806 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS Stroke A man who suffered a stroke is helped with his rehabilita- tion by a physical therapist. (© 1993 ATC Productions. Custom Medical Stock Photo. Reproduced by permission.) Other investigations that may be performed to guide treatment include an electrocardiogram, angiography,ul- trasound, and electroencephalogram. Treatment team Stroke treatment involves a multidisciplinary team. Physicians are responsible for caring for the stroke sur- vivor’s general health and providing guidance aimed at preventing a second stroke. Neurologists usually lead acute-care stroke teams and direct patient care during hos- pitalization. The team may include a physiatrist (a spe- cialist in rehabilitation), a rehabilitation nurse, a physical therapist, an occupational therapist, a speech-language pathologist, a social worker, a psychologist, and a voca- tional counselor. Treatment Emergency treatment Emergency treatment of stroke from a blood clot is aimed at dissolving the clot. This “thrombolytic therapy” is currently performed most often with tissue plasminogen activator, or t-PA. This t-PA must be administered within three hours of the stroke event. Therefore, patients who awaken with stroke symptoms are ineligible for t-PA ther- apy, as the time of onset cannot be accurately determined. The t-PA therapy has been shown to improve recovery and decrease long-term disability in selected patients. The t-PA therapy carries a 6.4% risk of inducing a cerebral hemor- rhage, and is not appropriate for patients with bleeding disorders, very high blood pressure, known aneurysms, any evidence of intracranial hemorrhage, or incidence of stroke, head trauma, or intracranial surgery within the past three months. Patients with clot-related (thrombotic or em- bolic) stroke who are ineligible for t-PA treatment may be treated with heparin or other blood thinners, or with as- pirin or other anti-clotting agents in some cases. Emergency treatment of hemorrhagic stroke is aimed at controlling intracranial pressure. Intravenous urea or mannitol plus hyperventilation are the most common treatments. Corticosteroids may also be used. Patients with reversible bleeding disorders such as those due to antico- agulant treatment should have these bleeding disorders re- versed, if possible. Surgery for hemorrhage due to aneurysm may be per- formed if the aneurysm is close enough to the cranial sur- face to allow access. Ruptured vessels are closed off to prevent rebleeding. For aneurysms that are difficult to reach surgically, endovascular treatment may be used. In this procedure, a catheter is guided from a larger artery up into the brain to reach the aneurysm. Small coils of wire are discharged into the aneurysm, which plug it up and block off blood flow from the main artery. Recovery and rehabilitation Rehabilitation refers to a comprehensive program designed to help the patient regain function as much as possible and compensate for permanent losses. Approxi- mately 10% of stroke survivors are without any significant disability and able to function independently. Another 10% are so severely affected that they must remain insti- tutionalized for severe disability. The remaining 80% can return home with appropriate therapy, training, support, and care services. Rehabilitation is coordinated by a team that may in- clude the services of a neurologist, a physiatrist, a phys- ical therapist, an occupational therapist, a speech-language pathologist, a nutritionist, a mental health professional, and a social worker. Rehabilitation services may be pro- vided in an acute care hospital, rehabilitation hospital, long-term care facility, outpatient clinic, or at home. The rehabilitation program is based on the patient’s in- dividual deficits and strengths. Strokes on the left side of the brain primarily affect the right half of the body, and vice versa. In addition, in left-brain-dominant people, who con- stitute a significant majority of the population, left-brain strokes usually lead to speech and language deficits, while right-brain strokes may affect spatial perception. Patients LetterS.qxd 10/1/04 11:09 AM Page 806 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS 807 Stroke Key Terms Aneurysm A pouch-like bulging of a blood vessel. Atrial fibrillation A disorder of the heartbeat as- sociated with a higher risk of stroke. In this disorder, the upper chambers (atria) of the heart do not com- pletely empty when the heart beats, which can allow blood clots to form. Cerebral embolism A blockage of blood flow through a vessel in the brain by a blood clot that formed elsewhere in the body and traveled to the brain. Cerebral thrombosis A blockage of blood flow through a vessel in the brain by a blood clot that formed in the brain itself. Intracerebral hemorrhage A cause of some strokes in which vessels within the brain begin bleeding. Subarachnoid hemorrhage A cause of some strokes in which arteries on the surface of the brain begin bleeding. Tissue plasminogen activator (tPA) A substance that is sometimes given to patients within three hours of a stroke to dissolve blood clots within the brain. with right-brain strokes may also deny their illness, neglect the affected side of their body, and behave impulsively. Rehabilitation may be complicated by cognitive losses, including diminished ability to understand and fol- low directions. Poor results are more likely in patients with significant or prolonged cognitive changes, sensory losses, language deficits, or incontinence. Preventing complications Rehabilitation begins with prevention of stroke re- currence and other medical complications. The risk of stroke recurrence may be reduced with many of the same measures used to prevent stroke, including quitting smok- ing and controlling blood pressure. One of the most common medical complications fol- lowing stroke is deep venous thrombosis, in which a clot forms within a limb immobilized by paralysis. Clots that break free can often become lodged in an artery feeding the lungs. This type of pulmonary embolism is a common cause of death in the weeks following a stroke. Resuming activity within a day or two after the stroke is an important preventive measure, along with use of elastic stockings on the lower limbs. Drugs that prevent clotting may be given, including intravenous heparin and oral warfarin. Weakness and loss of coordination of the swallowing muscles may impair swallowing (dysphagia), and allow food to enter the lower airway. This may lead to aspiration pneumonia, another common cause of death shortly after a stroke. Dysphagia may be treated with retraining exer- cises and temporary use of pureed foods. Depression occurs in 30–60% of stroke patients. An- tidepressants and psychotherapy may be used in combi- nation. Other medical complications include urinary tract in- fections, pressure ulcers, falls, and seizures. Types of rehabilitative therapy Brain tissue that dies in a stroke cannot regenerate. In some cases, other brain regions may perform the func- tions of that tissue after a training period. In other cases, compensatory actions may be developed to replace lost abilities. Physical therapy is used to maintain and restore range of motion and strength in affected limbs, and to maximize mobility in walking, wheelchair use, and transferring (from wheelchair to toilet or from standing to sitting, for instance). The physical therapist advises on mobility aids such as wheelchairs, braces, and canes. In the recovery pe- riod, a stroke patient may develop muscle spasticity and contractures, or abnormal contractions. Contractures may be treated with a combination of stretching and splinting. Occupational therapy improves self-care skills such as feeding, bathing, and dressing, and helps develop ef- fective compensatory strategies and devices for activities of daily living. A speech-language pathologist focuses on communication and swallowing skills. When dysphagia is a problem, a nutritionist can advise alternative meals that provide adequate nutrition. Mental health professionals may be involved in the treatment of depression or loss of thinking (cognitive) skills. A social worker may help coordinate services and ease the transition out of the hospital back into the home. Both social workers and mental health professionals may help counsel the patient and family during the difficult re- habilitation period. Caring for a person affected with stroke requires learning a new set of skills and adapting to new demands and limitations. Home caregivers may de- velop stress, anxiety, and depression. Caring for the care- giver is an important part of the overall stroke treatment program. Support groups can provide an important source of in- formation, advice, and comfort for stroke patients and for caregivers. Joining a support group can be one of the most important steps in the rehabilitation process. LetterS.qxd 10/1/04 11:09 AM Page 807 808 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS Sturge-Weber syndrome Clinical trials As of mid-2004, there were numerous open clinical trials for stroke, including: • “Adjunctive Drug Treatment for Ischemic Stroke Pa- tients,” “E-Selectin Nasal Spray to Prevent Stroke Re- currence,” “Improving Motor Learning in Stroke Patients,” “Aspirin or Warfarin to Prevent Stroke,” “Hand Exercise and Upper Arm Anesthesia to Improvements Hand Function in Chronic Stroke Patients,” “Preliminary Study of Transcranial Magnetic Stimulation for Stroke Rehabilitation,” and “Using fMRI to Understand the Roles of Brain Areas for Fine Hand Movements” are all sponsored by the National Institute of Neurological Dis- orders and Stroke. • “Preventing Post-Stroke Depression” is sponsored by the National Institute of Mental Health (NIMH). • “Walking Therapy in Hemiparetic Stroke Patients Using Robotic-Assisted Treadmill Training” is sponsored by the United States Department of Education. • “Brain Processing of Language Meanings” is sponsored by Warren G. Magnuson Clinical Center. Updated information on these and other ongoing tri- als for the study and treatment of stroke can be found at the National Institutes of Health Web site for clinical tri- als at <http://www.clinicaltrials.org>. Prognosis Stroke is fatal for about 27% of white males, 52% of black males, 23% of white females, and 40% of black fe- males. Stroke survivors may be left with significant deficits. Emergency treatment and comprehensive reha- bilitation can significantly improve both survival and re- covery. Prevention Damage from stroke may be significantly reduced through emergency treatment. Knowing the symptoms of stroke is as important as knowing those of a heart attack. Patients with stroke symptoms should seek emergency treatment without delay, which may mean dialing 911 rather than their family physician. The risk of stroke can be reduced through lifestyle changes, including: • stopping smoking • controlling blood pressure • getting regular exercise • keeping weight down • avoiding excessive alcohol consumption • getting regular checkups and following the doctor’s ad- vice regarding diet and medicines Treatment of atrial fibrillation may significantly re- duce the risk of stroke. Preventive anticoagulant therapy may benefit those with untreated atrial fibrillation. War- farin (Coumadin) has proven to be more effective than as- pirin for those with higher risk. Screening for aneurysms may be an effective preven- tive measure in those with a family history of aneurysms or autosomal polycystic kidney disease, which tends to be associated with aneurysms. Resources BOOKS Caplan, L. R., M. L. Dyken, and J. D. Easton. American Heart Association Family Guide to Stroke Treatment, Recovery, and Prevention. New York: Times Books, 1996. Warlow, C. P., et al. Stroke: A Practical Guide to Management. Boston: Blackwell Science, 1996. Weiner F., M. H. M. Lee, and H. Bell. Recovering at Home After a Stroke: A Practical Guide for You and Your Family. Los Angeles: The Body Press/Perigee Books, 1994. PERIODICALS Selman, W. R., R. Tarr, and D. M. D. Landis. “Brain Attack: Emergency Treatment of Ischemic Stroke.” American Family Physician 55 (June 1997): 2655–2662. Wolf, P. A., and D. E. Singer. “Preventing Stroke in Atrial Fibrillation.” American Family Physician (December 1997). ORGANIZATIONS National Stroke Association. 9707 E. Easter Lane, Englewood, Co. 80112. (800) 787-6537. (June 3, 2004). <http://www.stroke.org>. American Heart Association. 7320 Greenville Ave. Dallas, TX 75231. (214) 373-6300. (June 3, 2004). <http:// www.americanheart.org>. Richard Robinson ❙ Sturge-Weber syndrome Definition Sturge-Weber syndrome (SWS) is a condition in- volving specific brain changes that often cause seizures and mental delays. It also includes port-wine colored birth- marks (or “port-wine stains”), usually found on the face. Description The brain finding in SWS is leptomeningeal angioma, which is a swelling of the tissue surrounding the brain and spinal cord. These angiomas cause seizures in approxi- mately 90% of people with SWS. A large number of af- fected individuals are also mentally delayed. LetterS.qxd 10/1/04 11:09 AM Page 808 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS 809 Sturge-Weber syndrome This magnetic resonance image of the brain shows a patient affected with Sturge-Weber syndrome. The front of the brain is at the top. Green colored areas indicate fluid- filled ventricles. The blue area is where the brain has become calcified. Photo Researchers, Inc. Port-wine stains are present at birth. They can be quite large and are typically found on the face near the eyes or on the eyelids. Vision problems are common, especially if a port-wine stain covers the eyes. These vision problems can include glaucoma and vision loss. Facial features, such as port-wine stains, can be very challenging for individuals with SWS. These birthmarks can increase in size with time, and this may be particularly emotionally distressing for the individuals, as well as their parents. A state of unhappiness about this is more common during middle childhood and later than it is at younger ages. Genetic profile The genetics behind Sturge-Weber syndrome are still unknown. Interestingly, in other genetic conditions in- volving changes in the skin and brain (such as neurofi- bromatosis and tuberous sclerosis) the genetic causes are well described. It is known that most people with SRS are the only ones in their family with the condition; there is usually not a strong family history of the disease. How- ever, a gene known to cause SWS has not been identified. For now, SWS is thought to be caused by a random, spo- radic event. Demographics Sturge-Weber syndrome is a sporadic disease that is found throughout the world, affecting males and females equally. The total number of people with Sturge-Weber syndrome is not known, but estimates range between one in 400,000 to one in 40,000. Causes and symptoms People with SWS may have a larger head circumfer- ence (measurement around the head) than usual. Lep- tomeningeal angiomas can progress with time. They usually only occur on one side of the brain, but can exist on both sides in up to 30% of people with SWS. The an- giomas can also cause great changes within the brain’s white matter. Generalized wasting, or regression, of por- tions of the brain can result from large angiomas. Calcifi- cation of the portions of the brain underlying the angiomas can also occur. The larger and more involved the angiomas are, the greater the expected amount of mental delays in the individual. Seizures are common in SWS, and they can often begin in very early childhood. Occa- sionally, slight paralysis affecting one side of the body may occur. Port-wine stains are actually capillaries (blood ves- sels) that reach the skin’s surface and grow larger than usual. As mentioned earlier, the birthmarks mostly occur near the eyes; they often occur only on one side of the face. Though they can increase in size over time, port- wine stains cause no direct health problems for the person with SWS. Vision loss and other complications are common in SWS. The choroid of the eye can swell, and this may lead to increased pressure within the eye in 33–50% of people with SWS. Glaucoma is another common vision problem seen in SWS, and is more often seen when a person has a port-wine stain that is near or touches the eye. In a 2000 study about the psychological functioning of children with SWS, it was noted that parents and teach- ers report a higher incidence of social problems, emotional distress, and problems with compliance in these individu- als. Taking the mental delays into account, behaviors as- sociated with attention-deficit hyperactivity disorder (ADHD) were noted; as it turns out, about 22% of people with SWS are eventually diagnosed with ADHD. Diagnosis Because no genetic testing is available for Sturge- Weber syndrome, all diagnoses are made through a care- ful physical examination and study of a person’s medical history. Port-wine stains are present at birth, and seizures may occur in early childhood. If an individual has both of these features, SWS should be suspected. A brain MRI or CT scan can often reveal a leptomeningeal angioma or brain LetterS.qxd 10/1/04 11:09 AM Page 809 810 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS Stuttering Key Terms Calcification A process in which tissue becomes hardened due to calcium deposits. Choroid A vascular membrane that covers the back of the eye between the retina and the sclera and serves to nourish the retina and absorb scat- tered light. Computed tomography (CT) scan An imaging procedure that produces a three-dimensional pic- ture of organs or structures inside the body, such as the brain. Glaucoma An increase in the fluid eye pressure, eventually leading to damage of the optic nerve and ongoing visual loss. Leptomeningeal angioma A swelling of the tissue or membrane surrounding the brain and spinal cord, which can enlarge with time. Magnetic resonance imaging (MRI) A technique that employs magnetic fields and radio waves to create detailed images of internal body structures and organs, including the brain. Port-wine stain Dark-red birthmarks seen on the skin, named after the color of the dessert wine. Sclera The tough white membrane that forms the outer layer of the eyeball. calcifications, as well as any other associated white mat- ter changes. Treatment Treatment of seizures in SWS by anti-epileptic med- ications is often an effective way to control them. In the rare occasion that an aggressive seizure medication ther- apy is not effective, surgery may be necessary. The general goal of the surgery is to remove the portion of brain that is causing the seizures, while keeping the normal brain tissue intact. Though most patients with SWS only have brain surgery as a final attempt to treat seizures, some physicians favor earlier surgery because this may prevent some irre- versible damage to the brain (caused by the angiomas). Standard glaucoma treatment, including medications and surgery, is used to treat people with this complication. This can often reduce the amount of vision loss. There is no specific treatment for port-wine stains. Because they contain blood vessels, it could disrupt blood flow to remove or alter the birthmarks. Prognosis The prognosis for people with SWS is directly related to the amount of brain involvement for the leptomeningeal angiomas. For those individuals with smaller angiomas, prognosis is relatively good, especially if they do not have severe seizures or vision problems. Resources BOOKS Charkins, Hope. Children with Facial Difference: A Parent’s Guide. Bethesda, MD: Woodbine House, 1996. ORGANIZATIONS The Sturge-Weber Foundation. PO Box 418, Mount Freedom, NJ 07970. (800) 627-5482 or (973) 895-4445. Fax: (973) 895-4846. swfoffice@aol.com. <http://www.sturge weber.com/>. WEBSITES “Sturge-Weber Syndrome.” Family Village. <http://www. familyvillage.wisc.edu/lib_stur.htm>. Sturge-Weber Syndrome Support Group of New Zealand. <http://www.geocities.com/HotSprings/Spa/1563/>. Deepti Babu, MS ❙ Stuttering Definition Stuttering has no absolute definition that encom- passes all the aspects of the disorder. In general, it is a con- dition in which a person trying to speak has difficulty in expressing words normally. Morphemes (actual individual sounds such as “mm” or the explosive “p”) are not easily articulated. Two common symptoms of stuttering are the drawing out of the morpheme as in “mmmmmore” or the repetitious “l-l-l-look” of seemingly simple words. Stuttering is not to be confused with another speech disorder called cluttering. Cluttering has a much more de- finitive cause and clearer symptoms. Its neurogenic link has been more thoroughly established, while the roots of stuttering have not. Cluttering involves a rapid speech pat- tern, while stuttering can take on a variety of levels of complexity. Description In the past, researchers and speech therapists assumed that stuttering was a developmental disorder. Increasing evidence points to a genetic cause in many patients, espe- cially males. The results are far from clear and studies are conflicting in their data and conclusions. Many studies are focused on the fact that monozygotic (one egg) twins both seem to stutter when the disorder is present. LetterS.qxd 10/1/04 11:09 AM Page 810 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS 811 Stuttering Key Terms Clutter A fluency disorder where speech delivery is either abnormally fast, irregular, or both. Stutter A speech disorder involving hesitations and involuntary repetitions of certain sounds. Stuttering is usually identified in children. Unless the situation is extremely stressful, such as speaking in front of a large group of people, or an equally distressing con- dition is present, very few adults begin to stutter later in life. Stress and anxiety about the inability to easily express thoughts and words is very distressing for the child who stutters and can prolong recovery or even prevent it. The social anxiety accompanying stuttering is one of the reasons researchers have historically cited the lack of emotional well-being or the production of high anxiety as the root cause of the disorder. While at an early age, when peer pressure and social acceptance is extraordinarily im- portant, the lack of understanding by other children can be very difficult to overcome. At this point, stuttering does become an emotional as well as physical challenge. Demographics More than 1% of the population stutters. However, if every person who has, at some time, found themselves stuttering when anxious were included, the condition would be considered a great deal more common. Males are four times more likely than females to stutter. Stutter- ing is also more common in children than adults. The Stuttering Foundation of America has provided facts on who is likely to stutter. They describe four of the most common factors that lead to stuttering. The first is genetics. Clinical results indicate that around 60% of those who stutter have a family member who also stutters. A sec- ond possible cause for stuttering involves developmental delays. Researchers claim that children with other speech and language problems are more likely to stutter than those who do not. The third proposed reason for stuttering involves the physiology of the brain. With magnetic resonance im- aging (MRI) and other such examinations, it appears that some people process speech and language in different re- gions of the brain than those who do not stutter. Early lan- guage acquisition occurs in the Broca’s area of the brain, but this ability lasts only for a short time during childhood. After initial speech is acquired, language is learned in other regions of the brain. This may have an influence on those who stutter. Finally, family dynamics are implicated as reasons for stuttering. Parents with high expectations and little pa- tience may push a child to speak before he or she is ready. Without proper education, some parents may push their children to achieve certain goals by a particular age. If the goal is not, met a child may experience anxiety and it is possible this could result in stuttering. Causes and symptoms The actual physiological cause of stuttering is not conclusive. Neurogenic stutterers are those people who have de- veloped the disorder as a result of some sort of head injury or trauma. Their speech may be repetitious, prolonged, and they may even experience a mental block on certain words or phrases. However, they seem to lack the fears and anxieties of those who are designated as developmental stutterers. The severity of neurogenic stuttering is directly correlated with the degree of brain injury and degree of healing. Diagnosis A health professional or speech therapist trained in identifying varying speech disorders makes the diagnosis of stuttering. Stuttering must be isolated from anxious stammering, brain-related cluttering, and a variety of ad- ditional speech disorders. Treatment team The treatment team for a stutterer is multidisciplinary. Initially, a child’s parent or teacher may identify a problem in communication and reading aloud. The pediatrician usually identifies and makes the diagnosis of stuttering as opposed to other vocal disturbances. A neurological con- sultation may be sought. Occurrences such as head trauma or lesions of the brain must be ruled out as a contributing factor. Many speech and language pathologists have been trained and licensed to work with stutterers. They can pro- vide exercises, vocal awareness, and support that the stut- terer needs to begin a path to recovery. Many schools offer these types of support and are free to the students. One of the best teams for the treatment of stuttering is the family and friends of the person who stutters. It is likely the stutterer feels embarrassment or guilt over the condition. Family and friends who take the time to under- stand the condition and show their patience and accept- ance can help the person who stutters. Reading books about the condition and aiding in home therapies is a proven method of making the stutterer feel less shame and embarrassment. In turn, the benefits of therapy can be reached more quickly. LetterS.qxd 10/1/04 11:09 AM Page 811 812 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS Subacute sclerosing panencephalitis Treatment Most clinicians recommend a holistic approach in which patients are allowed to find their own most useful therapy. A good rapport should exist between the speech therapist and patient. Significantly, often when the person who stutters fo- cuses on a related task such as singing, the individual fails to show any symptoms. When a prescribed set of words and additional distraction are employed, it appears the stutterer has fewer problems speaking clearly. Singing and rhyming are strategies used by speech therapists as con- fidence boosters to illustrate that the person has the abil- ity to express language in a natural, easily flowing manner. Recently, some electrical devices for the treatment of stuttering have come onto the market, but their success is still not well documented. The Delayed Auditory Feed- back (DAF) and Frequency-Shifting Auditory Feedback (FAF) are electronic devices that pick up a voice from a microphone, delay the sound for a fraction of a second, and feed the voice back through earphones. Some clini- cians claim the feedback machines can significantly re- duce or eliminate stuttering. Recovery and rehabilitation Recovery from stuttering is unpredictable for several reasons. Many people must come to the aid of the stutterer. Family and friends, the therapist, schoolmates, and a vari- ety of additional environmental conditions must be in place for the stutterer to gain control over the disorder. If all is in place, the chance of significant improvement is excellent. Clinical trials As of early 2004, the National Institute on Deafness and Other Communication Disorders and the National In- stitute of Neurological Diseases and Stroke were spon- soring several clinical trials on the nature and treatment of stuttering. Information about the studies can be found at the National Institutes of Health clinical trials website: <http://www.clinicaltrials.gov/ct/search?term=stutter- ing&submit=Search>. Prognosis The prognosis for people who stutter can be very good. The American Society of Stuttering lists some fa- mous people who stutter and have proceeded to make ca- reers in which their voice is an asset. The list includes James Earl Jones, Mel Tillis, Winston Churchill, Marilyn Monroe, Carly Simon, and many more celebrities who make their living by announcing, acting, or singing. Special concerns Many childhood stutterers are not receiving adequate treatment because of poverty or financially stretched school resources. The American Institute for Stuttering offers in- formation on seeking financial resources for the treatment of stuttering, training of professionals to treat those who stutter, and additional information about stuttering. Resources BOOKS Guitar, Barry, and Theodore Peters. Stuttering: An Integrated Approach to Its Nature and Treatment, 2nd ed. Philadelphia: Lippincott, Williams & Wilkins, 1998. Kehoe, Thomas. Multifactoral Stuttering Therapy: A Guide for Persons Who Stutter, Parents, and Speech-Language Pathologists. Boulder, CO: Casa Futura Technologies, 2002. Logan, Robert. The Three Dimensions of Stuttering: Neurology, Behavior, and Emotion. London: Whurr Publishers, 1998. OTHER “How to React When Speaking with Someone Who Stutters.” Stuttering Foundation of America. April 4, 2004 (June 3, 2004). <http://206.104.238.56/brochures/ br_htr.htm>. “Stuttering.” University of Maryland Medicine. April 4, 2004 (June 3, 2004). <http://www.umm.edu/ent/stutter.htm>. ORGANIZATIONS American Speech-Language-Hearing Association. 10801 Rockville Pike, Rockville, MD 20852. (301) 897-5700 or (800) 638-8255; (301) 571-0457. actioncenter@asha.org. <http://www.nsastutter.org>. National Stuttering Association. 471 East La Palma Avenue, Suite A, Anaheim Hills, CA 92807. (714) 693-7480 or (800) 364-1677; (714) 630-7707. nsastutter@asha.org. <http://www.nsastutter.org>. Brook Ellen Hall, PhD ❙ Subacute sclerosing panencephalitis Definition Subacute sclerosing panencephalitis (SSPE) is a long- lasting (chronic) infection of the central nervous system that causes inflammation of the brain. The infection is caused by an altered form of the measles virus. The symp- toms appear years after the initial infection, following re- activation of the latent virus. LetterS.qxd 10/1/04 11:09 AM Page 812 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS 813 Subacute sclerosing panencephalitis Key Terms Antibody A special protein made by the body’s immune system as a defense against foreign mate- rial (bacteria, viruses, etc.) that enters the body. It is uniquely designed to attack and neutralize the spe- cific antigen that triggered the immune response. Encephalitis Inflammation of the brain, usually caused by a virus. The inflammation may interfere with normal brain function and may cause seizures, sleepiness, confusion, personality changes, weak- ness in one or more parts of the body, and even coma. Seizure A sudden attack, spasm, or convulsion. Description SSPE is one of three types of encephalitis that can occur following infection with the measles virus. The other forms are an acute (sudden appearance of symp- toms) form that is typically associated with the rash that forms during the measles infection. The other form of SSPE affects the myelin sheath surrounding nerve cells, and is likely part of an autoimmune reaction. SSPE develops when the measles virus, which is still present but is in an inactive (or latent) form, is reactivated. The appearance of symptoms typically leads to a disease that last from one to three years. The disease is also known as subacute sclerosing leukencephalitis and Dawson’s encephalitis. Demographics Children and young adults are primarily affected with SSPE. Males are also more affected than females, with a male-to-female ratio of 4:1. As well, there is a geograph- ical component to the infection, with those in rural areas being much more susceptible (approximately 85% of cases arise in rural environments). Since the measles vac- cine has been introduced, the disease has become rare in many areas of the globe, particularly the western world (about one in 1,000,000 people). Fewer than 10 cases per year occur in the United States. However, in the Middle East and India the incidence of the disease remains high (over 20 cases per 1,000,000 people). Causes and symptoms The disease is caused by the reactivated form of a mu- tated measles virus. The inactive form of the virus can be present in the body for up to 10 years following the initial bout of measles before the symptoms of SSPE develop. While normally the measles virus does not infect the brain, the mutated virus is capable of invading the brain. When symptoms do develop, motor skills and mental faculties become progressively worse. Initial symptoms include a change in behavior, irritability, memory loss, and difficulty in forming thoughts and solving problems. Sub- sequently, a person can experience involuntary movements and seizures (also known as myoclonic spasms), loss of the ability to walk, difficulty speaking, and swallowing difficulty (dysphagia). Blindness can occur. In the final stages of the disease, a patient with SSPE may become mute and can lapse into a coma. Monitoring the electrical activity of the brain has shown that SSPE causes disruptions that are consistent with the deterioration of the central nervous system. These changes tend to occur in stages, and so can be diagnostic of the progression of the disease. A different pattern of brain deterioration has been detected using the techniques of computed tomography and magnetic resonance im- aging. However, this latter pattern is not yet refined enough for diagnostic use. Examination of brain tissue has shown that the disease is associated with the deterioration of the cortex and loss of white matter. Diagnosis SSPE is diagnosed based on the early symptoms, de- tection of antibodies to the measles virus, detection of pro- tein in the spinal fluid, and the information gained from monitoring of the brain. Treatment team Initially, the family physician and local clinicians provide care. With the progression of the disease, spe- cialists such as neurologists can become involved. Nurses are critical for those patients with advanced disease. Fam- ily and friends are an important source of care throughout the disease. Treatment There is no cure for SSPE. In the past, the primary means of treatment included therapy to curb seizures and the use of supportive measures such as feeding tubes when swallowing becomes difficult. During the 1990s, evidence accumulated in the medical literature to support the con- tention that SSPE can be stabilized and the progressive de- terioration can be slowed by drug therapy. The drugs used lessen the damage inflicted by the immune system (im- munomodulators such as the interferons), or attack the virus. The drugs used are an orally administered form of the antiviral drug inosine pranobex (oral isoprinosine), LetterS.qxd 10/1/04 11:09 AM Page 813 814 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS Subdural hematoma oral isoprinosine combined with interferon alpha or beta, and interferon alpha combined with intravenous ribavirin (another antiviral). In particular, the isoprinosine-inter- feron alpha combination has been reported to produce up to a 50% rate of remission or improvement in symptoms. As promising as these results are, no controlled studies have yet been performed. Therefore, the treatments are not typically used. Recovery and rehabilitation As SSPE is almost always fatal, emphasis is placed upon maintaining comfort, rather than rehabilitation. Clinical trials There were no clinical trials in progress or planned in the United States as of January 2004. However, organ- izations such as the National Institute for Neurological Diseases and Stroke undertake and fund research aimed at furthering the understanding of the causes, prevention, and treatment of subacute sclerosing panencephalitis and re- lated diseases. Prognosis Without treatment, death usually occurs within one to three years following the first appearance of symptoms. Treatment with immunomodulators and antiviral drugs has achieved remission of the disease in some cases. As well, remission can occur spontaneously in approximately 5% of cases. Resources BOOKS Icon Health Publications. The Official Parent’s Sourcebook on Subacute Sclerosing Panencephalitis: A Revised and Updated Directory for the Internet Age. San Diego: Icon Grp. Int., 2002. PERIODICALS Forcic, D., M. Baricevic, R. Zgorelec, et al. “Detection and characterization of measles virus strains in cases of suba- cute sclerosing panencephalitis in Croatia.” Virus Research (January 1999): 51–56. Hayashi, M., N. Arai, J. Satoh, et al. “Neurodegenerative mechanisms in subacute sclerosing panencephalitis.” Journal of Child Neurology (October 2002): 725–730. OTHER National Library of Medicine. “Subacute Sclerosing Panencephalitis.” MEDLINE plus. <http://www.nlm.nih.gov/medlineplus/ency/arti- cle/001419.htm> (January 25, 2004). “Subacute Sclerosing Panencephalitis Information Page.” National Institute of Neurological Disorders and Stroke. <http://www.ninds.nih.gov/health_and_medical/ disorders/subacute_panencephalitis_.htm> (January 26, 2004). ORGANIZATIONS National Institute for Neurological Diseases and Stroke (NINDS). 6001 Executive Boulevard, Bethesda, MD 20892. (301) 496-5751 or (800) 352-9424. <http://www.ninds.nih.gov>. National Organization for Rare Disorders. 55 Kenosia Avenue, Danbury, CT 06813-1968. (203) 744-0100 or (800) 999- 6673; Fax: (203) 798-2291. orphan@rarediseases.org. <http://www.rarediseases.org>. Brian Douglas Hoyle, PhD Subarachnoid hemorrhage see Aneurysm Subcortical arteriosclerotic encephalitis see Binswanger disease ❙ Subdural hematoma Definition A subdural hematoma is a pooling of blood between the dura, which is a leathery membrane just under the skull, and the brain itself. Subdural hematomas usually occur fol- lowing a head trauma that breaks the blood vessels that sur- round the brain. The pressure of the accumulated blood on the brain can cause a variety of symptoms including prob- lems with speech, vision, or even a loss of consciousness. Description The bony skull encases the brain, protecting it from external damage. Between the skull and the brain itself is a tough leathery tissue, called the dura. This dura serves two purposes, forming a second layer of protection around the brain and providing vasculation that nourishes the brain with blood and spinal fluid. During a severe blunt head trauma, the bridging blood vessels that connect the dura to the skull may tear because of shear forces to the head. The broken vessels bleed into the space between the skull and the dura. This pooling of blood puts pressure on the brain, and it swells in response. Because the skull creates a de- fined volume, there is no extra room for the brain to swell and therefore, parts of the brain become compressed. This usually has neurological consequences including visual problems, speech dysfunction, and loss of consciousness. The term subdural hematoma has a variety of syn- onyms including SDH, subdural hemorrhage, and blood clot on the brain. Physicians may use the adjectives acute, subacute, and chronic to describe the time course and vol- ume of blood in subdural hematomas. Acute describes subdural hematomas that gather a large amount of blood LetterS.qxd 10/1/04 11:09 AM Page 814 GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS 815 Subdural hematoma Nuclear magnetic resonance image of the head of a person suffering from a subdural hematoma. The two elongated white areas on the left side of the brain represent the blood that has been lost into the space between the brain and the skull. (Hammersmith Hospital Medical School / Photo Researchers, Inc.) quickly. Subacute refers to subdural hematomas that occur between three and seven days following an injury to the head. In these patients, the blood clots will liquefy and in some cases the various cellular components of the blood clots will form layers that can be visualized using com- puterized tomography (CT). Chronic usually refers to sub- dural hematomas that produce symptoms two to three weeks following an injury. In these hematomas, the blood clot has become mostly blood serum. Additionally, sub- dural hematomas are classified as simple or complicated. About half of all cases are simple, which implies that there is no laceration or contusion in the brain. In complicated SDH, the brain has suffered some sort of traumatic injury. Demographics SDH can happen to anyone who experiences a head trauma. In the United States, between 15% and 30% of pa- tients suffering from head injuries have SDH. About half of the cases of SDH are simple SDH. The other half of the cases involves other complications such as laceration of the brain, and the mortality rate is much greater in these in- dividuals. SDH is more common in people older than 60 because their blood vessels are more fragile than those in younger people. SDH is also associated with child abuse. People with blood disorders, such as hemophiliacs, people on anticoagulants, and alcoholics, are at higher risk for de- veloping subdural hematomas. Causes and symptoms Subdural hematomas are most often caused by head trauma. Rarely, they can occur spontaneously, especially in elderly persons. Often the person will lose conscious- ness following the trauma, but SDH can occur when the person has remained conscious. Signs indicating the pres- ence of SDH include headaches, dizziness, nausea, pupil dilation, slurred speech, and weakness in the limbs. More severe symptoms include loss of consciousness, disorien- tation, amnesia, trouble with breathing, or even coma. Diagnosis Diagnosis of an acute or chronic subdural hematoma is most often accomplished by using a computerized to- mography (CT) scan, which is a specialized x ray. The SDH appears as a white crescent shape that lies along the skull. In subacute SDH, the shape of the pooled blood looks more lens-like and magnetic resonance imaging (MRI) is recommended to distinguish it from an epidural hematoma. Treatment In many cases, small subdural hematomas may be treated with observation and a series of CT scans to ensure that the blood is reabsorbing and not becoming calcified. In more severe cases, surgical intervention is necessary. The surgeon will open the skull in a procedure known as a craniotomy and remove the blood clot to release the pressure on the brain. The clot is removed with suction and irrigation. Recovery and rehabilitation Following surgical removal of a subdural hematoma, a patient will most likely need to remain in the intensive care unit for a period of time. Diuretics to decrease swelling of the brain and anticonvulsants to prevent seizures will be administered. Some of the complications associated with surgery are swelling of the brain, infec- tion, seizures, memory loss, headache, difficulty con- centrating, and chronic SDH. In about 50% of the cases, a hematoma may recur following surgery. Prognosis The prognosis for someone who has suffered a sub- dural hematoma depends on the size and severity of the blood clot. Acute SDH may have very high rates of death LetterS.qxd 10/1/04 11:09 AM Page 815 [...]... Box 1586, Longview, TX 75 60 6-1 586 (903) 23 6 -7 079 or (800) ASAP -2 8 2 ( 27 2- 72 8 2); Fax: (903) 75 7- 7 456 info@asap.org National Institute for Neurological Disorders and Stroke P.O Box 5801, Bethesda, MD 20 824 (301) 49 6-5 76 1 or (800) 3 5 2- 9 424 Antonio Farina, MD, PhD Systemic lupus erythematosus see Lupus GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS T S Tabes dorsalis... American Epilepsy Society 3 42 North Main Street, West Hartford, CT 061 17 -2 5 07 (860) 58 6 -7 505 Epilepsy Foundation of America 4351 Garden City Drive, Landover, MD 20 78 5- 72 2 3 (800) 3 3 2- 1000 International League Against Epilepsy Avenue Marcel Thiry 20 4, B- 120 0, Brussels, Belgium + 32 (0) 2 77 4 95 47; Fax: + 32 (0) 2 77 4 9690 ... Radiology 22 0, no 1 (July 20 01): 20 8 21 2 WEBSITES NINDS Tethered Spinal Cord Syndrome Information Page National Institute of Neurological Disorders and Stroke ORGANIZATIONS National Organization for Rare Disorders (NORD) P.O Box 1968 (55 Kenosia Avenue), Danbury, CT 0681 3-1 968 20 3 -7 4 4-0 100 or 80 0-9 99-NORD (6 673 ); Fax: 20 3 -7 9 822 91... Academy of Child and Adolescent Psychiatry (AACAP) 3615 Wisconsin Avenue NW, Washington, DC 20 01 6-3 0 07 (20 2) 96 6 -7 300; Fax: (20 2) 966 -2 8 91 National Institute of Neurological Disorders and Stroke (NINDS) 9000 Rockville Pike, Bethesda, MD 20 8 92 (301) 49 6-5 75 1 or (800) 3 5 2- 9 424 National Organization for Rare Disorders (NORD) P O Box 1968, Danbury, CT 0681 3-1 968... 0681 3-1 968 (20 3) 74 4-0 100 or (800) 999-NORD; Fax: (20 3) 79 8 -2 2 91 orphan@rarediseases.org WE MOVE—Worldwide Education and Awareness for Movement Disorders 20 4 West 84th Street, New York, NY 10 024 (21 2) 87 5-8 389 or (800) 4 3 7- MOV2 wemove@wemove.org Rebecca J Frey, PhD bone that houses the lower portion of the medulla oblongata, the structure that links the. .. muscular dystrophy) GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS Bolus A mass of a substance to be swallowed Esophagus The tube leading from the back of the mouth, down the throat, and into the stomach Larynx The “voice box,” located between the pharynx (upper area of the throat) and the trachea (windpipe) Peristalsis Waves of involuntary muscle contraction and relaxation Pharynx The part of the airway that... Toronto: Firefly Books, 20 01 OTHER “Ethosuximide Oral.” Medline Plus (May 1, 20 04) “Zarontin.” RxMed (May 1, 20 04) GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS 8 17 Swallowing disorders ORGANIZATIONS... include the oral phase, the pharyngeal phase, and the esophageal phase Oral refers to the mouth; pharyngeal refers to the pharynx (the area of the airway at the back of the mouth, and leading to the esophagus and the lungs); esophageal refers to the esophagus (the tube passageway between the mouth and the stomach) The oral phase refers to the aspects of swallowing that rely on intact mouth functioning The. .. TSCS, the flaw occurs most often along the lower (lumbar) portion of the spinal column and cord Cases of tethered cord in the cervical and thoracic regions of the spinal column are known but are extremely rare The developmental flaw causes soft tissues of the spinal column to grow into the hollow containing the spinal cord and to attach to the spinal cord, anchoring it at GALE ENCYCLOPEDIA OF NEUROLOGICAL. .. adulthood, depending on the time of tethering, degree of stretching of the spinal cord, and severity of damage to the nerves of the cord The next steps in diagnosis of TSCS are taking x-ray images of the spine to detect bone abnormalities, followed by the application of diagnostic neuro-imaging by means of MRI (magnetic resonance imaging) to produce threedimensional images of the spinal column and spinal . <http://www.rxmed.com/b.main/ b2.pharmaceutical/b2.1.monographs/ CPS- %20 Monographs/CPS- %20 (General %20 Monographs- %20 Z)/ZARONTIN.html> (May 1, 20 04). LetterS.qxd 10/1/04 11:09 AM Page 8 17 818 GALE ENCYCLOPEDIA OF NEUROLOGICAL. Parent’s Guide. Bethesda, MD: Woodbine House, 1996. ORGANIZATIONS The Sturge-Weber Foundation. PO Box 418, Mount Freedom, NJ 079 70. (800) 6 27 -5 4 82 or ( 973 ) 89 5-4 445. Fax: ( 973 ) 89 5-4 846. swfoffice@aol.com actioncenter@asha.org. <http://www.nsastutter.org>. National Stuttering Association. 471 East La Palma Avenue, Suite A, Anaheim Hills, CA 928 07. (71 4) 69 3 -7 480 or (800) 36 4-1 677 ; (71 4) 63 0 -7 7 07. nsastutter@asha.org. <http://www.nsastutter.org>. Brook