Instituto Superior de Cincias da Informao e da Administrao

Formao Especializada em Educao Especial

Cadeira: Neuropsicologia das Dificuldades de Aprendizagem

Professor:

Maria de Ftima Figueiredo

Caderno V: DEMNCIAS

Neurological Disorder Resources

Alzheimer's Disease Epilepsy Neuro-oncology Aphasia Encephalitis Neuroimmunology Bell's Palsy Huntington's Disease Neuro-otology Sleep Disorders Creutzfeldt-Jakob Disease Neuromuscular Disorders Pain Tourette Syndrome

Pediatric Neurology Phobia

Parkinson's Disease and other movement disorders

Alzheimer's Disease

Aphasia

Alzheimer Association Alzheimer's Disease - Doctor's Guide Alzheimer's Disease - ScienceWeek Focus Report Alzheimer's: Few Clues on the Mysteries of Memory Alzheimer's Disease - American Health Assistance Foundation Alzheimer's Disease Unraveling the Mystery Alzheimer's Disease Fact Sheet Alzheimer's Disease - American Federation for Aging Research Alzheimer's Disease Education and Referral (ADEAR) Center Alzheimer's Disease Bibliography for Children and Teens Alzheimer's Disease Neuroscience for Kids Alzheimer's Diease and Plaques - Soc. Neuroscience

Aphasia - Nat. Inst. on Deafness and Other Communication Disorders Aphasia - Nat. Inst. of Neurological Disorders and Stroke Aphasia - Amer. Speech-LanguageHearing Assoc. National Aphasia Association Aphasia - eMedicine

Creutzfeldt-Jakob Disease (CJD)

Blood Recall/Withdrawal - CJD

BSE -- Bovine Spongiform Encephalopathy ("Mad Cow Disease") CJD - National Center for Infectious Diseases CJD Voice Creutzfeldt-Jakob Disease Foundation, Inc. Official Mad Cow Disease Home Page Creutzfeldt-Jakob Disease - NINDS Creutzfeldt-Jakob Disease - Mayo Clinic

Cerebrovascular Disease Encephalitis

Aneurysm Information Project National Stroke Association NINDS Stroke Information Guide Stroke Diagnosis - Internet Stroke Center Stroke - National Women's Health Information Center Preventing Stroke

Encephalitis - eMedicine Encephalitis (Arbovirus) - CDC Encephalitis - Mayo Clinic Encephalitis (Nemours Foundation) Encephalitis Information Resource

Epilepsy

Huntington's Disease

Epilepsy - Neuroscience for Kids EpiCentre Epilepsy.com Epilepsy - World Health Organization (WHO) Epilepsy Treatment - WHO Epilepsy History- WHO Epilepsy - Social/Economic WHO Epilepsy Fact Sheet - NINDS, NIH Epilepsy Foundation of America Frequently Asked Questions about Epilepsy Epilepsy - Genes may build the

Index - Internet Resources for Huntington's Disease Huntington's Disease Resources Huntington's Disease Society of America Huntington's Disease - Nat. Inst. of Neurological Disorders and Stroke Huntington's Disease - Mayo Clinic Huntington's Disease - WeMove Huntington's Disease - American Speech-Language-Hearing Association Huntington's Disease Genetics - Soc. Neuroscience

road in treatment

Pain

American Council for Headache Education Migraine - Doctor's Guide to the Internet Migraine Resource Center Trigeminal Neuralgia Association Homepage Trigeminal Neuralgia International Radiosurgery Assoc. Intern. Assoc. for the Study of Pain

Parkinson's Disease and Other Movement Disorders

American Parkinson Disease Association Michael J. Fox Foundation Dystonias - National Institutes of Health National Parkinson Foundation Parkinson's Disease- Hope Through Research (NINDS, NIH) Parkinson's Disease Foundation Biology of Parkinson's Disease Science-Week Focus Report The Parkinson Alliance Parkinson's Disease: New Treatments Slow Onslaught of Symptoms Parkinson's Disease - Medline Plus Young Parkinson's Information and Referral Center - American Parkinson Disease Association Restless Legs Syndrome Foundation We Move Alpha-Synuclein and Parkinson's Disease - Soc. for Neuroscience Dystonia - Soc. for Neuroscience Parkinson's and Dopamine - Soc. Neuroscience

Tourette Syndrome

Tourette Syndrome Neuroscience for Kids Tourette Syndrome InformationNINDS, NIH Tourette Syndrome Home Page Tourette's Syndrome and Dopamine

Neuro-immunology

Multiple Sclerosis - Neuroscience for Kids site Multiple Sclerosis Central Multiple Sclerosis Information for Physician and Medical Students Multiple Sclerosis Society of America MS page by Aapo Halko Myelin Project

Basic Information About Parkinson's Disease

The Disease
Parkinson's disease is a progressive disorder of the central nervous system affecting more than 1.5 million people in the United States. Clinically, the disease is characterized by a decrease in spontaneous movements, gait difficulty, postural instability, rigidity and tremor. Parkinson's disease is caused by the degeneration of the pigmented neurons in the Substantia Nigra of the brain, resulting in decreased dopamine availability. The major symptoms of the disease were originally described in 1817 by an English physician, Dr. James Parkinson, who called it "Shaking Palsy." Only in the 1960's, however, pathological and biochemical changes in the brain of patients were identified, opening the way to the first effective medication for the disease. Incidence Men and women alike are affected. The frequency of the disease is considerably higher in the over-60 age group, even though there is an alarming increase of patients of younger age. In consideration of the increased life expectancy in this country and worldwide, an increasing number of people will be victims of Parkinson's disease. Treatment Administration of the drug levodopa has been the standard treatment for Parkinson's disease. Once it reaches the brain, levodopa is converted to dopamine which replaces the same substance not present in sufficient amounts in Parkinson's patients. Treatment with levodopa does not, however, prevent the progressive changes of the brain typical of Parkinson's disease. The drug may also produce side effects in some people, due to its change to dopamine before reaching the brain. The simultaneous administration with levodopa of substances inhibiting this change allows a higher concentration of levodopa to reach the brain and also considerably decreases the side effects. Some new drugs have recently been approved offering a wider choice of medications for the patient, while others are under investigation in this country and overseas in an effort to obtain better therapeutic results with fewer side effects. The American Parkinson Disease Association, Inc., founded in 1961, has sought to "Ease the Burden and Find the Cure" for this disease through research, patient and family support and education. Education Our education program provides information and resources to patients, their families, friends, doctors and other medical professionals and enhances public education and awareness of the disease.

A set of nine manuals dealing with symptoms and medications, support, physiotherapy, speech problems and equipment to be used in the home is available free of charge. Some of the manuals have already been translated or are being translated into other languages. Educational supplements dealing with specific subjects related to Parkinson's disease are periodically issued. A quarterly newsletter which focuses upon the latest developments in research and treatment of the disease is also mailed to more than 200,000 addresses. APDA also publishes an annual International Parkinson's Disease Resource Guide with the purpose of providing a worldwide exchange of information among the people afflicted by this disease and their supporting organizations. Support Recognizing the devastating effects of the illness upon the patient and the family, a support program was initiated to motivate the patients in maximizing strengths, minimizing impediments and achieving and maintaining full potential. The American Parkinson Disease Association, Inc. sponsors 65 chapters and more than 250 affiliated support groups which provide education, counseling, assistance and referrals throughout the United States. The chapters also sponsor regional symposia and conferences in cooperation with the Information and Referral Centers. To provide professional support to the educational, counseling and referral needs of the chapters, the support groups and the Parkinsonians at large, the American Parkinson Disease Association, Inc. funds 58 Information and Referral Centers from coast to coast. Their functions are to educate, counsel and refer patients to medical professionals, increase awareness of the incidence of the disease and to establish Parkinson's disease chapters and support groups in their areas. More than $2 million is allocated annually to maintain these centers. Research The research program funds five types of grants: 1. The Roger C. Duvoisin, MD Fellowship, established in 2002, carries a stipend of $80,000 for one year and is intended to support established researchers in Parkinson's disease studies. 2. The Cotzias Fellowship, awarded to young neurologists for three consecutive years, with yearly stipends of $70,000. 3. Research Grants of up to $50,000 a year are awarded to scientists working on Parkinson's disease. 4. Post-Doctoral Research Fellowships of $35,000 a year.

5. Medical Student Fellowships in the amount of $4,000 each per year. APDA is also funding eight Centers for Advanced Research in institutions across the country at a level of $100,000 a year for five consecutive years. More than 2.3 million dollars is allocated annually to support research projects Fund Raising and Public Relations Fund raising is of paramount importance to support our ambitious programs. Proceeds from contributions, direct mail, special events sponsored by the national office and chapters, along with a bequest program and gifts from foundations and corporations are used for funding our programs. A series of public service announcements by prominent individuals are distributed periodically. Our awareness campaigns and symposia throughout the United States have helped increase our visibility. Without public support we would not be able to fund our outstanding research projects and unique education programs. Since APDA is a privately funded association, we would like to thank all who have invested in our cause and look forward to a continued and growing relationship in our quest to "Ease the Burden and Find The Cure."

Epilepsy
Contents of this Page
Types of Epilepsy Causes Diagnosis Treatment Famous People Organizations

Epilepsy is NOT contagious and people with

epilepsy are NOT "crazy." The word "epilepsy" comes from a Greek word meaning "to possess, seize or hold." Because the brain uses electrochemical energy, any disruption of the electrical processes in the brain may cause abnormal functioning. Unfortunately, this is what happens during epilepsy: neurons in the cerebral hemispheres misfire and create abnormal electrical activity. People with epilepsy have seizures that are a bit like an electrical brainstorm. The seizure prevents the brain from:

interpreting and processing incoming sensory signals (like visual, somatosensory and auditory information). controlling muscles. That is why people with epilepsy may fall down and twitch.

Types of Epilepsy
There are many types of epilepsy. Each type of epilepsy has different behavioral effects and is treated with different methods. In some cases, people know they are about to have a seizure because they see or hear something, or feel dizzy, nauseous, or "strange." This is called an aura. An aura can act as an "early warning system" telling a person that a seizure is about to happen. The person can therefore minimize a possible injury by taking precautions such as sitting down. 1. Generalized seizures - uncontrollable discharge of neurons on BOTH sides of the brain. This is the most common type of epilepsy. The seizure starts in one area of the brain and spreads across the brain. These seizures produce muscle twitches, convulsions and loss of consciousness. People with this type of epilepsy do not remember having a seizure. There are several types of generalized seizures:

Tonic-clonic ("grand mal") seizure - This seizure occurs when there is a massive discharge of neurons on both cerebral hemispheres. The body becomes rigid and there is also jerking of the body. "Tonic-clonic" means "stiffness-violent." "Grand mal" means "great sickness." o Absence ("petit mal") seizure - This seizure is nonconvulsive. However, a person may become unaware of his or her surroundings and may stare off in space or freeze. This seizure lasts only 5-30 seconds. o Myoclonic seizure - This seizure involves the motor cortex and causes twitching or jerking of certain parts of the body. o Atonic seizure - This seizure is characterized by the loss of muscle tone and causes a person to fall down. o Status epilepticus - This seizure is characterized by frequent, longlasting seizures without regaining consciousness between attacks. This condition requires immediate medical attention. 2. Partial Seizures - This type of seizure is characterized by abnormal electrical activity involving only a small part of the brain. Sometimes a partial seizure can spread to the whole brain.
o

Two types of partial seizures are:

o

Simple partial seizures (also called "Jacksonian" or "focal" seizures) - Short-lasting seizures without loss of consciousness. People with these kinds of seizures often see, hear or smell something strange. Also, only part of the body may jerk. Complex partial (psychomotor) seizures - A seizure with a change, not a loss, in consciousness. People may hear or see things or memories may resurface. Feelings of deja vu may also occur.

Causes
Many (50-70%) cases of epilepsy have no known cause. For the remaining cases, there are many other events that may cause epileptic seizures:

Head injuries, such as a car accident or a fall. Brain tumor Stroke Arteriosclerosis (fatty plaque build-up in arteries) Brain injury before birth caused by infection or lack of oxygen to the brain Infection, such as meningitis or encephalitis

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Brain damage resulting from these events may cause a "scar" on the brain. This is where an epileptic seizure starts. At this time it is not known why a scar starts a seizure. There may be a genetic link for some types of epilepsy, but this is usually NOT the case. Sometimes a seizure will be started ("triggered") by:

stress lack of sleep flashing lights or sounds (like from a video game or TV) low blood sugar

Diagnosis
A doctor will want to find out several things before a diagnosis of epilepsy is made. The doctor may want to know the answers to the following questions:

How often do the seizures occur? When was the first seizure? Was there a head injury? What are the seizures like? Were there any unusual sensations (smells, sounds, lights)? Is there any memory of the seizures?

An electroencephalogram (EEG) is often used to help in the diagnosis of epilepsy. The EEG of people with epilepsy often shows large spikes. Sometimes the EEG must be recorded for a whole day in the hospital or at home because a short test does not always pick up the abnormal activity. However, the EEG does not always work because about 5% of people without epilepsy have "abnormal" EEG activity and about 20% of people with epilepsy have normal EEG activity. Brain imaging methods (magnetic resonance imaging [MRI] or computer tomography [CT] scanning) may also be used to find the location of a scar or damaged brain tissue. Sometimes positron emission tomography [PET] is used to examine brain blood flow.

Treatment for Epilepsy

Drugs

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Antiepileptic Drugs
Chemical Name Common Trade Name

Carbamazepine Tegretol Clobazam Clonazepam Diazepam Divalproex sodium Ethosuximide Gabapentin Lamotrigine Phenobarbital Phenytoin Valproic acid Frisium Rivotril Valium Depakote Zarontin Neurontin Lamictal many names Dilantin Depakene

Sometimes seizures stop without treatment. Many people take antiepileptic (also called "anticonvulsant") drugs to control seizures. These drugs, however, do not cure the disorder. Rather, these drugs control the symptoms and are effective in 60-80% of the cases. Antiepileptic drugs work by reducing the abnormal firing of cortical neurons. These drugs may change the activity of neurotransmitters responsible for seizures or alter the way ions flow in and out of neurons. Unfortunately, many of these drugs have side effects such as drowsiness, dizziness and nausea so doctors must balance these undesirable effects with seizure control. It is also important to remember that different drugs are used to treat different types of seizures.

Surgery
When medication fails and the area of the brain where the seizure occurs is known, surgery may be performed to treat epilepsy. There are several types of surgery that have been used:

Temporal Lobe Surgery - This is performed to remove brain tissue where the epileptic seizure starts. This type of surgery often removes part of the cortex of the temporal

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lobe, hippocampus and amygdala. [Brain mapping during surgery] Corpus Callosotomy ("Split Brain" operation) The corpus callosum is cut to separate the right and left cerebral hemispheres. This procedure is done to prevent the spread of the seizure from one side of the brain to the other. Hemispherectomy - One cerebral hemisphere is removed. The procedure is not performed very often. Children who have this surgery CAN function quite well although they often have trouble using their arm on the side of the body opposite to the surgery. In some surgeries, only specific lobes of the brain are removed.

Hemispherectomy

Experimental Treatments
Several treatments for epilepsy are still under investigation. How and if they work is still controversial.

Ketogenic Diet - a high fat, low protein/carbohydrate diet Biofeedback - people trained to control EEG patterns to shorten seizures Counterstimulation Exercise Relaxation Techniques Vagus Nerve Stimulation - an electrical stimulator is implanted to stimulate the vagus nerve (cranial nerve X) Vitamin and Mineral Supplements

Epilepsy is a fairly common neurological disorder. It occurs in about 1 in every 100-200 people. Throughout history there have been many famous people who have had epilepsy:

Famous People with Epilepsy

Julius Caesar - Roman Statesman (100-44 B.C.) George Frederick Handel Napoleon Bonaparte Emperor of France (17691821) Vincent van Gogh - Painter

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- Composer (1685-1759) Fyodor Dostoevski Writer (1821-1881) Peter the Great - Russian Czar (1682-1725) Did you know?

(1853-1890) Pius IX - Pope (17921878) Lord Byron - Poet (17881824)

Saint Valentine is the patron saint of epilepsy.

For more information on epilepsy, see:

An Epilepsy Education EpiCentre Epilepsy and Everyone Epilepsy and GABA Epilepsy: Taming the Seizures, Dispelling the Myths - from the U.S. FDA Epilepsy - World Health Organization (WHO) Epilepsy Treatment - WHO Epilepsy History- WHO Epilepsy - Social/Economic - WHO Epilepsy Fact Sheet- from NINDS, NIH Epilepsy Surgery - includes images of EEG and MRI; photographs during surgery

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Introduction Huntington's disease

In 1872, the American physician George Huntington wrote about an illness that he called "an heirloom from generations away back in the dim past." He was not the first to describe the disorder, which has been traced back to the Middle Ages at least. One of its earliest names was chorea,* which, as in "choreography," is the Greek word for dance. The term chorea describes how people affected with the disorder writhe, twist, and turn in a constant, uncontrollable dance-like motion. Later, other descriptive names evolved. "Hereditary chorea" emphasizes how the disease is passed from parent to child. "Chronic progressive chorea" stresses how symptoms of the disease worsen over time. Today, physicians commonly use the simple term Huntington's disease (HD) to describe this highly complex disorder that causes untold suffering for thousands of families. In the United States alone, about 30,000 people have HD; estimates of its prevalence are about 1 in every 10,000 persons. At least 150,000 others have a 50 percent risk of developing the disease and thousands more of their relatives live with the possibility that they, too, might develop HD. Until recently, scientists understood very little about HD and could only watch as the disease continued to pass from generation to generation. Families saw the disease destroy their loved ones' ability to feel, think, and move. In the last several years, scientists working with support from the National Institute of Neurological Disorders and Stroke (NINDS) have made several breakthroughs in the area of HD research. With these advances, our understanding of the disease continues to improve. This brochure presents information about HD, and about current research progress, to health professionals, scientists, caregivers, and, most important, to those already too familiar with the disorder: the many families who are affected by HD. What Causes Huntington's disease?

HD results from genetically programmed degeneration of nerve cells, called neurons,* in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. Specifically affected are cells of the basal ganglia, structures deep within the brain that have many important functions, including coordinating movement. Within the basal ganglia, HD especially targets neurons of the striatum, particularly those in the

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caudate nuclei and the pallidum. Also affected is the brain's outer surface, or cortex, which controls thought, perception, and memory. How is HD Inherited?

HD is found in every country of the world. It is a familial disease, passed from parent to child through a mutation or misspelling in the normal gene. A single abnormal gene, the basic biological unit of heredity, produces HD. Genes are composed of deoxyribonucleic acid (DNA), a molecule shaped like a spiral ladder. Each rung of this ladder is composed of two paired chemicals called bases. There are four types of basesadenine, thymine, cytosine, and guanineeach abbreviated by the first letter of its name: A, T, C, and G. Certain bases always "pair" together, and different combinations of base pairs join to form coded messages. A gene is a long string of this DNA in various combinations of A, T, C, and G. These unique combinations determine the gene's function, much like letters join together to form words. Each person has about 30,000 genesa billion base pairs of DNA or bits of information repeated in the nuclei of human cellswhich determine individual characteristics or traits. Genes are arranged in precise locations along 23 rod-like pairs of chromosomes. One chromosome from each pair comes from an individual's mother, the other from the father. Each half of a chromosome pair is similar to the other, except for one pair, which determines the sex of the individual. This pair has two X chromosomes in females and one X and one Y chromosome in males. The gene that produces HD lies on chromosome 4, one of the 22 non-sex-linked, or "autosomal," pairs of chromosomes, placing men and women at equal risk of acquiring the disease. The impact of a gene depends partly on whether it is dominant or recessive. If a gene is dominant, then only one of the paired chromosomes is required to produce its called-for effect. If the gene is recessive, both parents must provide chromosomal copies for the trait to be present. HD is called an autosomal dominant disorder because only one copy of the defective gene, inherited from one parent, is necessary to produce the disease. The genetic defect responsible for HD is a small sequence of DNA on chromosome 4 in which several base pairs are repeated many, many times. The normal gene has three DNA bases, composed of the sequence CAG. In people with HD, the sequence abnormally repeats itself dozens of times. Over time and with each successive generationthe number of CAG repeats may expand further.

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Each parent has two copies of every chromosome but gives only one copy to each child. Each child of an HD parent has a 50-50 chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it to subsequent generations. A person who inherits the HD gene, and survives long enough, will sooner or later develop the disease. In some families, all the children may inherit the HD gene; in others, none do. Whether one child inherits the gene has no bearing on whether others will or will not share the same fate. A small number of cases of HD are sporadic, that is, they occur even though there is no family history of the disorder. These cases are thought to be caused by a new genetic mutation-an alteration in the gene that occurs during sperm development and that brings the number of CAG repeats into the range that causes disease. What are the Major Effects of the Disease?

Early signs of the disease vary greatly from person to person. A common observation is that the earlier the symptoms appear, the faster the disease progresses. Family members may first notice that the individual experiences mood swings or becomes uncharacteristically irritable, apathetic, passive, depressed, or angry. These symptoms may lessen as the disease progresses or, in some individuals, may continue and include hostile outbursts or deep bouts of depression. HD may affect the individual's judgment, memory, and other cognitive functions. Early signs might include having trouble driving, learning new things, remembering a fact, answering a question, or making a decision. Some may even display changes in handwriting. As the disease progresses, concentration on intellectual tasks becomes increasingly difficult. In some individuals, the disease may begin with uncontrolled movements in the fingers, feet, face, or trunk. These movementswhich are signs of choreaoften intensify when the person is anxious. HD can also begin with mild clumsiness or problems with balance. Some people develop choreic movements later, after the disease has progressed. They may stumble or appear uncoordinated. Chorea often creates serious problems with walking, increasing the likelihood of falls. The disease can reach the point where speech is slurred and vital functions, such as swallowing, eating, speaking, and especially walking, continue to decline. Some individuals cannot recognize other family members. Many, however, remain aware of their environment and are able to express emotions.

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Some physicians have employed a recently developed Unified HD Rating Scale, or UHDRS, to assess the clinical features, stages, and course of HD. In general, the duration of the illness ranges from 10 to 30 years. The most common causes of death are infection (most often pneumonia), injuries related to a fall, or other complications. At What Age Does HD Appear?

The rate of disease progression and the age at onset vary from person to person. Adult-onset HD, with its disabling, uncontrolled movements, most often begins in middle age. There are, however, other variations of HD distinguished not just by age at onset but by a distinct array of symptoms. For example, some persons develop the disease as adults, but without chorea. They may appear rigid and move very little, or not at all, a condition called akinesia. Some individuals develop symptoms of HD when they are very youngbefore age 20. The terms "early-onset" or "juvenile" HD are often used to describe HD that appears in a young person. A common sign of HD in a younger individual is a rapid decline in school performance. Symptoms can also include subtle changes in handwriting and slight problems with movement, such as slowness, rigidity, tremor, and rapid muscular twitching, called myoclonus. Several of these symptoms are similar to those seen in Parkinson's disease, and they differ from the chorea seen in individuals who develop the disease as adults. These young individuals are said to have "akinetic-rigid" HD or the Westphal variant of HD. People with juvenile HD may also have seizures and mental disabilities. The earlier the onset, the faster the disease seems to progress. The disease progresses most rapidly in individuals with juvenile or early-onset HD, and death often follows within 10 years. Individuals with juvenile HD usually inherit the disease from their fathers. These individuals also tend to have the largest number of CAG repeats. The reason for this may be found in the process of sperm production. Unlike eggs, sperm are produced in the millions. Because DNA is copied millions of times during this process, there is an increased possibility for genetic mistakes to occur. To verify the link between the number of CAG repeats in the HD gene and the age at onset of symptoms, scientists studied a boy who developed HD symptoms at the age of two, one of the youngest and most severe cases ever recorded. They found that he had the largest number of CAG repeats of anyone studied so far nearly 100. The boy's case was central to the identification of the HD gene and at the same time helped confirm that juveniles with HD have the longest segments of CAG repeats, the only proven correlation between repeat length and age at onset.

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A few individuals develop HD after age 55. Diagnosis in these people can be very difficult. The symptoms of HD may be masked by other health problems, or the person may not display the severity of symptoms seen in individuals with HD of earlier onset. These individuals may also show symptoms of depression rather than anger or irritability, or they may retain sharp control over their intellectual functions, such as memory, reasoning, and problem-solving. There is also a related disorder called senile chorea. Some elderly individuals display the symptoms of HD, especially choreic movements, but do not become demented, have a normal gene, and lack a family history of the disorder. Some scientists believe that a different gene mutation may account for this small number of cases, bu this has not been proven. How is HD Diagnosed?

The great American folk singer and composer Woody Guthrie died on October 3, 1967, after suffering from HD for 13 years. He had been misdiagnosed, considered an alcoholic, and shuttled in and out of mental institutions and hospitals for years before being properly diagnosed. His case, sadly, is not extraordinary, although the diagnosis can be made easily by experienced neurologists. A neurologist will interview the individual intensively to obtain the medical history and rule out other conditions. A tool used by physicians to diagnose HD is to take the family history, sometimes called a pedigree or genealogy. It is extremely important for family members to be candid and truthful with a doctor who is taking a family history. The doctor will also ask about recent intellectual or emotional problems, which may be indications of HD, and will test the person's hearing, eye movements, strength, coordination, involuntary movements (chorea), sensation, reflexes, balance, movement, and mental status, and will probably order a number of laboratory tests as well. People with HD commonly have impairments in the way the eye follows or fixes on a moving target. Abnormalities of eye movements vary from person to person and differ, depending on the stage and duration of the illness. The discovery of the HD gene in 1993 resulted in a direct genetic test to make or confirm a diagnosis of HD in an individual who is exhibiting HD-like symptoms. Using a blood sample, the genetic test analyzes DNA for the HD mutation by counting the number of repeats in the HD gene region. Individuals who do not have HD usually have 28 or fewer CAG repeats. Individuals with HD usually have

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40 or more repeats. A small percentage of individuals, however, have a number of repeats that fall within a borderline region (see table 1).
Table 1

No. of CAG repeats < 28 29-34 35-39

Outcome Normal range; individual will not develop HD

Individual will not develop HD but the next generation is at risk Some, but not all, individuals in this range will develop HD; next generation is also at risk Individual will develop HD

> 40

The physician may ask the individual to undergo a brain imaging test. Computed tomography (CT) and magnetic

resonance imaging (MRI) provide excellent images of brain structures with little if any discomfort. Those with HD may show shrinkage of some parts of the brain particularly two areas known as the caudate nuclei and putamenand enlargement of fluid-filled cavities within the brain called ventricles. These changes do not definitely indicate HD, however, because they can also occur in other disorders. In addition, a person can have early symptoms of HD and still have a normal CT scan. When used in conjunction with a family history and record of clinical symptoms, however, CT can be an important diagnostic tool. Another technology for brain imaging includes positron emission tomography (PET,) which is important in HD research efforts but is not often needed for diagnosis. What is Presymptomatic Testing?

Presymptomatic testing is used for people who have a family history of HD but have no symptoms themselves. If either parent had HD, the person's chance would be 50-50. In the past, no laboratory test could positively identify people carrying the HD geneor those fated to develop HDbefore the onset of symptoms. That situation changed in 1983, when a team of scientists supported by the NINDS located the first genetic marker for HDthe initial step in developing a laboratory test for the disease. A marker is a piece of DNA that lies near a gene and is usually inherited with it. Discovery of the first HD marker allowed scientists to locate the HD gene on 20

chromosome 4. The marker discovery quickly led to the development of a presymptomatic test for some individuals, but this test required blood or tissue samples from both affected and unaffected family members in order to identify markers unique to that particular family. For this reason, adopted individuals, orphans, and people who had few living family members were unable to use the test. Discovery of the HD gene has led to a less expensive, scientifically simpler, and far more accurate presymptomatic test that is applicable to the majority of at-risk people. The new test uses CAG repeat length to detect the presence of the HD mutation in blood. This is discussed further in the next section. There are many complicating factors that reflect the complexity of diagnosing HD. In a small number of individuals with HD1 to 3 percentno family history of HD can be found. Some individuals may not be aware of their genetic legacy, or a family member may conceal a genetic disorder from fear of social stigma. A parent may not want to worry children, scare them, or deter them from marrying. In other cases, a family member may die of another cause before he or she begins to show signs of HD. Sometimes, the cause of death for a relative may not be known, or the family is not aware of a relative's death. Adopted children may not know their genetic heritage, or early symptoms in an individual may be too slight to attract attention. How is the Presymptomatic Test Conducted?

An individual who wishes to be tested should contact the nearest testing center. (A list of such centers can be obtained from the Huntington Disease Society of America at 1-800-345-HDSA.) The testing process should include several components. Most testing programs include a neurological examination, pretest counseling, and followup. The purpose of the neurological examination is to determine whether or not the person requesting testing is showing any clinical symptoms of HD. It is important to remember that if an individual is showing even slight symptoms of HD, he or she risks being diagnosed with the disease during the neurological examination, even before the genetic test. During pretest counseling, the individual will learn about HD, and about his or her own level of risk, about the testing procedure. The person will be told about the test's limitations, the accuracy of the test, and possible outcomes. He or she can then weigh the risks and benefits of testing and may even decide at that time against pursuing further testing. If a person decides to be tested, a team of highly trained specialists will be involved, which may include neurologists, genetic counselors, social workers, psychiatrists, and psychologists. This team of professionals helps the at-risk

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person decide if testing is the right thing to do and carefully prepares the person for a negative, positive, or inconclusive test result. Individuals who decide to continue the testing process should be accompanied to counseling sessions by a spouse, a friend, or a relative who is not at risk. Other interested family members may participate in the counseling sessions if the individual being tested so desires. The genetic testing itself involves donating a small sample of blood that is screened in the laboratory for the presence or absence of the HD mutation. Testing may require a sample of DNA from a closely related affected relative, preferably a parent, for the purpose of confirming the diagnosis of HD in the family. This is especially important if the family history for HD is unclear or unusual in some way. Results of the test should be given only in person and only to the individual being tested. Test results are confidential. Regardless of test results, followup is recommended. In order to protect the interests of minors, including confidentiality, testing is not recommended for those under the age of 18 unless there is a compelling medical reason (for example, the child is exhibiting symptoms). Testing of a fetus (prenatal testing) presents special challenges and risks; in fact some centers do not perform genetic testing on fetuses. Because a positive test result using direct genetic testing means the at-risk parent is also a gene carrier, at-risk individuals who are considering a pregnancy are advised to seek genetic counseling prior to conception. Some at-risk parents may wish to know the risk to their fetus but not their own. In this situation, parents may opt for prenatal testing using linked DNA markers rather than direct gene testing. In this case, testing does not look for the HD gene itself but instead indicates whether or not the fetus has inherited a chromosome 4 from the affected grandparent or from the unaffected grandparent on the side of the family with HD. If the test shows that the fetus has inherited a chromosome 4 from the affected grandparent, the parents then learn that the fetus's risk is the same as the parent (50-50), but they learn nothing new about the parent's risk. If the test shows that the fetus has inherited a chromosome 4 from the unaffected grandparent, the risk to the fetus is very low (less than 1%) in most cases. Another option open to parents is in vitro fertilization with preimplantation screening. In this procedure, embryos are screened to determine which ones carry the HD mutation. Embryos determined not to have the HD gene mutation are then implanted in the woman's uterus.

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In terms of emotional and practical consequences, not only for the individual taking the test but for his or her entire family, testing is enormously complex and has been surrounded by considerable controversy. For example, people with a positive test result may risk losing health and life insurance, suffer loss of employment, and other liabilities. People undergoing testing may wish to cover the cost themselves, since coverage by an insurer may lead to loss of health insurance in the event of a positive result, although this may change in the future. With the participation of health professionals and people from families with HD, scientists have developed testing guidelines. All individuals seeking a genetic test should obtain a copy of these guidelines, either from their testing center or from the organizations listed on the card in the back of this brochure. These organizations have information on sites that perform testing using the established procedures and they strongly recommend that individuals avoid testing that does not adhere to these guidelines. How Does a Person Decide Whether to be Tested?

The anxiety that comes from living with a 50 percent risk for HD can be overwhelming. How does a young person make important choices about longterm education, marriage, and children? How do older parents of adult children cope with their fears about children and grandchildren? How do people come to terms with the ambiguity and uncertainty of living at risk? Some individuals choose to undergo the test out of a desire for greater certainty about their genetic status. They believe the test will enable them to make more informed decisions about the future. Others choose not to take the test. They are able to make peace with the uncertainty of being at risk, preferring to forego the emotional consequences of a positive result, as well as possible losses of insurance and employment. There is no right or wrong decision, as each choice is highly individual. The guidelines for genetic testing for HD, discussed in the previous section, were developed to help people with this life-changing choice. Whatever the results of genetic testing, the at-risk individual and family members can expect powerful and complex emotional responses. The health and happiness of spouses, brothers and sisters, children, parents, and grandparents are affected by a positive test result, as are an individual's friends, work associates, neighbors, and others. Because receiving test results may prove to be devastating, testing guidelines call for continued counseling even after the test is complete and the results are known. Is There a Treatment for HD?

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Physicians may prescribe a number of medications to help control emotional and movement problems associated with HD. It is important to remember however, that while medicines may help keep these clinical symptoms under control, there is no treatment to stop or reverse the course of the disease. Antipsychotic drugs, such as haloperidol, or other drugs, such as clonazepam, may help to alleviate choreic movements and may also be used to help control hallucinations, delusions, and violent outbursts. Antipsychotic drugs, however, are not prescribed for another form of muscle contraction associated with HD, called dystonia, and may in fact worsen the condition, causing stiffness and rigidity. These medications may also have severe side effects, including sedation, and for that reason should be used in the lowest possible doses. For depression, physicians may prescribe fluoxetine, sertraline, nortriptyline, or other compounds. Tranquilizers can help control anxiety and lithium may be prescribed to combat pathological excitement and severe mood swings. Medications may also be needed to treat the severe obsessive-compulsive rituals of some individuals with HD. Most drugs used to treat the symptoms of HD have side effects such as fatigue, restlessness, or hyperexcitability. Sometimes it may be difficult to tell if a particular symptom, such as apathy or incontinence, is a sign of the disease or a reaction to medication. What Kind of Care Does the Individual with HD Need?

Although a psychologist or psychiatrist, a genetic counselor, and other specialists may be needed at different stages of the illness, usually the first step in diagnosis and in finding treatment is to see a neurologist. While the family doctor may be able to diagnose HD, and may continue to monitor the individual's status, it is better to consult with a neurologist about management of the varied symptoms. Problems may arise when individuals try to express complex thoughts in words they can no longer pronounce intelligibly. It can be helpful to repeat words back to the person with HD so that he or she knows that some thoughts are understood. Sometimes people mistakenly assume that if individuals do not talk, they also do not understand. Never isolate individuals by not talking, and try to keep their environment as normal as possible. Speech therapy may improve the individual's ability to communicate. It is extremely important for the person with HD to maintain physical fitness as much as his or her condition and the course of the disease allows. Individuals who exercise and keep active tend to do better than those who do not. A daily regimen of exercise can help the person feel better physically and mentally.

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Although their coordination may be poor, individuals should continue walking, with assistance if necessary. Those who want to walk independently should be allowed to do so as long as possible, and careful attention should be given to keeping their environment free of hard, sharp objects. This will help ensure maximal independence while minimizing the risk of injury from a fall. Individuals can also wear special padding during walks to help protect against injury from falls. Some people have found that small weights around the ankles can help stability. Wearing sturdy shoes that fit well can help too, especially shoes without laces that can be slipped on or off easily. Impaired coordination may make it difficult for people with HD to feed themselves and to swallow. As the disease progresses, persons with HD may even choke. In helping individuals to eat, caregivers should allow plenty of time for meals. Food can be cut into small pieces, softened, or pureed to ease swallowing and prevent choking. While some foods may require the addition of thickeners, other foods may need to be thinned. Dairy products, in particular, tend to increase the secretion of mucus, which in turn increases the risk of choking. Some individuals may benefit from swallowing therapy, which is especially helpful if started before serious problems arise. Suction cups for plates, special tableware designed for people with disabilities, and plastic cups with tops can help prevent spilling. The individual's physician can offer additional advice about diet and about how to handle swallowing difficulties or gastrointestinal problems that might arise, such as incontinence or constipation. Caregivers should pay attention to proper nutrition so that the individual with HD takes in enough calories to maintain his or her body weight. Sometimes people with HD, who may burn as many as 5,000 calories a day without gaining weight, require five meals a day to take in the necessary number of calories. Physicians may recommend vitamins or other nutritional supplements. In a long-term care institution, staff will need to assist with meals in order to ensure that the individual's special caloric and nutritional requirements are met. Some individuals and their families choose to use a feeding tube; others choose not to. Individuals with HD are at special risk for dehydration and therefore require large quantities of fluids, especially during hot weather. Bendable straws can make drinking easier for the person. In some cases, water may have to be thickened with commercial additives to give it the consistency of syrup or honey.

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Introduction Creutzfeldt - Jakob disease

Prion diseases are a group of rare, invariably fatal brain disorders which occur both in humans and certain animals. They first came to public attention in the mid 1980s in the form of the BSE epidemic in the United Kingdom. BSE (bovine spongiform encephalopathy) is a prion disease in cattle. Tissue from infected animals may have contaminated cattle feed, leading to the silent spread of the BSE epidemic. There is also a theory that BSE came from feed contaminated with scrapie, the long established sheep prion disease. Inevitably, concern over whether BSE could pass to humans mounted. In humans the best known of the prion diseases is Creutzfeldt-Jakob Disease (CJD), which reportedly affects around one person per million per year. In the United States this translates to 250-300 new cases per year. It is well known that CJD is very difficult to diagnose leading to speculation that the one case per million report may be incorrect. Most of the cases are "classical" or "sporadic" CJD (sCJD), occurring for no, as yet, known reason. The sporadic form accounts for approximately 85% of the cases, the familial form approximately 15%. There have also been a few cases which have occurred from contamination via medical procedures; this type is known as iatrogenic or Acquired CJD. Finally over the last few years, another type of Acquired CJD called variant (vCJD) has been identified in young people. vCJD has been linked to ingestion of beef tainted with BSE (bovine spongiform encephalopathy), most cases have occurred in the United Kingdom.

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Alzheimer's Disease
What is Alzheimer's Disease?
In 1986, President Ronald Reagan's "I don't remember," "I don't recall" responses seemed to many to be lame answers to questions about political dealings. However, it turns out that they may have been the truth. In 1994, former President Ronald Reagan announced that he had Alzheimer's disease (AD).

Ronald Reagan

Alzheimer's disease attacks the brain; it is not a normal part of aging. People with AD have a gradual memory loss and difficulties with language and emotions. The progressive loss of intellectual abilities is termed dementia. As the disease advances, the person may need help in all aspects of life: bathing, eating, and using the restroom. Because of this roundthe-clock care, families and friends of people with AD are greatly affected. The disease is irreversible and there is currently no cure.

Who Gets AD?

About 5-6% of the US population has AD or a related dementia. This means that approximately four million Americans have AD. As the population ages, the burden to caregivers and cost to society will grow. It is estimated that by 2050, 14 million people in the US will suffer from AD. Alzheimer's disease ranks fourth in the cause of death among adults. About 100,000 people die per year as a result of AD. Five to 10 percent of the population over age 65 have AD. At the age of 85 and older, about 50% have AD. Although age is a factor, research has shown that genetics also play a role. Because women tend to live longer than men, more women are affected by AD than men. Furthermore, 80% of caregivers are women, so they are also secondarily affected by the disease. Estimated Number of Cases of AD in the U.S. (millions)

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Symptoms
Memory loss, especially of recent events and newly acquired information, is perhaps the most noticeable trait of AD. The first symptoms have a gradual, subtle onset, and can be a sign of many dementias, not just AD. For example, a person may become lost in familiar surroundings, forget whether a task was done, repeat the same story, or is unable to learn new things. As the disease worsens, the person may be unable to find the right word or to make responsible decisions. One of the most painful aspects of the disease is that the person sometimes will not recognize friends or family members. Personality changes can occur, such as unusual agitation, paranoia, depression, and social withdrawal. Later, people with AD may wander, or be unable to find their way home. New research, however, has shown that a part of the brain that processes visual and spatial information may be damaged in people with AD. This may account for the problems AD patients have with orienting themselves.) Patients may also become inattentive, thus unable to care for their day-to-day bodily needs. Other parts of the brain including the basal forebrain and hippocampus, areas important for memory, are also affected by AD. Many AD sufferers die from other causes such as pneumonia. From the time of diagnosis, AD patients generally live 6-8 years, although many live for as long as 20 year after the diagnosis. The symptoms of AD vary tremendously from person to person, but in time, each person who has AD will experience worsening symptoms. Many of the behavioral changes associated with AD - depression, paranoia, and delusions - can be helped with medication. However, there is no cure for AD, although some treatments hold promise.

Areas of the brain affected by AD

A = Cerebral Cortex B = Basal Forebrain C = Hippocampus Image courtesy of the NIA Illustrator: Lydia Kibiuk

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A Closer Look at an AD Brain

The microscopic changes that occur in the brain of a person with AD were first noted by German neurologist Alois Alzheimer in 1906. He performed an autopsy on a woman who had become more and more confused in the years preceding her death. He called the changes he observed in her brain plaques and tangles. These features can only be seen upon autopsy. Tangles and plaques interfere with neuronal functions, such as communicating with each other and sending messages to other parts of the body. Research suggests that the formation of tangles in the brain may be a part of the normal aging process. Dr. John Morris of Washington University School of Medicine in St. Louis, Missouri, published a report titled "Tangles and plaques in nondemented aging and preclinical Alzheimer's disease" in the March 1999 issue of Annals of Neurology, Volume 45 Number 3, pages 358-368. This study showed that of the 39 nondemented people (people with no behavioral evidence of Alzheimer's disease), all had tangles in their brains. So perhaps developing tangles is an inevitable part of growing old. "This is further evidence that there is such a thing as truly healthy aging and that Alzheimer's disease is not inevitable," states Morris. More research is needed to figure out what role tangles and plaques play in AD. Are only plaques responsible, or do tangles and plaques interact? Other observed changes in the brain affected by AD include:

Image courtesy of the NIA Illustrator: Lydia Kibiuk

neuronal degeneration in a part of the brain called the nucleus basalis of Meynert. decreased brain levels of the neurotransmitter acetylcholine.

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Neurofibrillary Tangles
It is unclear how neurofibrillary tangles (NFT) form. NFTs are found inside of the neuron: the neurons themselves become deformed and clump together. NFTs have been described as looking like a rope tied in knots. A protein named tau has been shown to be involved in forming NFTs, but more research is needed to solve the mystery of how and why NFTs form, and how exactly they affect the brain.

Plaques
Unlike tangles, plaques occur outside the neuron. Plaques are mainly composed of a protein called beta amyloid, although other proteins contribute to plaque formation. Research has shown that a protein in our bodies called amyloid plays a significant role in AD. Proteins are vital molecules that control all sorts of processes in our body. The amyloid protein occurs naturally in our brains, but as we age, too much of it (in a form called beta amyloid) accumulates in the brain, forming plaques. Beta amyloid is formed when an enzyme clips the amyloid precursor protein; beta amyloid, a fragment from this process then aggregates in deposits. It is unknown whether these deposits are due to excess production or whether the enzymes that usually break it down are not functioning properly. This situation is similar that of cholesterol in our bodies. Some cholesterol is needed to keep our cells healthy, but too much cholesterol can block arteries and lead to heart attacks and other problems.

Image courtesy of the NIA Illustrator: Lydia Kibiuk

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Drug Therapies
Only two drugs for treating AD have been approved by the US Food and Drug Administration (FDA): Cognex and Aricept. These drugs are cholinesterase inhibitors (generic names tacrine and donepezil, respectively). These drugs inhibit the breakdown of acetylcholine by blocking the action of cholinesterase, the key enzyme in the breakdown of acetylcholine. Both drugs increase the level of the acetylcholine in the brain. Both drugs also slow memory loss and help the person perform daily tasks. It is important to note that these drugs are not a cure, they only lessen the symptoms of AD. Tacrine has side effects on liver enzymes, so a new experimental drug, rivastigmine, may soon be approved for AD. It is "brain selective" and does not affect liver enzymes. At least 17 other drugs for treating AD are awaiting approval from the US FDA.

Other Therapies

Vitamin E (an antioxidant) and aspirin (an anti-inflammatory) are being considered as treatments. How each works to lessen the symptoms of AD is not clear. It has been suggested that Vitamin E protects the nerve cell membranes from damage by a harmful chemical process called oxidation. Studies have shown that some architectural designs help AD patients. For example, buildings with circular floor plans allow AD patients to wander until they find their room. Signs reminding AD patients to do certain tasks can help focus attention. Having the same schedule each day can also aid the AD patient's memory. Some studies have shown modest effects of the plant Ginkgo biloba on the symptoms associated with AD. A controversial surgical treatment for AD has been reported by Dr. Harry S. Goldsmith at the University of Nevada School of Medicine. He places part of the omentum, a fatty membrane in the abdomen which holds organs in, on the brain. This has been shown to improve short-term memory in AD patients, perhaps because of a chemical in the omentum. Further research is warranted to explain how (and if) this surgery works.

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Expanding Diagnostic Tools

Because a definitive diagnosis of AD can only be made by examining the brain after a person dies, AD diagnosis in the living must be made by exclusion. This means that other diseases must be ruled out as a cause of the dementia. There is no single test to show that a person has AD, but several tests can suggest the presence of AD.

When analyzing cerebrospinal fluid, an elevated level of certain proteins, including amyloid beta protein, may indicate that a person is likely to have AD. As imaging technology becomes more sophisticated and accessible, health professionals can use CAT scans to visualize brain shrinkage. A brain with widened sulci (the indentations on the surface of the brain), and enlarged cerebral ventricles (the spaces in the brain which are filled with CSF) - all characteristics of AD (and other neurological disorders). Brain imaging methods can also be used to gain information about blood flow and metabolic activity in various parts of the brain. PET Scans

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Risk Factors
First and foremost, the chance of developing AD increases with age. With each additional year of life, there is increased likelihood that one will display symptoms of AD. Alzheimer's disease affects men and women about equally, and strikes all ethnic groups. There are actually two types of AD: familial (early onset) and sporadic (late onset). Familial AD is a rare form of AD that affects a small subset of people at a younger age, usually before their fifties. It has a strong genetic component: mutations on genes 21, 14, and 1 cause a predisposition to AD.(Reported in Neurology, July 1998.) Sporadic (late-onset) AD is the type most people are familiar with. It affects people most often after they reach 65 years of age. A gene called ApoE on chromosome 19 has been pinpointed as being a risk factor for AD.

What Can You Do to Protect Your Brain As You Age?

As you age, some connections in your brain may fail due to tangles or plaques, so it makes sense that the more neural connections you have overall, the more you will be able to compensate for the damaged connections. It's like a sports team. If one player gets injured and there is a qualified player on the bench who can substitute, the team will still function well. The more players available to play for injured team members, the better the team will fare. How do you get and maintain neural connections? It is thought that staying active, both mentally and physically, will help. Challenge your mind. Remember people's names. Work crossword puzzles. Do math. Read. Learn new words.

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Future Research
Understanding Alzheimer's disease is one of the most active areas in neuroscience research. In 1998, scientists took a big step forward by breeding mice that develop the symptoms of AD. These mice will help researchers unravel the mysteries of AD. Research into future treatments can be divided into several categories.

Chemical Theories
1. Biochemical Changes in the Brain Brain cells need certain nutrients to grow. One of these nutrients is called NGF, for nerve growth factor. A decrease in NGF could contribute to AD. Experiments on rats have shown that NGF promoted growth of new synaptic connections in a part of the brain called the hippocampus. This new growth, in theory, could help restore memory loss. The flip side to chemicals which help neurons grow are the chemicals which kill neurons. These chemicals are neurotoxic. Perhaps an increased level of neurotoxins contributes to the disease. If levels of these neurotoxic substances could be regulated, fewer neurons would die, thus lessening the symptoms of AD. 2. Neurotransmitter Deficiencies in the Brain Neurons use neurotransmitters to communicate. As mentioned previously, acetylcholine levels are lower in AD brains compared to non-AD brains. It has been shown that drugs with the side effect of lowering acetylcholine can cause temporary memory loss. Thus, drugs which promote increased levels of acetylcholine in the brain may slow the dementia. 3. Toxic Chemical Excesses in the Brain In the past, aluminum, mercury and other metals found in the brain tissue of AD patients caused speculation that these compounds contributed to the disease. Most scientists agree that aluminum and other metals probably do not cause AD, although they result from the disease process. Further research is needed to clarify

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these metals' role in AD.

The Genetic Theory

The genetics of AD are confusing at best. Some families may have many members affected, but because family members may be exposed to the same environment, it is impossible to say how much heredity is to blame. Several genes have been identified on chromosomes 21 and 14 in the familial type of AD. In the more common sporadic type, people with a gene for a protein called apolipoprotein E on chromosome 19 tend to have a higher incidence of AD than the general population. Much more work needs to be done to fully understand how genetics influences the incidence of AD.

The Autoimmune Theory

Your immune system fights off infection from bacteria, viruses, and other threats to your well-being. If this system goes awry, your immune system can attack its own tissue. Scientists hypothesize that if your immune system attacks your brain as you age, AD symptoms could result. However, it is unclear whether this leads to AD, because signs of this type of attack have been seen in non-AD brains.

The Slow Virus Theory

Some brain disorders which cause symptoms similar to AD are caused by slow viruses. However, no one has identified a virus specific for AD. "Alzheimer's can be called the long good-bye. You grieve about the loved one from the moment you begin to observe the gradual loss of memory and the speech and personality changes, because they are incurable. The person you love is gradually changing before your eyes. You say good-bye many times until the final good-bye at death." --- Norma Wylie, 1996 (in Sharing the Final Journey: Walking with the Dying)

They said it!

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References and further information

1. 2. 3. 4. 5. 6. 7. 8. 9. Alzheimer's: Few Clues on the Mysteries of Memory Alzheimer's Disease - American Health Assistance Foundation Alzheimer's Disease - Unraveling the Mystery Alzheimer's Disease Fact Sheet Alzheimer's Disease - American Federation for Aging Research Alzheimer's Disease - NINDS Alzheimer's Disease Education and Referral (ADEAR) Center The Alzheimer's Association information on issues relating to AD Alzheimer's Disease Bibliography for Children and Teens

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Introduction Dyslexia

What is Dyslexia? Dyslexia is a brain-based type of learning disability that specifically impairs a person's ability to read. These individuals typically read at levels significantly lower than expected despite having normal intelligence. Although the disorder varies from person to person, common characteristics among people with dyslexia are difficulty with phonological processing (the manipulation of sounds) and/or rapid visual-verbal responding. Is there any treatment? The main focus of treatment should be on the specific learning problems of affected individuals. The usual course is to modify teaching methods and the educational environment to meet the specific needs of the individual with dyslexia. What is the prognosis? For those with dyslexia, the prognosis is mixed. The disability affects such a wide range of people, producing different symptoms and varying degrees of severity, that predictions are hard to make. The prognosis is generally good, however, for individuals whose dyslexia is identified early, who have supportive family and friends and a strong self-image, and who are involved in a proper remediation program. What research is being done? The NINDS and other institutes of the National Institutes of Health, including the National Institute of Child Health and Human Development and the National Institute of Mental Health, conduct research on dyslexia. Current research avenues focus on developing techniques to diagnose and treat dyslexia and other learning disabilities, increasing the understanding of the biological basis of learning disabilities, and exploring the relationship between neurophysiological processes and cognitive functions with regard to reading ability.

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