such as radiation and radiosurgery to effectively treat CNS tumors.  Research areas being explored include tomotherapy, boron nuclear capture therapy, liquid radiation therapy, and the use of radiosensitizers.

Radiosensitizers are drugs that make rapidly dividing tumor tissue more vulnerable to radiation.  Early studies using radiosensitizers produced mixed results.  While some trials suggested these drugs may improve survival in certain patients, other trials were found to have little benefit.  Scientists are now testing an experimental drug that increases the amount of oxygen that is released from the blood into the tissues, making cancer cells more responsive to radiation and chemotherapy.  Studies show the lack of oxygen in a tumor can make radiation therapy less effective.

Tomotherapy combines CT scanning and intensity modulated radiation therapy to deliver small beams of high-dose radiation to the tumor while greatly reducing radiation exposure to surrounding tissue.  Studies are examining the effectiveness of radiation delivery through tomotherapy and to see if long-term side effects of radiation therapy can be reduced.

Boron Neutron Capture Therapy (BNCT) is an experimental treatment that uses fission to kill tumor cells.  An amino acid or other drug that carries boron is injected into the patient, where it collects more readily in tumor cells than in regular tissue.  A beam of low-energy neutrons is directed at the tumor from outside the body, causing the boron atoms to split (called neutron capture) and send high amounts of energy into the cancerous cells.  Radiation damage to surrounding cells is extremely small.  BNCT is being tested as a post-surgery treatment for patients with certain head and neck tumors.  If successful, BNCT could someday be used to treat children with brain tumors.

Liquid radiation therapy is an experimental treatment that uses a balloon catheter to deliver internal radiation therapy to the cavity that remains following tumor surgery.  A radioactive liquid is injected into the catheter and molds the balloon to the exact edges of the cavity.  The liquid stays in the balloon for several days until it and the catheter are removed.

Chemotherapeutic drug research focuses on ways to better deliver drugs across the blood-brain barrier and into the site of the tumor.  Since chemotherapeutic drugs work in different ways to stop tumor cells from dividing, several trials are testing whether giving more than one drug, and perhaps giving them in different ways (such as staggered delivery and low-dose, long-term treatment), may kill more tumor cells without causing or lessening damaging side effects than present therapy.  Researchers are examining different levels of chemotherapeutic drugs to determine if they are less toxic to normal tissue when combined with other cancer treatments.  Other studies are investigating gene therapy as a way to make cancer cells more sensitive to chemotherapy.  Research areas include differentiating drugs, osmotic blood-brain barrier disruption, and convection enhanced delivery.

Differentiating drugs change dividing cells into non-dividing cells and can halt tumor growth.  An early study using retinoids (made from vitamin A) as an independent treatment for certain tumors showed no significant effect.  Scientists are trying retinoids in combination with other chemotherapeutic drugs or treatments to slow the growth of malignant glioma.

Researchers are testing different drugs and molecules to see if they can modulate the normal activity of the blood-brain barrier and better target tumor cells and associated blood vessels. 

Osmotic blood-brain barrier disruption uses certain drugs to open blood vessels throughout the brain.  Scientists are currently studying whether certain chemotherapeutic drugs, when given with osmotic blood-brain barrier disruption, might be an effective way to kill cancer cells without harmful side effects.

Convection enhanced delivery sends a continuous, uniform stream of toxic drugs into the tumor via catheters that are inserted during surgery.  This drug delivery system, which bypasses the blood-brain barrier, is being evaluated in children with recurring brain cancer and in patients whose tumor location prevents its total surgical removal.

Also under investigation are ways to help the body respond to improved drug delivery or other cancer treatments.  Bone marrow and stem cell transplants may replace blood-forming cells that are killed by chemotherapy, radiation treatment, or a combination of therapies.  These transplants, which are given after radiation or chemotherapy, help the patient’s bone marrow recover and produce healthy blood cells.  Researchers are studying the effectiveness of these transplants in protecting blood cells in children with certain types of CNS tumors and in other patients with recurrent or hard to treat CNS cancer, who receive higher than normal doses of chemotherapy.

Although many new approaches to treatment appear promising, it is important to remember that all potential therapies must stand the tests of well-designed, carefully controlled clinical trials and long-term follow-up of treated patients before any conclusions can be drawn about their safety or effectiveness.   New trial designs are also being developed to more quickly evaluate novel agents and may involve pre-selection of patients based on the molecular abnormality in their tumor.

Past research has led to improved tumor treatments and techniques for many individuals with CNS tumors.   Current research promises to generate further improvements.   In the years ahead, physicians and individuals who have a CNS tumor can look forward to new, more effective, less toxic forms of therapy developed through a better molecular understanding of the unique traits of CNS tumors.

[1] Cellular Phone Use and Cancer Risk:  Update of a Danish Nationwide Cohort. Schüz et. al., Journal of the National Cancer Institute, Vol. 98, No. 23, December 6, 2006, pp. 1703-1713.

[2] Central Brain Tumor Registry of the United States, 2005-2006

[3] American Brain Tumor Foundation, 2008

[3] Central Brain Tumor Registry of the United States, 2005-2006

Most cerebral aneurysms are congenital, resulting from an inborn abnormality in an artery wall.  Cerebral aneurysms are also more common in people with certain genetic diseases, such as connective tissue disorders and polycystic kidney disease, and certain circulatory disorders, such as arteriovenous malformations.[1]

Other causes include trauma or injury to the head, high blood pressure, infection, tumors, atherosclerosis (a blood vessel disease in which fats build up on the inside of artery walls) and other diseases of the vascular system, cigarette smoking, and drug abuse.  Some investigators have speculated that oral contraceptives may increase the risk of developing aneurysms.

Aneurysms that result from an infection in the arterial wall are called mycotic aneurysms.  Cancer-related aneurysms are often associated with primary or metastatic tumors of the head and neck.  Drug abuse, particularly the habitual use of cocaine, can inflame blood vessels and lead to the development of brain aneurysms.

There are three types of cerebral aneurysm.  A saccular aneurysm is a rounded or pouch-like sac of blood that is attached by a neck or stem to an artery or a branch of a blood vessel.  Also known as a berry aneurysm (because it resembles a berry hanging from a vine), this most common form of cerebral aneurysm is typically found on arteries at the base of the brain.  Saccular aneurysms occur most often in adults.  A lateral aneurysm appears as a bulge on one wall of the blood vessel, while a fusiform aneurysm is formed by the widening along all walls of the vessel.

Aneurysms are also classified by size.  Small aneurysms are less than 11 millimeters in diameter (about the size of a standard pencil eraser), larger aneurysms are 11-25 millimeters (about the width of a dime), and giant aneurysms are greater than 25 millimeters in diameter (more than the width of a quarter).

Brain aneurysms can occur in anyone, at any age.  They are more common in adults than in children and slightly more common in women than in men.  People with certain inherited disorders are also at higher risk.

All cerebral aneurysms have the potential to rupture and cause bleeding within the brain.  The incidence of reported ruptured aneurysm is about 10 in every 100,000 persons per year (about 27,000 patients per year in the U.S. ), most commonly in people between ages 30 and 60 years.  Possible risk factors for rupture include hypertension, alcohol abuse, drug abuse (particularly cocaine), and smoking.  In addition, the condition and size of the aneurysm affects the risk of rupture.

Most cerebral aneurysms do not show symptoms until they either become very large or burst.  Small, unchanging aneurysms generally will not produce symptoms, whereas a larger aneurysm that is steadily growing may press on tissues and nerves.  Symptoms may include pain above and behind the eye; numbness, weakness, or paralysis on one side of the face; dilated pupils; and vision changes.  When an aneurysm hemorrhages, an individual may experience a sudden and extremely severe headache, double vision, nausea, vomiting, stiff neck, and/or loss of consciousness.  Patients usually describe the headache as “the worst headache of my life” and it is generally different in severity and intensity from other headaches patients may experience.  “Sentinel” or warning headaches may result from an aneurysm that leaks for days to weeks prior to rupture.  Only a minority of patients have a sentinel headache prior to aneurysm rupture.

Other signs that a cerebral aneurysm has burst include nausea and vomiting associated with a severe headache, a drooping eyelid, sensitivity to light, and change in mental status or level of awareness.  Some individuals may have seizures.  Individuals may lose consciousness briefly or go into prolonged coma.  People experiencing this “worst headache,” especially when it is combined with any other symptoms, should seek immediate medical attention.

Most cerebral aneurysms go unnoticed until they rupture or are detected by brain imaging that may have been obtained for another condition.  Several diagnostic methods are available to provide information about the aneurysm and the best form of treatment.  The tests are usually obtained after a subarachnoid hemorrhage, to confirm the diagnosis of an aneurysm.

Angiography is a dye test used to analyze the arteries or veins.  An intracerebral angiogram can detect the degree of narrowing or obstruction of an artery or blood vessel in the brain, head, or neck, and can identify changes in an artery or vein such as a weak spot like an aneurysm.  It is used to diagnose stroke and to precisely determine the location, size, and shape of a brain tumor, aneurysm, or blood vessel that has bled.  This test is usually performed in a hospital angiography suite.  Following the injection of a local anesthetic, a flexible catheter is inserted into an artery and threaded through the body to the affected artery.  A small amount of contrast dye (one that is highlighted on x-rays) is released into the bloodstream and allowed to travel into the head and neck.  A series of x-rays is taken and changes, if present, are noted.

Computed tomography (CT) of the head is a fast, painless, noninvasive diagnostic tool that can reveal the presence of a cerebral aneurysm and determine, for those aneurysms that have burst, if blood has leaked into the brain.  This is often the first diagnostic procedure ordered by a physician following suspected rupture.  X-rays of the head are processed by a computer as two-dimensional cross-sectional images, or “slices,” of the brain and skull.  Occasionally a contrast dye is injected into the bloodstream prior to scanning.  This process, called CT angiography, produces sharper, more detailed images of blood flow in the brain arteries.  CT is usually conducted at a testing facility or hospital outpatient setting.

Magnetic resonance imaging (MRI) uses computer-generated radio waves and a powerful magnetic field to produce detailed images of the brain and other body structures.  Magnetic resonance angiography (MRA) produces more detailed images of blood vessels.  The images may be seen as either three-dimensional pictures or two-dimensional cross-slices of the brain and vessels.  These painless, noninvasive procedures can show the size and shape of an unruptured aneurysm and can detect bleeding in the brain.

Cerebrospinal fluid analysis may be ordered if a ruptured aneurysm is suspected.  Following application of a local anesthetic, a small amount of this fluid (which protects the brain and spinal cord) is removed from the subarachnoid space—located between the spinal cord and the membranes that surround it—by surgical needle and tested to detect any bleeding or brain hemorrhage.  In patients with suspected subarachnoid hemorrhage, this procedure is usually done in a hospital.

Not all cerebral aneurysms burst.  Some patients with very small aneurysms may be monitored to detect any growth or onset of symptoms and to ensure aggressive treatment of coexisting medical problems and risk factors.  Each case is unique, and considerations for treating an unruptured aneurysm include the type, size, and location of the aneurysm; risk of rupture; patient’s age, health, and personal and family medical history; and risk of treatment.

Two surgical options are available for treating cerebral aneurysms, both of which carry some risk to the patient (such as possible damage to other blood vessels, the potential for aneurysm recurrence and rebleeding, and the risk of post-operative stroke).

Microvascular clipping involves cutting off the flow of blood to the aneurysm.  Under anesthesia, a section of the skull is removed and the aneurysm is located.  The neurosurgeon uses a microscope to isolate the blood vessel that feeds the aneurysm and places a small, metal, clothespin-like clip on the aneurysm’s neck, halting its blood supply.  The clip remains in the patient and prevents the risk of future bleeding.  The piece of the skull is then replaced and the scalp is closed.  Clipping has been shown to be highly effective, depending on the location, shape, and size of the aneurysm.  In general, aneurysms that are completely clipped surgically do not return.

A related procedure is an occlusion, in which the surgeon clamps off (occludes) the entire artery that leads to the aneurysm.  This procedure is often performed when the aneurysm has damaged the artery.  An occlusion is sometimes accompanied by a bypass, in which a small blood vessel is surgically grafted to the brain artery, rerouting the flow of blood away from the section of the damaged artery.

Endovascular embolization is an alternative to surgery.  Once the patient has been anesthetized, the doctor inserts a hollow plastic tube (a catheter) into an artery (usually in the groin) and threads it, using angiography, through the body to the site of the aneurysm.  Using a guide wire, detachable coils (spirals of platinum wire) or small latex balloons are passed through the catheter and released into the aneurysm.  The coils or balloons fill the aneurysm, block it from circulation, and cause the blood to clot, which effectively destroys the aneurysm.  The procedure may need to be performed more than once during the patient’s lifetime.

Patients who receive treatment for aneurysm must remain in bed until the bleeding stops.  Underlying conditions, such as high blood pressure, should be treated.  Other treatment for cerebral aneurysm is symptomatic and may include anticonvulsants to prevent seizures and analgesics to treat headache.  Vasospasm can be treated with calcium channel-blocking drugs and sedatives may be ordered if the patient is restless.  A shunt may be surgically inserted into a ventricle several months following rupture if the buildup of cerebrospinal fluid is causing harmful pressure on surrounding tissue.  Patients who have suffered a subarachnoid hemorrhage often need rehabilitative, speech, and occupational therapy to regain lost function and learn to cope with any permanent disability.

An unruptured aneurysm may go unnoticed throughout a person’s lifetime.  A burst aneurysm, however, may be fatal or could lead to hemorrhagic stroke, vasospasm (the leading cause of disability or death following a burst aneurysm), hydrocephalus, coma, or short-term and/or permanent brain damage.

The prognosis for persons whose aneurysm has burst is largely dependent on the age and general health of the individual, other preexisting neurological conditions, location of the aneurysm, extent of bleeding (and rebleeding), and time between rupture and medical attention.  It is estimated that about 40 percent of patients whose aneurysm has ruptured do not survive the first 24 hours; up to another 25 percent die from complications within 6 months.  Patients who experience subarachnoid hemorrhage may have permanent neurological damage.  Other individuals may recover with little or no neurological deficit.  Delayed complications from a burst aneurysm may include hydrocephalus and vapospasm.  Early diagnosis and treatment are important.

Individuals who receive treatment for an unruptured aneurysm generally require less rehabilitative therapy and recover more quickly than persons whose aneurysm has burst.  Recovery from treatment or rupture may take weeks to months.

Results of the International Subarachnoid Aneurysm Trial (ISAT), sponsored primarily by health ministries in the United Kingdom, France, and Canada and announced in October 2002, found that outcome for patients who are treated with endovascular coiling may be superior in the short-term (1 year) to outcome for patients whose aneurysm is treated with surgical clipping.  Long-term results of coiling procedures are unknown and investigators need to conduct more research on this topic, since some aneurysms can recur after coiling.  Before treatment patients may want to consult with a specialist in both endovascular and surgical repair of aneurysms, to help provide greater understanding of treatment options.  (The American Association of Neurological Surgeons notes that most of the centers involved in the ISAT were in Europe [primarily in England], Australia, and Canada, and that results may not be applicable to patients in the United States, “where practice patterns, particularly in reference to the degree of sub-specialization of neurovascular surgeons in major centers, are different.”[1])

The National Institute of Neurological Disorders and Stroke (NINDS), a component of the National Institutes of Health (NIH) within the U.S. Department of Health and Human Services, is the nation’s primary supporter of research on the brain and nervous system.  As part of its mission, the NINDS conducts research on intracranial aneurysms and other vascular lesions of the nervous system and supports studies through grants to medical institutions across the country.

The NINDS recently sponsored the International Study of Unruptured Intracranial Aneurysms, which included more than 4,000 patients at 61 sites in the United States, Canada, and Europe.  The findings suggest that the risk of rupture for most very small aneurysms (less than 7 millimeters in size) is low.  The results also provide a more comprehensive look at these vascular defects and offer guidance to patients and physicians facing the difficult decision about whether or not to treat an aneurysm surgically.

NINDS scientists are studying the effects of an experimental drug in treating vasospasm that occurs following rupture of a cerebral aneurysm.  The drug, developed at the NIH, delivers nitric oxide to the arteries and has been shown to reverse and prevent brain artery spasms in animals. 

Other scientists hope to improve diagnosis and prediction of cerebral vasospasm by developing antibodies to molecules known to cause vasospasm.  These molecules can be detected in the cerebrospinal fluid of subarachnoid hemorrhage patients.  An additional study will compare standard treatment for subarachnoid hemorrhage to standard treatment plus transluminal balloon angioplasty immediately after severe bleeding.  Transluminal balloon angioplasty involves the insertion, via catheter, of a deflated balloon through the affected artery and into the clot.  The balloon is inflated to widen the artery and restore blood flow (the deflated balloon and catheter are then withdrawn).

Researchers are building a new, noninvasive, high-resolution x-ray detector system that can be used to guide the placement of stents (small tube-like devices that keep blood vessels open) used to modify blood flow during treatment for brain aneurysms.

Several groups of NINDS-funded researchers are conducting genetic linkage studies to identify risk factors for familial intracranial aneurysm and/or subarachnoid hemorrhage.  One study hopes to establish patterns of inheritance in patients of different ethnic backgrounds.  Another project is aimed at targeting and providing prevention and treatment strategies for persons who are genetically at high risk for the development of brain aneurysms.  And other investigators will establish a blood and tissue sampling bank for genetic linkage and molecular analyses.

Scientists are investigating the use of intraoperative hypothermia during microclip surgery as a means to improve the rate of recovery of cognitive functions and to reduce early and postoperative complications and neurological damage.  Other studies are investigating ways to improve or replace the coils used in endovascular embolization.

Additional research being funded by the NINDS includes the development of a new animal model of human saccular aneurysm, a new method for tissue processing that should allow routine evaluation of the biological response to implantation of occlusion devices, and a computer simulation model to evaluate the outcomes of neurosurgery in patients with cerebral aneurysms.

In the 1860s, an English surgeon named William Little wrote the first medical descriptions of a puzzling disorder that struck children in the first years of life, causing stiff, spastic muscles in their legs and, to a lesser degree, in their arms. These children had difficulty grasping objects, crawling, and walking. Unlike most other diseases that affect the brain, this condition didn’t get worse as the children grew older.  Instead, their disabilities stayed relatively the same. 

The disorder, which was called Little's disease for many years, is now known as spastic diplegia. It is one of a group of disorders that affect the control of movement and are gathered under the umbrella term of “cerebral palsy.”      

Because it seemed that many of Little’s patients were born following premature or complicated deliveries, the doctor suggested their condition was the result of oxygen deprivation during birth, which damaged sensitive brain tissues controlling movement.  But in 1897, the famous psychiatrist Sigmund Freud disagreed. Noting that children with cerebral palsy often had other neurological problems such as mental retardation, visual disturbances, and seizures, Freud suggested that the disorder might have roots earlier in life, during the brain's development in the womb. "Difficult birth, in certain cases," he wrote, "is merely a symptom of deeper effects that influence the development of the fetus."

In spite of Freud’s observation, for many decades the belief that birth complications caused most cases of cerebral palsy was widespread among physicians, families, and even medical researchers.  In the 1980s, however, scientists funded by the National Institute of Neurological Disorders and Stroke (NINDS) analyzed extensive data from more than 35,000 newborns and their mothers, and discovered that complications during birth and labor accounted for only a fraction of the infants born with cerebral palsy — probably less than 10 percent. In most cases, they could find no single, obvious cause.

This finding challenged the accepted medical theory about the cause of cerebral palsy.  It also stimulated researchers to search for other factors before, during, and after birth that were associated with the disorder.      

Advances in imaging technology, such as magnetic resonance imaging (MRI), have given researchers a way to look into the brains of infants and children with cerebral palsy and discover unique structural malformations and areas of damage.  Basic science studies have identified genetic mutations and deletions associated with the abnormal development of the fetal brain.  These discoveries offer provocative clues about what could be going wrong during brain development to cause the abnormalities that lead to cerebral palsy. 

Much of this new understanding about what causes cerebral palsy is the result of research spanning the past two decades that has been sponsored by the NINDS, the federal government’s leading supporter of neurological research.  These findings from NINDS research have:

This brochure describes what cerebral palsy is, its causes, its treatments, and how it might possibly be prevented.  Medical terms in italics are defined in the glossary at the back of the booklet. 

Doctors use the term cerebral palsy to refer to any one of a number of neurological disorders that appear in infancy or early childhood and permanently affect body movement and muscle coordination but aren’t progressive, in other words, they don’t get worse over time.  The term cerebral refers to the two halves or hemispheres of the brain, in this case to the motor area of the brain’s outer layer (called the cerebral cortex), the part of the brain that directs muscle movement; palsy refers to the loss or impairment of motor function.    

Even though cerebral palsy affects muscle movement, it isn’t caused by problems in the muscles or nerves.  It is caused by abnormalities inside the brain that disrupt the brain’s ability to control movement and posture.

In some cases of cerebral palsy, the cerebral motor cortex hasn’t developed normally during fetal growth.  In others, the damage is a result of injury to the brain either before, during, or after birth.  In either case, the damage is not repairable and the disabilities that result are permanent.      

Children with cerebral palsy exhibit a wide variety of symptoms, including: 

• lack of muscle coordination when performing voluntary movements (ataxia);
• stiff or tight muscles and exaggerated reflexes (spasticity);
• walking with one foot or leg dragging;
• walking on the toes, a crouched gait, or a “scissored” gait;
• variations in muscle tone, either too stiff or too floppy;
• excessive drooling or difficulties swallowing or speaking;
• shaking (tremor) or random involuntary movements; and
• difficulty with precise motions, such as writing or buttoning a shirt.

The symptoms of cerebral palsy differ in type and severity from one person to the next, and may even change in an individual over time.  Some people with cerebral palsy also have other medical disorders, including mental retardation, seizures, impaired vision or hearing, and abnormal physical sensations or perceptions.   

Cerebral palsy doesn’t always cause profound disabilities.   While one child with severe cerebral palsy might be unable to walk and need extensive, lifelong care, another with mild cerebral palsy might be only slightly awkward and require no special assistance.

Cerebral palsy isn’t a disease.  It isn’t contagious and it can’t be passed from one generation to the next.  There is no cure for cerebral palsy, but supportive treatments, medications, and surgery can help many individuals improve their motor skills and ability to communicate with the world.   

The United Cerebral Palsy (UCP) Foundation estimates that nearly 800,000 children and adults in the United States are living with one or more of the symptoms of cerebral palsy. According to the federal government’s Centers for Disease Control and Prevention, each year about 10,000 babies born in the United States will develop cerebral palsy.

Despite advances in preventing and treating certain causes of cerebral palsy, the percentage of babies who develop the condition has remained the same over the past 30 years.  Improved care in neonatal intensive-care units has resulted in higher survival rates for very low birthweight babies.  Many of these infants will have developmental defects in their nervous systems or suffer brain damage that will cause the characteristic symptoms of cerebral palsy. 

The majority of children with cerebral palsy are born with it, although it may not be detected until months or years later.  This is called congenital cerebral palsy.  In the past, if doctors couldn’t identify another cause, they attributed most cases of congenital cerebral palsy to problems or complications during labor that caused asphyxia (a lack of oxygen) during birth.  However, extensive research by NINDS scientists and others has shown that few babies who experience asphyxia during birth grow up to have cerebral palsy or any other neurological disorder. Birth complications, including asphyxia, are now estimated to account for only 5 to 10 percent of the babies born with congenital cerebral palsy.

A small number of children have acquired cerebral palsy, which means the disorder begins after birth.  In these cases, doctors can often pinpoint a specific reason for the problem, such as brain damage in the first few months or years of life, brain infections such as bacterial meningitis or viral encephalitis, or head injury from a motor vehicle accident, a fall, or child abuse.

What causes the remaining 90 to 95 percent?  Research has given us a bigger and more accurate picture of the kinds of events that can happen during early fetal development, or just before, during, or after birth, that cause the particular types of brain damage that will result in congenital cerebral palsy.  There are multiple reasons why cerebral palsy happens – as the result of genetic abnormalities, maternal infections or fevers, or fetal injury, for example.  But in all cases the disorder is the result of four types of brain damage that cause its characteristic symptoms:         

Damage to the white matter of the brain (periventricular leukomalacia [PVL]). The white matter of the brain is responsible for transmitting signals inside the brain and to the rest of the body.   PVL describes a type of damage that looks like tiny holes in the white matter of an infant’s brain.  These gaps in brain tissue interfere with the normal transmission of signals.  There are a number of events that can cause PVL, including maternal or fetal infection.  Researchers have also identified a period of selective vulnerability in the developing fetal brain, a period of time between 26 and 34 weeks of gestation, in which periventricular white matter is particularly sensitive to insults and injury.   

Abnormal development of the brain (cerebral dysgenesis).   Any interruption of the normal process of brain growth during fetal development can cause brain malformations that interfere with the transmission of brain signals.  The fetal brain is particularly vulnerable during the first 20 weeks of development.  Mutations in the genes that control brain development during this early period can keep the brain from developing normally.  Infections, fevers, trauma, or other conditions that cause unhealthy conditions in the womb also put an unborn baby’s nervous system at risk.    

Bleeding in the brain (intracranial hemorrhage).  Intracranial hemorrhage describes bleeding inside the brain caused by blocked or broken blood vessels.  A common cause of this kind of damage is fetal stroke.   Some babies suffer a stroke while still in the womb because of blood clots in the placenta that block blood flow.  Other types of fetal stroke are caused by malformed or weak blood vessels in the brain or by blood-clotting abnormalities.  Maternal high blood pressure (hypertension) is a common medical disorder during pregnancy that has been known to cause fetal stroke.  Maternal infection, especially pelvic inflammatory disease, has also been shown to increase the risk of fetal stroke.    

Brain damage caused by a lack of oxygen in the brain (hypoxic-ischemic encephalopathy or intrapartum asphyxia).   Asphyxia, a lack of oxygen in the brain caused by an interruption in breathing or poor oxygen supply, is common in babies due to the stress of labor and delivery.  But even though a newborn’s blood is equipped to compensate for short-term low levels of oxygen, if the supply of oxygen is cut off or reduced for lengthy periods, an infant can develop a type of brain damage called hypoxic-ischemic encephalopathy, which destroys tissue in the cerebral motor cortex and other areas of the brain.    This kind of damage can also be caused by severe maternal low blood pressure, rupture of the uterus, detachment of the placenta, or problems involving the umbilical cord.

Just as there are particular types of brain damage that cause cerebral palsy, there are also certain medical conditions or events that can happen during pregnancy and delivery that will increase a baby’s risk of being born with cerebral palsy.  Research scientists have examined thousands of expectant mothers, followed them through childbirth, and monitored their children’s early neurological development to establish these risk factors.  If a mother or her baby has any of these risk factors, it doesn’t mean that cerebral palsy is inevitable, but it does increase the chance for the kinds of brain damage that cause it. 

Low birthweight and premature birth.  The risk of cerebral palsy is higher among babies who weigh less than 5 ½ pounds at birth or are born less than 37 weeks into pregnancy. The risk increases as birthweight falls or weeks of gestation shorten. Intensive care for premature infants has improved dramatically over the course of the past 30 years.  Babies born extremely early are surviving, but with medical problems that can put them at risk for cerebral palsy.  Although normal- or heavier-weight babies are at relatively low individual risk for cerebral palsy, term or near-term babies still make up half of the infants born with the condition. 

Multiple births.   Twins, triplets, and other multiple births -- even those born at term -- are linked to an increased risk of cerebral palsy.   The death of a baby’s twin or triplet further increases the risk.

Infections during pregnancy.   Infectious diseases caused by viruses, such as toxoplasmosis, rubella (German measles), cytomegalovirus, and herpes, can infect the womb and placenta.  Researchers currently think that maternal infection leads to elevated levels of immune system cells called cytokines that circulate in the brain and blood of the fetus.  Cytokines respond to infection by triggering inflammation.  Inflammation may then go on to cause central nervous system damage in an unborn baby.  Maternal fever during pregnancy or delivery can also set off this kind of inflammatory response.

Blood type incompatibility.   Rh incompatibility is a condition that develops when a mother’s Rh blood type (either positive or negative) is different from the blood type of her baby.  Because blood cells from the baby and mother mix during pregnancy, if a mother is negative and her baby positive, for example, the mother’s system won’t tolerate the presence of Rh-positive red blood cells.  Her body will begin to make antibodies that will attack and kill her baby’s blood cells.  Rh incompatibility is routinely tested for and treated in the United States , but conditions in other countries continue to keep blood type incompatibility a risk factor for cerebral palsy.   

Exposure to toxic substances.   Mothers who have been exposed to toxic substances during pregnancy, such as methyl mercury, are at a heightened risk of having a baby with cerebral palsy. 

Mothers with thyroid abnormalities, mental retardation, or seizures.   Mothers with any of these conditions are slightly more likely to have a child with cerebral palsy.

There are also medical conditions during labor and delivery, and immediately after delivery, that act as warning signs for an increased risk of cerebral palsy.  Knowing these warning signs helps doctors keep a close eye on children who face a higher risk.  However, parents shouldn’t become too alarmed if their baby has one or more of these conditions at birth. Most of these children will not develop cerebral palsy. Warning signs include: 

Breech presentation.   Babies with cerebral palsy are more likely to be in a breech position (feet first) instead of head first at the beginning of labor.

Complicated labor and delivery.   A baby who has vascular or respiratory problems during labor and delivery may already have suffered brain damage or abnormalities.

Small for gestational age.   Babies born smaller than normal for their gestational age are at risk for cerebral palsy because of factors that kept them from growing naturally in the womb.

Low Apgar score.   The Apgar score is a numbered rating that reflects a newborn's condition.   To determine an Apgar score, doctors periodically check a baby's heart rate, breathing, muscle tone, reflexes, and skin color during the first minutes after birth. They then assign points; the higher the score, the more normal a baby's condition. A low score at 10-20 minutes after delivery is often considered an important sign of potential problems such as cerebral palsy.

Jaundice.  More than 50 percent of newborns develop jaundice after birth when bilirubin, a substance normally found in bile, builds up faster than their livers can break it down and pass it from the body.  Severe, untreated jaundice can cause a neurological condition known as kernicterus, which kills brain cells and can cause deafness and cerebral palsy. 

Seizures.   An infant who has seizures faces a higher risk of being diagnosed later in childhood with cerebral palsy.

Cerebral palsy related to genetic abnormalities is not preventable, but a few of the risk factors for congenital cerebral palsy can be managed or avoided.  For example, rubella, or German measles, is preventable if women are vaccinated against the disease before becoming pregnant.   Rh incompatibilities can also be managed early in pregnancy.  But there are still risk factors that can’t be controlled or avoided in spite of medical intervention. 

For example, the use of electronic fetal monitoring machines to keep track of an unborn baby’s heartbeat during labor, and the use of emergency cesarean section surgery when there are significant signs of fetal distress, haven’t lowered the numbers of babies born with cerebral palsy.  Interventions to treat other prenatal causes of cerebral palsy, such as therapies to prevent prenatal stroke or antibiotics to cure intrauterine infections, are either difficult to administer or haven’t yet been proven to lower the risk of cerebral palsy in vulnerable infants.    

Fortunately, acquired cerebral palsy, often due to head injury, is preventable using common safety tactics, such as using car seats for infants and toddlers, and making sure young children wear helmets when they ride bicycles.  In addition, common sense measures around the household, such as supervising babies and young children closely when they bathe, can reduce the risk of accidental injury.

Despite the best efforts of parents and physicians, however, children will still be born with cerebral palsy. Since in many cases the cause or causes of cerebral palsy aren’t fully known, little can currently be done to prevent it. As investigators learn more about the causes of cerebral palsy through basic and clinical research, doctors and parents will know more about how to prevent this disorder.

The specific forms of cerebral palsy are determined by the extent, type, and location of a child’s abnormalities.  Doctors classify cerebral palsy according to the type of movement disorder involved -- spastic (stiff muscles), athetoid (writhing movements), or ataxic (poor balance and coordination) -- plus any additional symptoms.   Doctors will often describe the type of cerebral palsy a child has based on which limbs are affected. The names of the most common forms of cerebral palsy use Latin terms to describe the location or number of affected limbs, combined with the words for weakened (paresis) or paralyzed (plegia).    For example, hemiparesis (hemi = half) indicates that only one side of the body is weakened.  Quadriplegia (quad = four) means all four limbs are paralyzed. 

Spastic hemiplegia/hemiparesis.   This type of cerebral palsy typically affects the arm and hand on one side of the body, but it can also include the leg.  Children with spastic hemiplegia generally walk later and on tip-toe because of tight heel tendons.  The arm and leg of the affected side are frequently shorter and thinner.  Some children will develop an abnormal curvature of the spine (scoliosis).  Depending on the location of the brain damage, a child with spastic hemiplegia may also have seizures.  Speech will be delayed and, at best, may be competent, but intelligence is usually normal.    

Spastic diplegia/diparesis.   In this type of cerebral palsy, muscle stiffness is predominantly in the legs and less severely affects the arms and face, although the hands may be clumsy.  Tendon reflexes are hyperactive.  Toes point up.  Tightness in certain leg muscles makes the legs move like the arms of a scissor.  Children with this kind of cerebral palsy may require a walker or leg braces.  Intelligence and language skills are usually normal. 

Spastic quadriplegia/quadriparesis.   This is the most severe form of cerebral palsy, often associated with moderate-to-severe mental retardation.  It is caused by widespread damage to the brain or significant brain malformations.   Children will often have severe stiffness in their limbs but a floppy neck.  They are rarely able to walk.  Speaking and being understood are difficult.  Seizures can be frequent and hard to control. 

Dyskinetic cerebral palsy (also includes athetoid, choreoathetoid, and dystonic cerebral palsies).  This type of cerebral palsy is characterized by slow and uncontrollable writhing movements of the hands, feet, arms, or legs.  In some children, hyperactivity in the muscles of the face and tongue makes them grimace or drool.  They find it difficult to sit straight or walk.  Children may also have problems coordinating the muscle movements required for speaking.  Intelligence is rarely affected in these forms of cerebral palsy.    

Ataxic cerebral palsy.   This rare type of cerebral palsy affects balance and depth perception. Children will often have poor coordination and walk unsteadily with a wide-based gait, placing their feet unusually far apart.  They have difficulty with quick or precise movements, such as writing or buttoning a shirt. They may also have intention tremor, in which a voluntary movement, such as reaching for a book, is accompanied by trembling that gets worse the closer their hand gets to the object. 

Mixed types.  It is common for children to have symptoms that don’t correspond to any single type of cerebral palsy.  Their symptoms are a mix of types.  For example, a child with mixed cerebral palsy may have some muscles that are too tight and others that are too relaxed, creating a mix of stiffness and floppiness.

Many individuals with cerebral palsy have no additional medical disorders. However, because cerebral palsy involves the brain and the brain controls so many of the body’s functions, cerebral palsy can also cause seizures, impair intellectual development, and affect vision, hearing, and behavior.  Coping with these disabilities may be even more of a challenge than coping with the motor impairments of cerebral palsy. 

These additional medical conditions include:   

Mental retardation.  Two-thirds of individuals with cerebral palsy will be intellectually impaired.  Mental impairment is more common among those with spastic quadriplegia than in those with other types of cerebral palsy, and children who have epilepsy and an abnormal electroencephalogram (EEG) or MRI are also more likely to have mental retardation. 

Seizure disorder.   As many as half of all children with cerebral palsy have seizures.   Seizures can take the form of the classic convulsions of tonic-clonic seizures or the less obvious focal (partial) seizures, in which the only symptoms may be muscle twitches or mental confusion.

Delayed growth and development.   A syndrome called failure to thrive is common in children with moderate-to-severe cerebral palsy, especially those with spastic quadriparesis.   Failure to thrive is a general term doctors use to describe children who lag behind in growth and development.  In babies this lag usually takes the form of too little weight gain.  In young children it can appear as abnormal shortness, and in teenagers it may appear as a combination of shortness and lack of sexual development.

In addition, the muscles and limbs affected by cerebral palsy tend to be smaller than normal. This is especially noticeable in children with spastic hemiplegia because limbs on the affected side of the body may not grow as quickly or as long as those on the normal side.   

Spinal deformities.  Deformities of the spine -- curvature (scoliosis), humpback (kyphosis), and saddle back (lordosis) -- are associated with cerebral palsy.  Spinal deformities can make sitting, standing, and walking difficult and cause chronic back pain. 

Impaired vision, hearing, or speech.  A large number of children with cerebral palsy have strabismus, commonly called “cross eyes,” in which the eyes are misaligned because of differences between the left and right eye muscles. In an adult, strabismus causes double vision. In children, the brain adapts to the condition by ignoring signals from one of the misaligned eyes. Untreated, this can lead to poor vision in one eye and can interfere with the ability to judge distance. In some cases, doctors will recommend surgery to realign the muscles.   

Children with hemiparesis may have hemianopia, which is defective vision or blindness that blurs the normal field of vision in one eye.   In homonymous hemianopia, the impairment affects the same part of the visual field in both eyes.

Impaired hearing is also more frequent among those with cerebral palsy than in the general population.   Speech and language disorders, such as difficulty forming words and speaking clearly, are present in more than a third of those with cerebral palsy.  

Drooling.   Some individuals with cerebral palsy drool because they have poor control of the muscles of the throat, mouth, and tongue.  Drooling can cause severe skin irritation.  Because it is socially unacceptable, drooling may also isolate children from their peers.   

Incontinence.   A common complication of cerebral palsy is incontinence, caused by poor control of the muscles that keep the bladder closed. Incontinence can take the form of bed-wetting, uncontrolled urination during physical activities, or slow leaking of urine throughout the day.   

Abnormal sensations and perceptions.   Some children with cerebral palsy have difficulty feeling simple sensations, such as touch.  They may have stereognosia, which makes it difficult to perceive and identify objects using only the sense of touch. A child with stereognosia, for example, would have trouble closing his eyes and sensing the difference between a hard ball or a sponge ball placed in his hand.

Early signs of cerebral palsy may be present from birth.  Most children with cerebral palsy are diagnosed during the first 2 years of life.  But if a child’s symptoms are mild, it can be difficult for a doctor to make a reliable diagnosis before the age of 4 or 5.  Nevertheless, if a doctor suspects cerebral palsy, he or she will most likely schedule an appointment to observe the child and talk to the parents about their child’s physical and behavioral development. 

Doctors diagnose cerebral palsy by evaluating a child’s motor skills and taking a careful and thorough look at their medical history. In addition to checking for the most characteristic symptoms -- slow development, abnormal muscle tone, and unusual posture -- a doctor also has to rule out other disorders that could cause similar symptoms.  Most important, a doctor has to determine that the child's condition is not getting worse. Although symptoms may change over time, cerebral palsy by definition is not progressive. If a child is continuously losing motor skills, the problem more likely begins elsewhere – such as a genetic or muscle disease, metabolism disorder, or tumors in the nervous system. A comprehensive medical history, special diagnostic tests, and, in some cases, repeated check-ups can help confirm that other disorders are not at fault.

Additional tests are often used to rule out other movement disorders that could cause the same symptoms as cerebral palsy.  Neuroimaging techniques that allow doctors to look into the brain (such as an MRI scan) can detect abnormalities that indicate a potentially treatable movement disorder.  If it is cerebral palsy, an MRI scan can also show a doctor the location and type of brain damage.

Neuroimaging methods include:

• Cranial ultrasound.   This test is used for high-risk premature infants because it is the least intrusive of the imaging techniques, although it is not as successful as the two methods described below at capturing subtle changes in white matter – the type of brain tissue that is damaged in cerebral palsy. 
• Computed tomography (CT) scan.   This technique creates images that show the structure of the brain and the areas of damage.
• Magnetic resonance imaging (MRI) scan.   This test uses a computer, a magnetic field, and radio waves to create an anatomical picture of the brain's tissues and structures.    Doctors prefer MRI imaging because it offers finer levels of detail.

On rare occasions, metabolic disorders can masquerade as cerebral palsy and some children will require additional tests to rule them out.  Most of the childhood metabolic disorders have characteristic brain abnormalities or malformations that will show up in an MRI.    

Other types of disorders can also be mistaken for cerebral palsy.  For example, coagulation disorders (which prevent blood from clotting) can cause prenatal or perinatal strokes that damage the brain and cause symptoms characteristic of cerebral palsy.  Because stroke is so often the cause of hemiplegic cerebral palsy, a doctor may find it necessary to perform diagnostic testing on children with this kind of cerebral palsy to rule out the presence of a coagulation disorder.  If left undiagnosed, coagulation disorders can cause additional strokes and more extensive brain damage. 

To confirm a diagnosis of cerebral palsy, a doctor may refer a child to additional doctors with specialized knowledge and training, such as a child neurologist, developmental pediatrician, ophthalmologist (eye doctor), or otologist (ear doctor).  Additional observations help a doctor make a more accurate diagnosis and begin to develop a specific plan for treatment.

Cerebral palsy can’t be cured, but treatment will often improve a child's capabilities.   Many children go on to enjoy near-normal adult lives if their disabilities are properly managed. In general, the earlier treatment begins, the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them.   

There is no standard therapy that works for every individual with cerebral palsy.  Once the diagnosis is made, and the type of cerebral palsy is determined, a team of health care professionals will work with a child and his or her parents to identify specific impairments and needs, and then develop an appropriate plan to tackle the core disabilities that affect the child’s quality of life.   

A comprehensive management plan will pull in a combination of health professionals with expertise in the following:   

physical therapy to improve walking and gait, stretch spastic muscles, and prevent deformities; 

occupational therapy to develop compensating tactics for everyday activities such as dressing, going to school, and participating in day-to-day activities; 

speech therapy to address swallowing disorders, speech impediments, and other obstacles to communication; 

counseling and behavioral therapy to address emotional and psychological needs and help children cope emotionally with their disabilities;

drugs to control seizures, relax muscle spasms, and alleviate pain;

surgery to correct anatomical abnormalities or release tight muscles;

braces and other orthotic devices to compensate for muscle imbalance, improve posture and walking, and increase independent mobility;

mechanical aids such as wheelchairs and rolling walkers for individuals who are not independently mobile; and

communication aids such as computers, voice synthesizers, or symbol boards to allow severely impaired individuals to communicate with others.  

Doctors use tests and evaluation scales to determine a child’s level of disability, and then make decisions about the types of treatments and the best timing and strategy for interventions.  Early intervention programs typically provide all the required therapies within a single treatment center.  Centers also focus on parents’ needs, often offering support groups, babysitting services, and respite care.   

The members of the treatment team for a child with cerebral palsy will most likely include the following:    

A physician, such as a pediatrician, pediatric neurologist, or pediatric physiatrist, who is trained to help developmentally disabled children. This doctor, who often acts as the leader of the treatment team, integrates the professional advice of all team members into a comprehensive treatment plan, makes sure the plan is implemented properly, and follows the child’s progress over a number of years.

An orthopedist, a surgeon who specializes in treating the bones, muscles, tendons, and other parts of the skeletal system. An orthopedist is often brought in to diagnose and treat muscle problems associated with cerebral palsy.

A physical therapist, who designs and puts into practice special exercise programs to improve strength and functional mobility.

An occupational therapist, who teaches the skills necessary for day-to-day living, school, and work.

A speech and language pathologist, who specializes in diagnosing and treating disabilities relating to difficulties with swallowing and communication.   

A social worker, who helps individuals and their families locate community assistance and education programs.

A psychologist, who helps individuals and their families cope with the special stresses and demands of cerebral palsy. In some cases, psychologists may also oversee therapy to modify unhelpful or destructive behaviors.

An educator, who may play an especially important role when mental retardation or learning disabilities present a challenge to education.

Regardless of age or the types of therapy that are used, treatment doesn’t end when an individual with cerebral palsy leaves the treatment center.  Most of the work is done at home.   Members of the treatment team often act as coaches, giving parents and children techniques and strategies to practice at home.  Studies have shown that family support and personal determination are two of the most important factors in helping individuals with cerebral palsy reach their long-term goals.

While mastering specific skills is an important focus of treatment on a day-to-day basis, the ultimate goal is to help children grow into adulthood with as much independence as possible. 

As a child with cerebral palsy grows older, the need for therapy and the kinds of therapies required, as well as support services, will likely change.   Counseling for emotional and psychological challenges may be needed at any age, but is often most critical during adolescence. Depending on their physical and intellectual abilities, adults may need help finding attendants to care for them, a place to live, a job, and a way to get to their place of employment. 

Addressing the needs of parents and caregivers is also an important component of the treatment plan.  The well-being of an individual with cerebral palsy depends upon the strength and well-being of his or her family.  For parents to accept a child’s disabilities and come to grips with the extent of their caregiving responsibilities will take time and support from health care professionals.  Family-centered programs in hospitals and clinics and community-based organizations usually work together with families to help them make well-informed decisions about the services they need.  They also coordinate services to get the most out of treatment.    

A good program will encourage the open exchange of information, offer respectful and supportive care, encourage partnerships between parents and the health care professionals they work with, and acknowledge that although medical specialists may be the experts, it’s parents who know their children best.

Physical therapy, usually begun in the first few years of life or soon after the diagnosis is made, is a cornerstone of cerebral palsy treatment. Physical therapy programs use specific sets of exercises and activities to work toward two important goals: preventing weakening or deterioration in the muscles that aren’t being used (disuse atrophy), and keeping muscles from becoming fixed in a rigid, abnormal position (contracture).

Resistive exercise programs (also called strength training) and other types of exercise are often used to increase muscle performance, especially in children and adolescents with mild cerebral palsy.  Daily bouts of exercise keep muscles that aren’t normally used moving and active and less prone to wasting away.  Exercise also reduces the risk of contracture, one of the most common and serious complications of cerebral palsy.

Normally growing children stretch their muscles and tendons as they run, walk, and move through their daily activities.  This insures that their muscles grow at the same rate as their bones. But in children with cerebral palsy, spasticity prevents muscles from stretching.  As a result, their muscles don’t grow fast enough to keep up with their lengthening bones.  The muscle contracture that results can set back the gains in function they’ve made.  Physical therapy alone or in combination with special braces (called orthotic devices) helps prevent contracture by stretching spastic muscles.

Occupational therapy.  This kind of therapy focuses on optimizing upper body function, improving posture, and making the most of a child’s mobility.  An occupational therapist helps a child master the basic activities of daily living, such as eating, dressing, and using the bathroom alone.  Fostering this kind of independence boosts self-reliance and self-esteem, and also helps reduce demands on parents and caregivers.   

Recreational therapies.   Recreational therapies, such as therapeutic horseback riding (also called hippotherapy), are sometimes used with mildly impaired children to improve gross motor skills.  Parents of children who participate in recreational therapies usually notice an improvement in their child’s speech, self-esteem, and emotional well-being. 

Controversial physical therapies.  "Patterning" is a physical therapy based on the principle that children with cerebral palsy should be taught motor skills in the same sequence in which they develop in normal children.  In this controversial approach, the therapist begins by teaching a child elementary movements such as crawling -- regardless of age – before moving on to walking skills. Some experts and organizations, including the American Academy of Pediatrics, have expressed strong reservations about the patterning approach because studies have not documented its value.

Experts have similar reservations about the Bobath technique (which is also called “neurodevelopmental treatment”), named for a husband and wife team who pioneered the approach in England .   In this form of physical therapy, instructors inhibit abnormal patterns of movement and encourage more normal movements. 

The Bobath technique has had a widespread influence on the core physical therapies of cerebral palsy treatment, but there is no evidence that the technique improves motor control.  The American Academy of Cerebral Palsy and Developmental Medicine reviewed studies that measured the impact of neurodevelopmental treatment and concluded that there was no strong evidence supporting its effectiveness for children with cerebral palsy. 

Conductive education, developed in Hungary in the 1940s, is another physical therapy that at one time appeared to hold promise.  Conductive education instructors attempt to improve a child’s motor abilities by combining rhythmic activities, such as singing and clapping, with physical maneuvers on special equipment.  The therapy, however, has not been able to produce consistent or significant improvements in study groups. 

Speech and language therapy.  About 20 percent of children with cerebral palsy are unable to produce intelligible speech.  They also experience challenges in other areas of communication, such as hand gestures and facial expressions, and they have difficulty participating in the basic give and take of a normal conversation.  These challenges will last throughout their lives. 

Speech and language therapists (also known as speech therapists or speech-language pathologists) observe, diagnose, and treat the communication disorders associated with cerebral palsy.   They use a program of exercises to teach children how to overcome specific communication difficulties.

For example, if a child has difficulty saying words that begin with "b," the therapist may suggest daily practice with a list of "b" words, increasing their difficulty as each list is mastered. Other kinds of exercises help children master the social skills involved in communicating by teaching them to keep their head up, maintain eye contact, and repeat themselves when they are misunderstood.

Speech therapists can also help children with severe disabilities learn how to use special communication devices, such as a computer with a voice synthesizer, or a special   board covered with symbols of everyday objects and activities to which a child can point to indicate his or her wishes.          

Speech interventions often use a child’s family members and friends to reinforce the lessons learned in a therapeutic setting.  This kind of indirect therapy encourages people who are in close daily contact with a child to create opportunities for him or her to use their new skills in conversation. 

Treatments for problems with eating and drooling are often necessary when children with cerebral palsy have difficulty eating and drinking because they have little control over the muscles that move their mouth, jaw, and tongue.  They are also at risk for breathing food or fluid into the lungs.  Some children develop gastroesophageal reflux disease (GERD, commonly called heartburn) in which a weak diaphragm can’t keep stomach acids from spilling into the esophagus.  The irritation of the acid can cause bleeding and pain. 

Individuals with cerebral palsy are also at risk for malnutrition, recurrent lung infections, and progressive lung disease.  The individuals most at risk for these problems are those with spastic quadriplegia.

Initially, children should be evaluated for their swallowing ability, which is usually done with a modified barium swallow study.  Recommendations regarding diet modifications will be derived from the results of this study.

In severe cases where swallowing problems are causing malnutrition, a doctor may recommend tube feeding, in which a tube delivers food and nutrients down the throat and into the stomach, or gastrostomy, in which a surgical opening allows a tube to be placed directly into the stomach.

Although numerous treatments for drooling have been tested over the years, there is no one treatment that helps reliably.  Anticholinergic drugs – such as glycopyrolate -- can reduce the flow of saliva but may cause unpleasant side effects, such as dry mouth, constipation, and urinary retention.  Surgery, while sometimes effective, carries the risk of complications.   Some children benefit from biofeedback techniques that help them recognize more quickly when their mouths fall open and they begin to drool.  Intraoral devices (devices that fit into the mouth) that encourage better tongue positioning and swallowing are still being evaluated, but appear to reduce drooling for some children.

Oral medications such as diazepam, baclofen, dantrolene sodium, and tizanidine are usually used as the first line of treatment to relax stiff, contracted, or overactive muscles.  These drugs are easy to use, except that dosages high enough to be effective often have side effects, among them drowsiness, upset stomach, high blood pressure, and possible liver damage with long-term use.  Oral medications are most appropriate for children who need only mild reduction in muscle tone or who have widespread spasticity.

Doctors also sometimes use alcohol “washes” -- injections of alcohol into muscles -- to reduce spasticity.   The benefits last from a few months to 2 years or more, but the adverse effects include a significant risk of pain or numbness, and the procedure requires a high degree of skill to target the nerve.

The availability of new and more precise methods to deliver antispasmodic medications is moving treatment for spasticity toward chemodenervation, in which injected drugs are used to target and relax muscles.  

Botulinum toxin (BT-A), injected locally, has become a standard treatment for overactive muscles in children with spastic movement disorders such as cerebral palsy.  BT-A relaxes contracted muscles by keeping nerve cells from over-activating muscle.  Although BT-A is not approved by the Food and Drug Administration (FDA) for treating cerebral palsy, since the 1990s doctors have been using it off-label to relax spastic muscles.  A number of studies have shown that it reduces spasticity and increases the range of motion of the muscles it targets. 

The relaxing effect of a BT-A injection lasts approximately 3 months.  Undesirable side effects are mild and short-lived, consisting of pain upon injection and occasionally mild flu-like symptoms.  BT-A injections are most effective when followed by a stretching program including physical therapy and splinting.    BT-A injections work best for children who have some control over their motor movements and have a limited number of muscles to treat, none of which is fixed or rigid. 

Because BT-A does not have FDA approval to treat spasticity in children, parents and caregivers should make sure that the doctor giving the injection is trained in the procedure and has experience using it in children.  

Intrathecal baclofen therapy uses an implantable pump to deliver baclofen, a muscle relaxant, into the fluid surrounding the spinal cord.  Baclofen works by decreasing the excitability of nerve cells in the spinal cord, which then reduces muscle spasticity throughout the body.  Because it is delivered directly into the nervous system, the intrathecal dose of baclofen can be as low as one one-hundredth of the oral dose.  Studies have shown it reduces spasticity and pain and improves sleep.   

The pump is the size of a hockey puck and is implanted in the abdomen.  It contains a refillable reservoir connected to an alarm that beeps when the reservoir is low.  The pump is programmable with an electronic telemetry wand.  The program can be adjusted if muscle tone is worse at certain times of the day or night.    

The baclofen pump carries a small but significant risk of serious complications if it fails or is programmed incorrectly, if the catheter becomes twisted or kinked, or if the insertion site becomes infected.  Undesirable, but infrequent, side effects include overrelaxation of the muscles, sleepiness, headache, nausea, vomiting, dizziness, and constipation. 

As a muscle-relaxing therapy, the baclofen pump is most appropriate for individuals with chronic, severe stiffness or uncontrolled muscle movement throughout the body.  Doctors have successfully implanted the pump in children as young as 3 years of age. 

Orthopedic surgery is often recommended when spasticity and stiffness are severe enough to make walking and moving about difficult or painful.  For many people with cerebral palsy, improving the appearance of how they walk – their gait – is also important.  A more upright gait with smoother transitions and foot placements is the primary goal for many children and young adults. 

In the operating room, surgeons can lengthen muscles and tendons that are proportionately too short.  But first, they have to determine the specific muscles responsible for the gait abnormalities.  Finding these muscles can be difficult.  It takes more than 30 major muscles working at the right time using the right amount of force to walk two strides with a normal gait. A problem with any of those muscles can cause an abnormal gait.  

In addition, because the body makes natural adjustments to compensate for muscle imbalances, these adjustments could appear to be the problem, instead of a compensation.   In the past, doctors relied on clinical examination, observation of the gait, and the measurement of motion and spasticity to determine the muscles involved.  Now, doctors have a diagnostic technique known as gait analysis. 

Gait analysis uses cameras that record how an individual walks, force plates that detect when and where feet touch the ground, a special recording technique that detects muscle activity (known as electromyography), and a computer program that gathers and analyzes the data to identify the problem muscles. Using gait analysis, doctors can precisely locate which muscles would benefit from surgery and how much improvement in gait can be expected.

The timing of orthopedic surgery has also changed in recent years.  Previously, orthopedic surgeons preferred to perform all of the necessary surgeries a child needed at the same time, usually between the ages of 7 and 10.  Because of the length of time spent in recovery, which was generally several months, doing them all at once shortened the amount of time a child spent in bed.  Now most of the surgical procedures can be done on an outpatient basis or with a short inpatient stay.  Children usually return to their normal lifestyle within a week. 

Consequently, doctors think it is much better to stagger surgeries and perform them at times appropriate to a child’s age and level of motor development.  For example, spasticity in the upper leg muscles (the adductors), which causes a “scissor pattern” walk, is a major obstacle to normal gait.  The optimal age to correct this spasticity with adduction release surgery is 2 to 4 years of age.  On the other hand, the best time to perform surgery to lengthen the hamstrings or Achilles tendon is 7 to 8 years of age.  If adduction release surgery is delayed so that it can be performed at the same time as hamstring lengthening, the child will have learned to compensate for spasticity in the adductors.  By the time the hamstring surgery is performed, the child’s abnormal gait pattern could be so ingrained that it might not be easily corrected.       

With shorter recovery times and new, less invasive surgical techniques, doctors can schedule surgeries at times that take advantage of a child’s age and developmental abilities for the best possible result.   

Selective dorsal rhizotomy (SDR) is a surgical procedure recommended only for cases of severe spasticity when all of the more conservative treatments – physical therapy, oral medications, and intrathecal baclofen -- have failed to reduce spasticity or chronic pain.  In the procedure, a surgeon locates and selectively severs overactivated nerves at the base of the spinal column.

Because it reduces the amount of stimulation that reaches muscles via the nerves, SDR is most commonly used to relax muscles and decrease chronic pain in one or both of the lower or upper limbs.  It is also sometimes used to correct an overactive bladder.  Potential side effects include sensory loss, numbness, or uncomfortable sensations in limb areas once supplied by the severed nerve. 

Even though the use of microsurgery techniques has refined the practice of SDR surgery, there is still controversy about how selective SDR actually is.  Some doctors have concerns since it is invasive and irreversible and may only achieve small improvements in function.  Although recent research has shown that combining SDR with physical therapy reduces spasticity in some children, particularly those with spastic diplegia, whether or not it improves gait or function has still not been proven.  Ongoing research continues to look at this surgery's effectiveness.  

Spinal cord stimulation was developed in the 1980s to treat spinal cord injury and other neurological conditions involving motor neurons.  An implanted electrode selectively stimulates nerves at the base of the spinal cord to inhibit and decrease nerve activity.   The effectiveness of spinal cord stimulation for the treatment of cerebral palsy has yet to be proven in clinical studies.  It is considered a treatment alternative only when other conservative or surgical treatments have been unsuccessful at relaxing muscles or relieving pain.     

Orthotic devices – such as braces and splints – use external force to correct muscle abnormalities.  The technology of orthotics has advanced over the past 30 years from metal rods that hooked up to bulky orthopedic shoes, to appliances that are individually molded from high-temperature plastics for a precise fit.   Ankle-foot orthoses are frequently prescribed for children with spastic diplegia to prevent muscle contracture and to improve gait.  Splints are also used to correct spasticity in the hand muscles.

Devices that help individuals move about more easily and communicate successfully at home, at school, or in the workplace can help a child or adult with cerebral palsy overcome physical and communication limitations.   There are a number of devices that help individuals stand straight and walk, such as postural support or seating systems, open-front walkers, quadrupedal canes (lightweight metal canes with four feet), and gait poles.  Electric wheelchairs let more severely impaired adults and children move about successfully. 

The computer is probably the most dramatic example of a communication device that can make a big difference in the lives of people with cerebral palsy. Equipped with a computer and voice synthesizer, a child or adult with cerebral palsy can communicate successfully with others.   For example, a child who is unable to speak or write but can make head movements may be able to control a computer using a special light pointer that attaches to a headband.

Therapeutic (subthreshold) electrical stimulation, also called neuromuscular electrical stimulation (NES), pulses electricity into the motor nerves to stimulate contraction in selective muscle groups.  Many studies have demonstrated that NES appears to increase range of motion and muscular strength. 

Threshold electrical stimulation, which involves the application of electrical stimulation at an intensity too low to stimulate muscle contraction, is a controversial therapy.  Studies have not been able to demonstrate its effectiveness or any significant improvement with its use. 

Hyperbaric oxygen therapy.  Some children have cerebral palsy as the result of brain damage from oxygen deprivation.  Proponents of hyperbaric oxygen therapy propose that the brain tissue surrounding the damaged area can be “awakened” by forcing high concentrations of oxygen into the body under greater than atmospheric pressure. 

A recent study compared a group of children who received no hyperbaric treatment to a group that received 40 treatments over 8 weeks.  On every measure of function (gross motor, cognitive, communication, and memory) at the end of 2 months of treatment and after a further 3 months of follow up, the two groups were identical in outcome.  There was no added benefit from hyperbaric oxygen therapy.

Epilepsy.  Twenty to 40 percent of children with mental retardation and cerebral palsy also have epilepsy.  Doctors usually prescribe medications to control seizures.  The classic medications for this purpose are phenobarbital, phenytoin, carbamazepine, and valproate.  Although these drugs generally are effective in controlling seizures, their use is hampered by harmful or unpleasant side effects. 

Treatment for epilepsy has advanced significantly with the development of new medications that have fewer side effects.  These drugs include felbamate, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, tiagabine, topiramate, vigabatrin, and zonisamide.

In general, drugs are prescribed based on the type of seizures an individual experiences, since no one drug controls all types. Some individuals may need a combination of two or more drugs to achieve good seizure control.

Incontinence.  Medical treatments for incontinence include special exercises, biofeedback, prescription drugs, surgery, or surgically implanted devices to replace or aid muscles. Specially designed absorbent undergarments can also be used to protect against accidental leaks.   

Osteopenia.  Children with cerebral palsy who aren’t able to walk risk developing poor bone density (osteopenia), which makes them more likely to break bones.  In a study of older Americans funded by the National Institutes of Health (NIH), a family of drugs called bisphosphonates, which was recently approved by the FDA to treat mineral loss in elderly patients, also appeared to increase bone mineral density.  Doctors may choose to selectively prescribe the drug off-label to children to prevent osteopenia.   

Pain.   Pain can be a problem for people with cerebral palsy due to spastic muscles and the stress and strain on parts of the body that are compensating for muscle abnormalities.  Some individuals may also have frequent and irregular muscle spasms that can’t be predicted or medicated in advance. 

Doctors often prescribe diazepam to reduce the pain associated with muscle spasms, but it’s not known exactly how the drug works to interfere with pain signals.  The drug gabapentin has been used successfully to decrease the severity and frequency of painful spasms.  BT-A injections have also been shown to decrease spasticity and pain, and are commonly given under anesthesia to avoid the pain associated with the injections.  Intrathecal baclofen has shown good results in reducing pain, but its delivery is invasive, time intensive, and expensive.

Some children and adults have been able to decrease pain by using noninvasive and drug-free interventions such as distraction, relaxation training, biofeedback, and therapeutic massage.

Before the mid-twentieth century, few children with cerebral palsy survived to adulthood.  Now, because of improvements in medical care, rehabilitation, and assistive technologies, 65 to 90 percent of children with cerebral palsy live into their adult years.    This increase in life expectancy is often accompanied by a rise in medical and functional problems – some of them beginning at a relatively early age – including the following: 

Premature aging.  The majority of individuals with cerebral palsy will experience some form of premature aging by the time they reach their 40s because of the extra stress and strain the disease puts upon their bodies.  The developmental delays that often accompany cerebral palsy keep some organ systems from developing to their full capacity and level of performance.  As a consequence, organ systems such as the cardiovascular system (the heart, veins, and arteries) and pulmonary system (lungs) have to work harder and they age prematurely.

Functional issues at work.  The day-to-day challenges of the workplace are likely to increase as an employed individual with cerebral palsy reaches middle age.  Some individuals will be able to continue working with accommodations such as an adjusted work schedule, assistive equipment, or frequent rest periods.   Early retirement may be necessary for others. 

Depression.  Mental health issues can also be of concern as someone with cerebral palsy grows older.  The rate of depression is three to four times higher in people with disabilities such as cerebral palsy.  It appears to be related not so much to the severity of their disabilities, but to how well they cope with them.  The amount of emotional support someone has, how successful they are at coping with disappointment and stress, and whether or not they have an optimistic outlook about the future all have a significant impact on mental health.       

Post-impairment syndrome.  Most adults with cerebral palsy experience what is called post-impairment syndrome, a combination of pain, fatigue, and weakness due to muscle abnormalities, bone deformities, overuse syndromes (sometimes also called repetitive motion injuries), and arthritis.   Fatigue is often a challenge, since individuals with cerebral palsy use three to five times the amount of energy that able-bodied people use when they walk and move about.   

Osteoarthritis and degenerative arthritis.  Musculoskeletal abnormalities that may not produce discomfort during childhood can cause pain in adulthood.  For example, the abnormal relationships between joint surfaces and excessive joint compression can lead to the early development of painful osteoarthritis and degenerative arthritis.  Individuals with cerebral palsy also have limited strength and restricted patterns of movement, which puts them at risk for overuse syndromes and nerve entrapments.

Pain.   Issues related to pain often go unrecognized by health care providers since individuals with cerebral palsy may not be able to describe the extent or location of their pain.  Pain can be acute or chronic, and is experienced most commonly in the hips, knees, ankles, and the upper and lower back.  Individuals with spastic cerebral palsy have an increased number of painful sites and worse pain than those with other types of cerebral palsy.  The best treatment for pain due to musculoskeletal abnormalities is preventive – correcting skeletal and muscle abnormalities early in life to avoid the progressive accumulation of stress and strain that causes pain.  Dislocated hips, which are particularly likely to cause pain, can be surgically repaired.   If it is managed properly, pain does not have to become a chronic condition. 

Other medical conditions.  Adults have higher than normal rates of other medical conditions secondary to their cerebral palsy, such as hypertension, incontinence, bladder dysfunction, and swallowing difficulties.  Curvature of the spine (scoliosis) is likely to progress after puberty, when bones have matured into their final shape and size.  People with cerebral palsy also have a higher incidence of bone fractures, occurring most frequently during physical therapy sessions.  A combination of mouth breathing, poor hygiene, and abnormalities in tooth enamel increase the risk of cavities and periodontal disease.   Twenty-five percent to 39 percent of adults with cerebral palsy have vision problems; eight to 18 percent have hearing problems.

Because of their unique medical situations, adults with cerebral palsy benefit from regular visits to their doctor and ongoing evaluation of their physical status.  It is important to evaluate physical complaints to make sure they are not the result of underlying conditions.  For example, adults with cerebral palsy are likely to experience fatigue, but fatigue can also be due to undiagnosed medical problems that could be treated and reversed. 

Because many individuals with cerebral palsy outlive their primary caregiver, the issue of long-term care and support should be taken into account and planned for. 

Investigators from many fields of medicine and health are using their expertise to help improve the treatment and diagnosis of cerebral palsy.  Much of their work is supported through the NINDS, the National Institute of Child Health and Human Development (NICHD), other agencies within the federal government, nonprofit groups such as the United Cerebral Palsy Research and Educational Foundation, and other private institutions.

The ultimate hope for curing cerebral palsy rests with prevention. In order to prevent cerebral palsy, however, scientists have to understand normal fetal brain development so that they can understand what happens when a baby’s brain develops abnormally. 

Between conception and the birth of a baby, one cell divides to form a handful of cells, and then hundreds, millions, and, eventually, billions of cells. Some of these cells specialize to become brain cells, and then specialize even further into particular types of neurons that travel to their appropriate place in the brain (a process that scientists call neuronal migration).  Once they are in the right place, they establish connections with other brain cells.  This is how the brain develops and becomes able to communicate with the rest of the body -- through overlapping neural circuits made up of billions of interconnected and interdependent neurons.

Many scientists now think that a significant number of children develop cerebral palsy because of mishaps early in brain development. They are examining how brain cells specialize and form the right connections, and they are looking for ways to prevent the factors that disrupt the normal processes of brain development.

Genetic defects are sometimes responsible for the brain malformations and abnormalities that cause cerebral palsy.  Scientists funded by the NINDS are searching for the genes responsible for these abnormalities by collecting DNA samples from people with cerebral palsy and their families and using genetic screening techniques to discover linkages between individual genes and specific types of abnormality – primarily those associated with abnormal neuronal migration. 

Scientists are scrutinizing events in newborn babies’ brains, such as bleeding, epileptic seizures, and breathing and circulation problems, which can cause the abnormal release of chemicals that trigger the kind of damage that causes cerebral palsy.  For example, research has shown that bleeding in the brain unleashes dangerously high amounts of a brain chemical called glutamate.   Although glutamate is necessary in the brain to help neurons communicate, too much glutamate overexcites and kills neurons. Scientists are now looking closely at glutamate to detect how its release harms brain tissue.  By learning how brain chemicals that are normally helpful become dangerously toxic, scientists will have opportunities to develop new drugs to block their harmful effects.

Scientists funded by the NINDS are also investigating whether substances in the brain that protect neurons from damage, called neurotrophins, could be used to prevent brain damage as a result of stroke or oxygen deprivation.  Understanding how these neuroprotective substances act would allow scientists to develop synthetic neurotrophins that could be given immediately after injury to prevent neuron death and damage.

The relationship between uterine infections during pregnancy and the risk of cerebral palsy continues to be studied by researchers funded by the NIH.  There is evidence that uterine infections trigger inflammation and the production of immune system cells called cytokines, which can pass into an unborn baby’s brain and interrupt normal development.  By understanding what cytokines do in the fetal brain and the type of damage these immune system cells cause, researchers have the potential to develop medications that could be given to mothers with uterine infections to prevent brain damage in their unborn children. 

Approximately 10 percent of newborns are born prematurely, and of those babies, more than 10 percent will have brain injuries that will lead to cerebral palsy and other brain-based disabilities.   A particular type of damage to the white matter of the brain, called periventricular leukomalacia (PVL), is the predominant form of brain injury in premature infants.  NINDS-sponsored researchers studying PVL are looking for new strategies to prevent this kind of damage by developing safe, nontoxic therapies delivered to at-risk mothers to protect their unborn babies.

Although congenital cerebral palsy is a condition that is present at birth, a year or two can pass before any disabilities are noticed.  Researchers have shown that the earlier rehabilitative treatment begins, the better the outcome for children with cerebral palsy.  But an early diagnosis is hampered by the lack of diagnostic techniques to identify brain damage or abnormalities in infants. 

Research funded by the NINDS is using imaging techniques, devices that measure electrical activity in the brain, and neurobehavioral tests to predict those preterm infants who will develop cerebral palsy.  If these screening techniques are successful, doctors will be able to identify infants at risk for cerebral palsy before they are born.        

Noninvasive methods to record the brain activity of unborn babies in the womb and to identify those with brain damage or abnormalities would also be a valuable addition to the diagnostic tool kit.  Another NINDS-funded study focuses on the development of fetal magnetoencephalography (fMEG) – a technology that would allow doctors to look for abnormalities in fetal brain activity.

Epidemiological studies – studies that look at the distribution and causes of disease among people -- help scientists understand risk factors and outcomes for particular diseases and medical conditions.  Researchers have established that preterm birth (when a baby is born before 32 weeks’ gestation) is the highest risk factor for cerebral palsy.  Consequently, the increasing rate of premature births in the United States puts more babies at risk.  A large, long-term study funded by the NIH is following a group of more than 400 mothers and their infants born between 24 and 31 weeks’ gestation.  They are looking for relationships between preterm birth, maternal uterine infection, fetal exposure to infection, and short-term and long-term health and neurological outcomes.  The researchers are hoping to discover environmental or lifestyle factors, or particular characteristics of mothers, which might protect preterm babies from neurological disabilities.     

While this research offers hope for preventing cerebral palsy in the future, ongoing research to improve treatment brightens the outlook for those who must face the challenges of cerebral palsy today. An important thrust of such research is the evaluation of treatments already in use so that physicians and parents have valid information to help them choose the best therapy. A good example of this effort is an ongoing NINDS-supported study that promises to yield new information about which patients are most likely to benefit from selective dorsal rhizotomy, a surgical technique that is increasingly being used to reduce spasticity (see Surgery).

Similarly, although physical therapy programs are used almost universally to rehabilitate children with cerebral palsy, there are no definitive studies to indicate which techniques work best.  For example, constraint-induced therapy (CIT) is a type of physical therapy that has been used successfully with adult stroke survivors and individuals who have traumatic brain injury and are left with a weak or disabled arm on one side of the body.  The therapy involves restraining the stronger arm in a cast and forcing the weaker arm to perform 6 hours of intensive “shaping” activities every day over the course of 3 weeks.  The researchers who conducted the clinical trials in adult stroke survivors realized CIT’s potential for strengthening children’s arms weakened by cerebral palsy. 

In a randomized, controlled study of children with cerebral palsy funded by the NIH, researchers put one group of children through conventional physical therapy and another group through 21 consecutive days of CIT.   Researchers looked for evidence of improvement in the movement and function of the disabled arm, whether the improvement lasted after the end of treatment, and if it was associated with significant gains in other areas, such as trunk control, mobility, communication, and self-help skills. 

Children receiving CIT outperformed the children receiving conventional physical therapy across all measures of success, including how well they could move their arms after therapy and their ability to do new tasks during the study and then at home with their families.  Six months later they still had better control of their arm.  The results from this study are the first to prove the benefits of a physical therapy.  Additional research to determine the optimal length and intensity of CIT will allow doctors to add this therapy to the cerebral palsy treatment toolbox. 

Studies have shown that functional electrical stimulation is an effective way to target and strengthen spastic muscles, but the method of delivering the electrical pulses requires expensive, bulky devices implanted by a surgeon, or skin surface stimulation applied by a trained therapist.  NINDS-funded researchers have developed a high-tech method that does away with the bulky apparatus and lead wires by using a hypodermic needle to inject microscopic wireless devices into specific muscles or nerves.  The devices are powered by a telemetry wand that can direct the number and strength of their pulses by remote control.  The device has been used to activate and strengthen muscles in the hand, shoulder, and ankle in people with cerebral palsy as well as in stroke survivors.   

As researchers continue to explore new treatments for cerebral palsy and to expand our knowledge of brain development, we can expect significant improvements in the care of children with cerebral palsy and many other disorders that strike in early life.