Alzheimer's disease (redirected from Alzheimer's)

Alzheimer's Disease 

Definition

Alzheimer's disease (AD) is the most common form of dementia, a neurologic disease characterized by loss of mental ability severe enough to interfere with normal activities of daily living, lasting at least six months, and not present from birth. AD usually occurs in old age, and is marked by a decline in cognitive functions such as remembering, reasoning, and planning.

Description

A person with AD usually has a gradual decline in mental functions, often beginning with slight memory loss, followed by losses in the ability to maintain employment, to plan and execute familiar tasks, and to reason and exercise judgment. Communication ability, mood, and personality also may be affected. Most people who have AD die within eight years of their diagnosis, although the interval may be as short as one year or as long as 20 years. AD is the fourth leading cause of death in adults after heart disease, cancer, and stroke.

Between two and four million Americans have AD; that number is expected to grow to as many as 14 million by the middle of the 21st century as the population ages. While a small number of people in their 40s and 50s develop the disease (called earlyonset AD), AD predominantly affects the elderly. AD affects about 3% of all people between ages 65 and 74, about 19% of those between 75 and 84, and about 47% of those over 85. Slightly more women than men are affected with AD, but this may be because women tend to live longer, leaving a higher proportion of women in the most affected age groups.

The cost of caring for a person with AD is considerable. The annual cost of caring for one AD patient in 1998 was estimated as about $18,400 for a patient with mild AD, $30,100 for a patient with moderate AD, and $36,100 for a patient with severe AD. The annual direct and indirect costs of caring for AD patients in the United States was estimated to be as much as $100 billion. Slightly more than half of people with AD are cared for at home, while the remainder are cared for in a variety of health care institutions.

Causes and symptoms

Causes

The cause or causes of Alzheimer's disease are largely unknown, though some forms have genetic links. Some strong leads have been found through recent research, however, and these have given some theoretical support to several new experimental treatments.

At first AD destroys neurons (nerve cells) in parts of the brain that control memory, including the hippocampus, which is a structure deep in the deep that controls short-term memory. As these neurons in the hippocampus stop functioning, the person's short-term memory fails, and the ability to perform familiar tasks decreases. Later AD affects the cerebral cortex, particularly the areas responsible for language and reasoning. Many language skills are lost and the ability to make judgments is affected. Personality changes occur, which may include emotional outbursts, wandering, and agitation. The severity of these changes increases with disease progression. Eventually many other areas of the brain become involved, the brain regions affected atrophy (shrink and lose function), and the person with AD becomes bedridden, incontinent, helpless, and non-responsive.

Autopsy of a person with AD shows that the regions of the brain affected by the disease become clogged with two abnormal structures, called neurofibrillary tangles and amyloid plaques. Neurofibrillary tangles are twisted masses of protein fibers inside nerve cells, or neurons. In AD, tau proteins, which normally help bind and stabilize parts of neurons, are changed chemically, become twisted and tangled, and no longer can stabilize the neurons. Amyloid plaques consist of insoluble deposits of beta-amyloid, (a protein fragment from a larger protein called amyloid precursor protein (APP), mixed with parts of neurons and non-nerve cells. Plaques are found in the spaces between the nerve cells of the brain. While it is not clear exactly how these structures cause problems, many researchers believe that their formation is responsible for the mental changes of AD, presumably by interfering with the normal communication between neurons in the brain and later leading to the death of neurons. By 2000, three drugs for the treatment of AD symptoms were approved by the U.S. Food and Drug Administration (FDA). They act by increasing the level of chemical signaling molecules in the brain, known as neurotransmitters, to make up for this decreased communication ability. All act by inhibiting the activity of acetyl-cholinesterase, which is an enzyme that breaks down acetylcholine, an important neurotransmitter released by neurons that is necessary for cognitive function. These drugs modestly increase cognition and improve one's ability to perform normal activities of daily living.

Exactly what triggers the formation of plaques and tangles and the development of AD is unknown. AD likely results from many interrelated factors, including genetic, environmental, and others not yet identified. Two types of AD exist: familial AD (FAD), which is a rare autosomal dominant inherited disease, and sporadic AD, with no obvious inheritance pattern. AD also is described in terms of age at onset, with early onset AD occurring in people younger than 65, and late-onset occurring in those 65 and older. Early onset AD comprises about 5-10 % of AD cases and affects people aged 30 to 60. Some cases of early onset AD are inherited and are common in some families. Early-onset AD often progresses faster than the more common late-onset type.

All cases of FAD, which is relatively uncommon, that have been identified to date are the early onset type. As many as 50% of FAD cases are known to be caused by three genes located on three different chromosomes. Some families have mutations in the APP gene located on chromosome 21, which causes the production of abnormal APP protein. Others have mutations in a gene called presenilin 1 located on chromosome 14, which causes the production of abnormal presenilin 1 protein, and others have mutations in a similar gene called presenilin 2 located on chromosome 1, which causes production of abnormal presenilin 2. Presenilin 1 may be one of the enzymes that clips APP into beta-amyloid; it also may be important in the synaptic connections between brain cells.

Key terms

Acetylcholine — —One of the substances in the body that helps transmit nerve impulses.

Dementia — —Impaired intellectual function that interferes with normal social and work activities.

Ginkgo — —An herb from the Ginkgo biloba tree that some alternative practitioners recommend for the prevention and treatment of AD.

Neurofibrillary tangle — —Twisted masses of protein inside nerve cells that develop in the brains of people with AD.

Senile plaque — —Structures composed of parts of neurons surrounding brain proteins called beta-amyloid deposits found in the brains of people with AD.

There is no evidence that the mutated genes that cause early onset FAD also cause late onset AD, but genetics appears to play a role in this more common form of AD. Discovered by researchers at Duke University in the early 1990s, potentially the most important genetic link to AD was on chromosome 19. A gene on this chromosome, called APOE (apolipoprotein E), codes for a protein involved in transporting lipids into neurons. APOE occurs in at least three forms (alleles), called APOE e2, APOE e3, and APOE e4. Each person inherits one APOE from each parent, and therefore can either have one copy of two different forms, or two copies of one. The relatively rare APOE e2 appears to protect some people from AD, as it seems to be associated with a lower risk of AD and a later age of onset if AD develops. APOE e3 is the most common version found in the general population, and only appears to have a neutral role in AD. However, APOE e4 appears to increase the risk of developing late onset AD with the inheritance of one or two copies of APOE e4. Compared to those without APOE e4, people with one copy are about three times as likely to develop late-onset AD, and those with two copies are almost four times as likely to do so. Having APOE e4 also can lower the age of onset by as much as 17 years. However, APOE e4 only increases the risk of developing AD and does not cause it, as not everyone with APOE e4 develops AD, and people without it can still have the disease. Why APOE e4 increases the chances of developing AD is not known with certainty. However, one theory is that APOE e4 facilitates beta-amyloid buildup in plaques, thus contributing to the lowering of the age of onset of AD; other theories involve interactions with cholesterol levels and effects on nerve cell death independent of its effects on plaque buildup. In 2000, four new AD-related regions in the human genome were identified, where one out of several hundred genes in each of these regions may be a risk factor gene for AD. These genes, which are not yet identified, appear to make a contribution to the risk of developing late-onset AD that is at least as important as APOE e4.

Other non-genetic factors have been studied in relation to the causes of AD. Inflammation of the brain may play a role in development of AD, and use of nonsteroidal anti-inflammatory drugs (NSAIDs) were once thought to reduce the risk of developing AD. Other agents once thought to reduce chances of dementia are now thought to increase its risk. In 2002, hormone replacement therapy (HRT), which combines estrogen and progestogen, was found to double the risk of developing dementia in postmenopausal women. Highly reactive molecular fragments called free radicals damage cells of all kinds, especially brain cells, which have smaller supplies of protective antioxidants thought to protect against free radical damage. Vitamin E is one such antioxidant, and its use in AD may be of possible theoretical benefit.

While the ultimate cause or causes of Alzheimer's disease still are unknown, there are several risk factors that increase a person's likelihood of developing the disease. The most significant one is, of course, age; older people develop AD at much higher rates than younger ones. There is some evidence that strokes and AD may be linked, with small strokes that go undetected clinically contributing to the injury of neurons. A 2003 Dutch study reported that symptomless, unnoticed strokes could double the risk of AD and other dementias. Blood cholesterol levels also may be important. Scientists have shown that high blood cholesterol levels in special breeds of genetically engineered (transgenic) mice may increase the rate of plaque deposition. There are also parallels between AD and other progressive neurodegenerative disorders that cause dementia, including prion diseases, Parkinson's disease, and Huntington's disease.

Numerous epidemiological studies of populations also are being conducted to learn more about whether and to what extent early life events, socioeconomic factors, and ethnicity have an impact on the development of AD. For example, a 2003 report showed that the more formal education a person has, the better his or her memory is, despite presence of AD. Other studies have related education level or participation in leisure activities such as playing cards or doing crossword puzzles to delayed onset of AD.

Many environmental factors have been suspected of contributing to AD, but epidemiological population studies have not borne out these links. Among these have been pollutants in drinking water, aluminum from commercial products, and metal dental fillings. To date, none of these factors has been shown to cause AD or increase its likelihood. Further research may yet turn up links to other environmental factors.

Symptoms

The symptoms of Alzheimer's disease begin gradually, usually with memory lapses. Occasional memory lapses are of course common to everyone, and do not by themselves signify any change in cognitive function. The person with AD may begin with only the routine sort of memory lapse—forgetting where the car keys are—but progress to more profound or disturbing losses, such as forgetting that he or she can even drive a car. Becoming lost or disoriented on a walk around the neighborhood becomes more likely as the disease progresses. A person with AD may forget the names of family members, or forget what was said at the beginning of a sentence by the time he hears the end.

As AD progresses, other symptoms appear, including inability to perform routine tasks, loss of judgment, and personality or behavior changes. Some people with AD have trouble sleeping and may suffer from confusion or agitation in the evening ("sunsetting" or Sundowner's Syndrome). In some cases, people with AD repeat the same ideas, movements, words, or thoughts. In the final stages people may have severe problems with eating, communicating, and controlling their bladder and bowel functions.

The Alzheimer's Association has developed a list of 10 warning signs of AD. A person with several of these symptoms should see a physician for a thorough evaluation:

• memory loss that affects job skills
• difficulty performing familiar tasks
• problems with language
• disorientation of time and place

• poor or decreased judgment
• problems with abstract thinking
• misplacing things
• changes in mood or behavior
• changes in personality
• loss of initiative

Other types of dementia, including some that are reversible, can cause similar symptoms. It is important for the person with these symptoms to be evaluated by a professional who can weigh the possibility that his or her symptoms may have another cause. Approximately 20% of those originally suspected of having AD turn out to have some other disorder; about half of these cases are treatable.

Diagnosis

Diagnosis of Alzheimer's disease is complex, and may require office visits to several different specialists over several months before a diagnosis can be made. While a confident provisional diagnosis may be made in most cases after thorough testing, AD cannot be diagnosed definitively until autopsy examination of the brain for plaques and neurofibrillary tangles.

The diagnosis of AD begins with a thorough physical exam and complete medical history. Except in the disease's earliest stages, accurate history from family members or caregivers is essential. Since there are both prescription and over-the-counter drugs that can cause the same mental changes as AD, a careful review of the patient's drug, medicine, and alcohol use is important. AD-like symptoms also can be provoked by other medical conditions, including tumors, infection, and dementia caused by mild strokes (multi-infarct dementia). These possibilities must be ruled out as well through appropriate blood and urine tests, brain magnetic resonance imaging (MRI), positron emission tomography (PET) or single photon emission computed tomography (SPECT) scans, tests of the brain's electrical activity (electroencephalographs or EEGs), or other tests. Several types of oral and written tests are used to aid in the AD diagnosis and to follow its progression, including tests of mental status, functional abilities, memory, and concentration. Still, the neurologic exam is normal in most patients in early stages.

One of the most important parts of the diagnostic process is to evaluate the patient for depression and delirium, since each of these can be present with AD, or may be mistaken for it. (Delirium involves a decreased consciousness or awareness of one's environment.) Depression and memory loss both are common in the elderly, and the combination of the often can be mistaken for AD. On the other hand, depression can be a risk factor for AD. A 2003 study showed that a history of depressive symptoms can be associated with nearly twice the risk of eventually developing AD. Depression can be treated with drugs, although some antidepressants can worsen dementia if it is present, further complicating both diagnosis and treatment.

An early and accurate diagnosis of AD is important in developing strategies for managing symptoms and for helping patients and their families planning for the future and pursuing care options while the patient can still take part in the decision-making process.

A genetic test for the APOE e4 gene is available, but is not used for diagnosis, since possessing even two copies does not ensure that a person will develop AD. In addition, access to genetic information could affect the insurability of a patient if disclosed, and also affect employment status and legal rights.

Treatment

Alzheimer's disease is presently incurable. Recent reports show that prompt intervention can slow decline from AD. The use of medications mentioned below as early as possible in the course of AD can help people with the disease maintain independent function as long as possible. The remaining treatment for a person with AD is good nursing care, providing both physical and emotional support for a person who is gradually able to do less and less for himself, and whose behavior is becoming more and more erratic. Modifications of the home to increase safety and security often are necessary. The caregiver also needs support to prevent anger, despair, and burnout from becoming overwhelming. Becoming familiar with the issues likely to lie ahead, and considering the appropriate financial and legal issues early on, can help both the patient and family cope with the difficult process of the disease. Regular medical care by a practitioner with a non-defeatist attitude toward AD is important so that illnesses such as urinary or respiratory infections can be diagnosed and treated properly, rather than being incorrectly attributed to the inevitable decline seen in AD.

People with AD often are depressed or anxious, and may suffer from sleeplessness, poor nutrition, and general poor health. Each of these conditions is treatable to some degree. It is important for the person with AD to eat well and continue to exercise. Professional advice from a nutritionist may be useful to provide healthy, easy-to-prepare meals. Finger foods may be preferable to those requiring utensils to be eaten. Regular exercise (supervised if necessary for safety) promotes overall health. A calm, structured environment with simple orientation aids (such as calendars and clocks) may reduce anxiety and increase safety. Other psychiatric symptoms, such as depression, anxiety, hallucinations (seeing or hearing things that aren't there), and delusions (false beliefs) may be treated with drugs if necessary.

Drugs

As of 2003, four drugs—tacrine (Cognex), donepezil hydrochloride (Aricept), and rivastigmine (Exelon)—have been approved by the FDA for its treatment. Tacrine has been shown to be effective for improving memory skills, but only in patients with mild-to-moderate AD, and even then in less than half of those who take it. Its beneficial effects are usually mild and temporary, but it may delay the need for nursing home admission. The most significant side effect is an increase in a liver enzyme known as alanine aminotransferase, or ALT. Patients taking tacrine must have a weekly blood test to monitor their ALT levels. Other frequent side effects include nausea, vomiting, diarrhea, abdominal pain, indigestion, and skin rash. The cost of tacrine was about $125 per month in early 1998, with additional costs for the weekly blood monitoring. Despite its high cost, tacrine appears to be cost-effective for those who respond to it, since it may decrease the number of months a patient needs nursing care. Donepezil is the drug most commonly used to treat mild to moderate symptoms of AD, although it only helps some patients for periods of time ranging from months to about two years. Donepezil has two advantages over tacrine: it has fewer side effects, and it can be given once daily rather than three times daily. Donepezil does not appear to affect liver enzymes, and therefore does not require weekly blood tests. The frequency of abdominal side effects is also lower. The monthly cost is approximately the same. Rivastigmine, approved for use in April of 2000, has been shown to improve the ability of patients to carry out daily activities, such as eating and dressing, decrease behavioral symptoms such as delusions and agitation, and improve cognitive functions such as thinking, memory, and speaking. The cost is similar to those of the other two drugs. However, none of these three drugs stops or reverses the progression of AD. Galantamine (Reminyl) works in the early and moderates stages of AD. It has fewer side effects than other drugs, with the exception of donepezil and must be taken twice a day. Three other drugs were being tested for AD treatment in mid-2003.

Estrogen, the female sex hormone, is widely prescribed for post-menopausal women to prevent osteoporosis. Studies once showed that estrogen was beneficial to women with AD, but in 2003, a large clinical trial called the Women's Health Initiative showed dementia among other negative effects of combined estrogen therapy.

Preliminary studies once suggested a reduced risk for developing AD in elderly people who regularly used nonsteroidal anti-inflammatory drugs (NSAIDs), including aspirin, ibuprofen, and naproxen, although not acetaminophen. However, an important study published in 2003 showed that NSAIDs were not effective in preventing or slowing the progression of AD. The study authors recommended that people stop taking NSAIDs to slow dementia.

Antioxidants, which act to inhibit and protect against oxidative damage caused by free radicals, have been shown to inhibit toxic effects of beta-amyloid in tissue culture. Therefore, research is being conducted to see whether antioxidants may delay or prevent AD.

Another antioxidant, vitamin E, is also thought to delay AD onset. Hoever, it is not yet clear whether this is due to the specific action of vitamin E on brain cells, or to an increase in the overall health of those taking it.

Drugs such as antidepressants, anti-psychotics, and sedatives are used to treat the behavioral symptoms (agitation, aggression, wandering, and sleep disorders) of AD. Research is being conducted to search for better treatments, including non-drug approaches for AD patients.

Nursing care and safety

The person with Alzheimer's disease will gradually lose the ability to dress, groom, feed, bathe, or use the toilet by himself; in the later stages of the disease, he may be unable to move or speak. In addition, the person's behavior becomes increasingly erratic. A tendency to wander may make it difficult to leave him unattended for even a few minutes and make even the home a potentially dangerous place. In addition, some people with AD may exhibit inappropriate sexual behaviors.

The nursing care required for a person with AD is well within the abilities of most people to learn. The difficulty for many caregivers comes in the constant but unpredictable nature of the demands put on them. In addition, the personality changes undergone by a person with AD can be heartbreaking for family members as a loved one deteriorates, seeming to become a different person. Not all people with AD develop negative behaviors. Some become quite gentle, and spend increasing amounts of time in dreamlike states.

A loss of good grooming may be one of the early symptoms of AD. Mismatched clothing, unkempt hair, and decreased interest in personal hygiene become more common. Caregivers, especially spouses, may find these changes socially embarrassing and difficult to cope with. The caregiver usually will need to spend increasing amounts of time on grooming to compensate for the loss of attention from the patient, although some adjustment of expectations (while maintaining cleanliness) is often needed as the disease progresses.

Proper nutrition is important for a person with AD, and may require assisted feeding early on, to make sure the person is taking in enough nutrients. Later on, as movement and swallowing become difficult, a feeding tube may be placed into the stomach through the abdominal wall. A feeding tube requires more attention, but is generally easy to care for if the patient is not resistant to its use.

For many caregivers, incontinence becomes the most difficult problem to deal with at home, and is a principal reason for pursuing nursing home care. In the early stages, limiting fluid intake and increasing the frequency of toileting can help. Careful attention to hygiene is important to prevent skin irritation and infection from soiled clothing.

Persons with dementia must deal with six basic safety concerns: injury from falls, injury from ingesting dangerous substances, leaving the home and getting lost, injury to self or others from sharp objects, fire or burns, and the inability to respond rapidly to crisis situations. In all cases, a person diagnosed with AD should no longer be allowed to drive, because of the increased potential for accidents and the increased likelihood of wandering very far from home while disoriented. In the home, simple measures such as grab bars in the bathroom, bed rails on the bed, and easily negotiable passageways can greatly increase safety. Electrical appliances should be unplugged and put away when not in use, and matches, lighters, knives, or weapons should be stored safely out of reach. The hot water heater temperature may be set lower to prevent accidental scalding. A list of emergency numbers, including the poison control center and the hospital emergency room, should be posted by the phone. As the disease progresses, caregivers need to periodically reevaluate the physical safety of the home and introduce new strategies for continued safety.

Care for the caregiver

Family members or others caring for a person with AD have an extremely difficult and stressful job, which becomes harder as the disease progresses. Dementia caregivers spend significantly more time on caregiving than do people providing care for those with other types of illnesses. This type of caregiving also has a greater impact in terms of employment complications, caregiver strain, mental and physical health problems, time for leisure and other family members, and family conflict than do other types of caregiving. It is common for AD caregivers to develop feelings of anger, resentment, guilt, and hopelessness, in addition to the sorrow they feel for their loved one and for themselves. Depression is an extremely common consequence of being a full-time caregiver for a person with AD. Support groups are an important way to deal with the stress of caregiving. Becoming a member of an AD caregivers' support group can be one of the most important things a family member does, not only for him or herself, but for the person with AD as well. The location and contact numbers for AD caregiver support groups are available from the Alzheimer's Association; they also may be available through a local social service agency, the patient's physician, or pharmaceutical companies that manufacture the drugs used to treat AD. Medical treatment for depression may be an important adjunct to group support.

Outside help, nursing homes, and governmental assistance

Most families eventually need outside help to relieve some of the burden of around-the-clock care for a person with AD. Personal care assistants, either volunteer or paid, may be available through local social service agencies. Adult daycare facilities are becoming increasingly common. Meal delivery, shopping assistance, or respite care may be available as well.

Providing the total care required by a person with late-stage AD can become an overwhelming burden for a family, even with outside help. At this stage, many families consider nursing home care. This decision often is one of the most difficult for the family, since it is often seen as an abandonment of the loved one and a failure of the family. Careful counseling with a sympathetic physician, clergy, or other trusted adviser may ease the difficulties of this transition. Selecting a nursing home may require a difficult balancing of cost, services, location, and availability. Keeping the entire family involved in the decision may help prevent further stress from developing later on.

Several federal government programs may ease the cost of caring for a person with AD, including Social Security Disability, Medicare, and Supplemental Security Income. Each of these programs may provide some assistance for care, medication, or other costs, but none of them will pay for nursing home care indefinitely. Medicaid is a state-funded program that may provide for some or all of the cost of nursing home care, although there are important restrictions. Details of the benefits and eligibility requirements of these programs are available through the local Social Security or Medicaid office, or from local social service agencies.

Private long-term care insurance, special "reverse mortgages," viatical insurance, and other financial devices are other ways of paying for care for those with the appropriate financial situations. Further information on these options may be available through resources listed below.

Alternative treatment

Several substances are currently being tested for their ability to slow the progress of Alzheimer's disease. These include acetylcarnitine, a supplement that acts on the cellular energy structures known as mitochondria. Ginkgo extract, derived from the leaves of the Ginkgo biloba tree, appears to have antioxidant as well as anti-inflammatory and anticoagulant properties. Ginkgo extract has been used for many years in China and is widely prescribed in Europe for treatment of circulatory problems. A 1997 study of patients with dementia seemed to show that ginkgo extract could improve their symptoms, though the study was criticized for certain flaws in its method. Large scale follow-up studies are being conducted to determine whether Ginkgo extract can prevent or delay the development of AD. Ginkgo extract is available in many health food or nutritional supplement stores. Some alternative practitioners also advise people with AD to take supplements of phosphatidylcholine, vitamin B₁₂, gotu kola, ginseng, St. Johnõs Wort, rosemary, saiko-keishi-to-shakuyaku (A Japanese herbal mixture), and folic acid.

Prognosis

While Alzheimer's disease may not be the direct cause of death, the generally poorer health of a person with AD increases the risk of life-threatening infection, including pneumonia. In addition, other diseases common in old age—cancer, stroke, and heart disease—may lead to more severe consequences in a person with AD. On average, people with AD live eight years past their diagnosis, with a range from one to 20 years.

Prevention

Currently, there is no sure way to prevent Alzheimer's disease. treatments discussed above may eventually be proven to reduce the risk of developing the disease. Avoiding risks such as hormone replacement therapy may help prevent development of AD.

Research on the prevention of AD is focusing on blocking the production of amyloid in the brain as well as breaking down beta-amyloid once it is released from cells but before it has a chance to aggregate into insoluble plaques. There also are promising studies being conducted to develop an AD vaccine, where immune responses may result in the elimination of the formation of amyloid plaques.

The Alzheimer's Disease Research Centers (ADCs) program promotes research, training and education, technology transfer, and multicenter and cooperative studies in AD, other dementias, and normal brain aging. Each ADC enrolls and performs studies on AD patients and healthy older people. Persons can participate in research protocols and clinical drug trials at these centers. Data from the ADCs as well as from other sources are coordinated and made available for use by researchers at the National Alzheimer's Coordinating Center, established in 1999.

Resources

Books

Cohen, Donna, and Carl Eisdorfer. The Loss of Self: A Family Resource for the Care of Alzheimer's Disease and Related Disorders. Revised. NewYork: W.W. Norton & Company, 2001.

Geldmacher, David S. Contemporary Diagnosis and Management of Alzheimer's Disease. Newtown, PA: Associates in Medical Marketing Co., Inc., 2001.

Gruetzner, Howard. Alzheimer's: A Caregiverõs Guideand Sourcebook. 3rd ed. New York: John Wiley & Sons, 2001.

Mace, Nancy L., and Peter V. Rabins. The 36-Hour Day: A Family Guide for Caring with Persons with Alzheimer Disease, Related Dementing Illnesses, and MemoryLoss in Later Life. New York: Warner Books, 2001.

Teitel, Rosette, and Marc L. Gordon. The Handholderõs Handbook: A Guide for Caregivers of Alzheimerõs and other Dementias. NewBrunswick, NJ: Rutgers University Press, 2001.

Periodicals

"Alzheimer's Could be Linked to Depression." GP (May 26, 2003): 4.

"Alzheimer's Could Reduced by Education." The Lancet (June 28, 2003): 2215.

"Contrary to Some Earlier Results, New Study Shows NSAIDs Do Not Slow Progression of Alzheimer's Disease." The Brown University Geriatric Psychopharmacology Update (July 2003): 1.

Gitlin, L.N., and M. Corcoran. "Making Homes Safer: Environmental Adaptations for People with Dementia." Alzheimer's Care Quarterly 1 (2000): 50-58.

Helmuth, L. "Alzheimer's Congress: Further Progress on aB-Amyloid Vaccine." Science 289, no. 5476 (2000): 375.

Josefson, Deborah. "Latests HRT Trial Results Show Risk of Dementia." British Medical Journal (June 7, 2003): 1232.

McReady, Norah. "Prompt Intervention May Slow Alzheimer's Decline." Family Practice News (May 1, 2003): 32-41.

Naditz, Alan. "Deeply Affected: As the Nation Ages, Alzheimer's Will Strike More People Close to Us." Contemporary Long Term Care (July 2003): 20-23.

"Researchers Believe "Silent" Strokes Boost Risk." GP (April 14, 2003): 9.

Other

Alzheimer's Disease Books and Videotapes. http://www.alzheimersbooks.com.

National Institute on Aging, National Institutes of Health. 2000: Progress Report on Alzheimer's Disease—Taking the Next Steps. NIH Publication No. 4859 (2000). 〈http://www.alzheimers.org/pubs/prog00.htm#References〉.

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disease /dis·ease/ (dĭ-zēz´) any deviation from or interruption of the normal structure or function of any body part, organ, or system that is manifested by a characteristic set of symptoms and signs and whose etiology, pathology, and prognosis may be known or unknown. See also entries under syndrome.

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acquired cystic disease of kidney  the development of cysts in the formerly noncystic failing kidney in end-stage renal disease.

Addison's disease  bronzelike pigmentation of the skin, severe prostration, progressive anemia, low blood pressure, diarrhea, and digestive disturbance, due to adrenal hypofunction.

Albers-Schönberg disease  osteopetrosis.

allogeneic disease  graft-versus-host reaction occurring in immunosuppressed animals receiving injections of allogeneic lymphocytes.

Alpers' disease  a rare disease of young children, characterized by neuronal deterioration of the cerebral cortex and elsewhere, progressive mental deterioration, motor disturbances, seizures, and early death.

alpha chain disease  heavy chain disease characterized by plasma cell infiltration of the lamina propria of the small intestine resulting in malabsorption with diarrhea, abdominal pain, and weight loss, possibly accompanied by pulmonary involvement.

Alzheimer's disease  progressive degenerative disease of the brain, of unknown cause; characterized by diffuse atrophy throughout the cerebral cortex with distinctive histopathological changes.

Andersen's disease  glycogen storage d., type IV.

apatite deposition disease  a connective tissue disorder marked by deposition of hydroxyapatite crystals in one or more joints or bursae.

Aran-Duchenne disease  spinal muscular atrophy.

arteriosclerotic cardiovascular disease  (ASCVD) atherosclerotic involvement of arteries to the heart and to other organs, resulting in debility or death; sometimes used specifically for ischemic heart disease.

arteriosclerotic heart disease  (ASHD) ischemic heart d.

autoimmune disease  any of a group of disorders in which tissue injury is associated with humoral or cell-mediated responses to the body's own constituents; they may be systemic or organ-specific.

Ayerza's disease  polycythemia vera with chronic cyanosis, dyspnea, bronchitis, bronchiectasis, hepatosplenomegaly, bone marrow hyperplasia, and pulmonary artery sclerosis.

Banti's disease  congestive splenomegaly.

Barlow disease  scurvy in infants.

Barraquer's disease  partial lipodystrophy.

Basedow's disease  Graves' d.

Batten disease , Batten-Mayou disease

1. Vogt-Spielmeyer d.

2. more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.

Bayle's disease  general paresis.

Bazin's disease  erythema induratum.

Bekhterev's (Bechterew's) disease  ankylosing spondylitis.

Benson's disease  asteroid hyalosis.

Berger's disease  IgA glomerulonephritis.

Bernhardt's disease , Bernhardt-Roth disease meralgia paresthetica.

Besnier-Boeck disease  sarcoidosis.

Best's disease  congenital macular degeneration.

Bielschowsky-Janský disease  Janský-Bielschowsky d.

Binswanger's disease  a degenerative dementia of presenile onset caused by demyelination of the subcortical white matter of the brain.

black disease  a fatal disease of sheep, and sometimes of humans, in the United States and Australia, due to Clostridium novyi, marked by necrotic areas in the liver.

Blocq's disease  astasia-abasia.

Blount disease  tibia vara.

Boeck's disease  sarcoidosis.

Bornholm disease  epidemic pleurodynia.

Bowen's disease  a squamous cell carcinoma in situ, often due to prolonged exposure to arsenic; usually occurring on sun-exposed areas of skin. The corresponding lesion on the glans penis is termed erythroplasia of Queyrat.

Brill's disease  Brill-Zinsser d.

Brill-Symmers disease  giant follicular lymphoma.

Brill-Zinsser disease  mild recrudescence of epidemic typhus years after the initial infection, because Rickettsia prowazekii has persisted in body tissue in an inactive state, with humans as the reservoir.

broad beta disease  familial dysbetalipoproteinemia; named for the electrophoretic mobility of the abnormal chylomicron and very-low-density lipoprotein remnants produced.

Busse-Buschke disease  cryptococcosis.

Caffey's disease  infantile cortical hyperostosis.

calcium hydroxyapatite deposition disease  apatite deposition d.

calcium pyrophosphate deposition disease  (CPDD) an acute or chronic inflammatory arthropathy caused by deposition of calcium pyrophosphate dihydrate (CPPD) crystals in the joints, chondrocalcinosis, and crystals in the synovial fluid. Acute attacks are sometimes called pseudogout.

Calvé-Perthes disease  osteochondrosis of capitular epiphysis of femur.

Camurati-Engelmann disease  diaphyseal dysplasia.

Canavan disease , Canavan-van Bogaert-Bertrand disease spongy degeneration of the central nervous system.

Carrión's disease  bartonellosis.

Castleman disease  a benign or premalignant condition resembling lymphoma but without recognizable malignant cells; there are isolated masses of lymphoid tissue and lymph node hyperplasia, usually in the abdominal or mediastinal area.

cat-scratch disease  a usually benign, self-limited disease of the regional lymph nodes, caused by Bartonella henselae and characterized by a papule or pustule at the site of a cat scratch, subacute painful regional lymphadenitis, and mild fever.

celiac disease  a malabsorption syndrome precipitated by ingestion of gluten-containing foods, with loss of villous structure of the proximal intestinal mucosa, bulky, frothy diarrhea, abdominal distention, flatulence, weight loss, and vitamin and electrolyte depletion.

Chagas disease  trypanosomiasis due to Trypanosoma cruzi; its course may be acute, subacute, or chronic.

Charcot-Marie-Tooth disease  muscular atrophy of variable inheritance, beginning in the muscles supplied by the peroneal nerves and progressing to those of the hands and arms.

cholesteryl ester storage disease  (CESD) a lysosomal storage disease due to deficiency of lysosomal cholesterol esterase, variably characterized by some combination of hepatomegaly, hyperbetalipoproteinemia, and premature atherosclerosis.

Christmas disease  hemophilia B.

chronic granulomatous disease  frequent, severe infections of the skin, oral and intestinal mucosa, reticuloendothelial system, bones, lungs, and genitourinary tract associated with a genetically determined defect in the intracellular bactericidal function of leukocytes.

chronic obstructive pulmonary disease  (COPD) any disorder marked by persistent obstruction of bronchial air flow.

Coats' disease  exudative retinopathy.

collagen disease  any of a group of diseases characterized by widespread pathologic changes in connective tissue; they include lupus erythematosus, dermatomyositis, scleroderma, polyarteritis nodosa, thrombotic purpura, rheumatic fever, and rheumatoid arthritis. Cf. collagen disorder.

communicable disease  a disease the causative agents of which may pass or be carried from one person to another directly or indirectly.

Concato's disease  progressive malignant polyserositis with large effusions into the pericardium, pleura, and peritoneum.

constitutional disease  one involving a system of organs or one with widespread symptoms.

Cori's disease  glycogen storage d., type III.

coronary artery disease  (CAD) atherosclerosis of the coronary arteries, which may cause angina pectoris, myocardial infarction, and sudden death; risk factors include hypercholesterolemia, hypertension, smoking, diabetes mellitus, and low levels of high-density lipoproteins.

coronary heart disease  (CHD) ischemic heart d.

Cowden disease  a hereditary disease marked by multiple ectodermal, mesodermal, and endodermal nevoid and neoplastic anomalies.

Creutzfeldt-Jakob disease  a rare prion disease existing in sporadic, familial, and infectious forms, with onset usually in middle life, and having a wide variety of clinical and pathological features. The most commonly seen are spongiform degeneration of neurons, neuronal loss, gliosis, and amyloid plaque formation, accompanied by rapidly progressive dementia, myoclonus, motor disturbances, and encephalographic changes, with death occurring usually within a year of onset.

Crigler-Najjar disease  see under syndrome.

Crohn's disease  regional enteritis; a chronic granulomatous inflammatory disease usually in the terminal ileum with scarring and thickening of the wall, often leading to intestinal obstruction and formation of fistulas and abscesses.

Crouzon's disease  craniofacial dysostosis.

Cruveilhier's disease  spinal muscular atrophy.

Cushing's disease  Cushing's syndrome in which the hyperadrenocorticism is secondary to excessive pituitary secretion of adrenocorticotropic hormone.

cystic disease of breast  mammary dysplasia with formation of blue dome cysts.

cytomegalic inclusion disease , cytomegalovirus disease an infection due to cytomegalovirus and marked by nuclear inclusion bodies in enlarged infected cells. In the congenital form, there is hepatosplenomegaly with cirrhosis, and microcephaly with mental or motor retardation. Acquired disease may cause a clinical state similar to infectious mononucleosis. When acquired by blood transfusion, postperfusion syndrome results.

deficiency disease  a condition caused by dietary or metabolic deficiency, including all diseases due to an insufficient supply of essential nutrients.

degenerative joint disease  osteoarthritis.

Dejerine's disease , Dejerine-Sottas disease progressive hypertrophic neuropathy.

demyelinating disease  any condition characterized by destruction of the myelin sheaths of nerves.

disappearing bone disease  gradual resorption of a bone or group of bones, sometimes associated with multiple hemangiomas, usually in children or young adults and following trauma.

diverticular disease  a general term including the prediverticular state, diverticulosis, and diverticulitis.

Duchenne's disease 

1. spinal muscular atrophy.

2. progressive bulbar paralysis.

3. tabes dorsalis.

4. Duchenne's muscular dystrophy.

Duchenne-Aran disease  spinal muscular atrophy.

Duhring's disease  dermatitis herpetiformis.

Dukes' disease  a febrile disease of childhood marked by an exanthematous eruption, probably due to a virus of the Coxsackie-ECHO group.

Durand-Nicolas-Favre disease  lymphogranuloma venereum.

Duroziez's disease  congenital mitral stenosis.

Ebola virus disease  fatal acute hemorrhagic fever resembling Marburg virus disease but caused by Ebola virus, seen in the Sudan and Zaire.

Ebstein's disease  see under anomaly.

end-stage renal disease  chronic irreversible renal failure.

Erb's disease  Duchenne's muscular dystrophy.

Erb-Goldflam disease  myasthenia gravis.

Eulenburg's disease  paramyotonia congenita.

extrapyramidal disease  any of a group of clinical disorders marked by abnormal involuntary movements, alterations in muscle tone, and postural disturbances; they include parkinsonism, chorea, athetosis, etc.

Fabry's disease  an X-linked lysosomal storage disease of glycosphingolipid catabolism resulting from deficiency of α-galactosidase A and leading to accumulation of ceramide trihexoside in the cardiovascular and renal systems.

Farber's disease  a lysosomal storage disease due to defective ceramidase and characterized by hoarseness, aphonia, dermatitis, bone and joint deformities, granulomatous reaction, and psychomotor retardation.

Fazio-Londe disease  a rare type of progressive bulbar palsy occurring in childhood.

Feer disease  acrodynia.

fibrocystic disease of breast  a form of mammary dysplasia with formation of cysts of various size containing a semitransparent, turbid fluid that imparts a brown to blue color to the unopened cysts; believed due to abnormal hyperplasia of the ductal epithelium and dilatation of the ducts of the mammary gland, resulting from exaggeration and distortion of normal menstrual cycle–related breast changes.

fibrocystic disease of the pancreas  cystic fibrosis.

fifth disease  erythema infectiosum.

flint disease  chalicosis.

floating beta disease  familial dysbetalipoproteinemia.

focal disease  a localized disease.

foot-and-mouth disease  an acute, contagious viral disease of wild and domestic cloven-footed animals and occasionally humans, marked by vesicular eruption on the lips, buccal cavity, pharynx, legs, and feet.

Forbes' disease  glycogen storage d., type III.

fourth disease  Dukes' d.

fourth venereal disease  granuloma inguinale.

Fox-Fordyce disease  a persistent and recalcitrant, itchy, papular eruption, chiefly of the axillae and pubes, due to inflammation of apocrine sweat glands.

Freiberg's disease  osteochondrosis of the head of the second metatarsal bone.

Friedländer's disease  endarteritis obliterans.

Friedreich's disease  paramyoclonus multiplex.

functional disease  see under disorder.

Garré's disease  sclerosing nonsuppurative osteomyelitis.

gastroesophageal reflux disease  (GERD) any condition resulting from gastroesophageal reflux, characterized by heartburn and regurgitation; see also reflux esophagitis.

Gaucher's disease  a hereditary disorder of glucocerebroside metabolism, marked by the presence of Gaucher's cells in the marrow, and by hepatosplenomegaly and erosion of the cortices of long bones and pelvis. The adult form is associated with moderate anemia and thrombocytopenia, and yellowish pigmentation of the skin; in the infantile form there is, in addition, marked central nervous system impairment; in the juvenile form there are rapidly progressive systemic manifestations but moderate central nervous system involvement.

genetic disease  a general term for any disorder caused by a genetic mechanism, comprising chromosome aberrations (or anomalies), mendelian (or monogenic or single-gene) disorders, and multifactorial disorders.

gestational trophoblastic disease  see under neoplasia.

Gilbert disease  a familial, benign elevation of bilirubin levels without evidence of liver damage or hematologic abnormalities.

Gilles de la Tourette's disease  see under syndrome.

Glanzmann disease  see thrombasthenia.

glycogen storage disease  any of a number of rare inborn errors of metabolism caused by defects in specific enzymes or transporters involved in the metabolism of glycogen.

type I glucose-6-phosphatase deficiency: a severe hepatorenal form due to deficiency of the hepatic enzyme glucose-6-phosphatase, resulting in liver and kidney involvement, with hepatomegaly, hypoglycemia, hyperuricemia, and gout

type IA glycogen storage d., type I.

type IB a form resembling type I but additionally predisposing to infection due to neutropenia and to chronic inflammatory bowel disease; due to a defect in the transport system for glucose 6-phosphate.

type II a disorder due to deficiency of the lysosomal enzyme α-1,4,-glucosidase, the severe infant form resulting in generalized glycogen accumulation, with cardiomegaly, cardiorespiratory failure, and death, and a milder adult form being a gradual skeletal myopathy that sometimes causes respiratory problems.

type III a form due to deficiency of debrancher enzyme (amylo-1,6-glucosidase) in muscle, liver, or both; defects in the liver enzyme are characterized by hepatomegaly and hypoglycemia while defects in the muscle enzyme are characterized by progressive muscle wasting and weakness.

type IV brancher enzyme deficiency; cirrhosis of the liver, hepatosplenomegaly, progressive hepatic failure, and death due to deficiency of the glycogen brancher enzyme (1,4-α-glucan branching enzyme).

type V muscle cramps and fatigue during exercise due to a defect in the skeletal muscle isozyme of glycogen phosphorylase (muscle phosphorylase).

type VI hepatomegaly, mild to moderate hypoglycemia and mild ketosis, due to deficiency of the liver isozyme of glycogen phosphorylase (hepatic phosphorylase).

type VII muscle weakness and cramping after exercise due to deficiency of the muscle isozyme of 6-phosphofructokinase.

type VIII phosphorylase .

graft-versus-host (GVH) disease  disease caused by the immune response of histoincompatible, immunocompetent donor cells against the tissue of immunocompromised host, as a complication of bone marrow transplantation, or as a result of maternal-fetal blood transfusion, or therapeutic transfusion to an immunocompromised recipient.

Graves' disease  an association of hyperthyroidism, goiter, and exophthalmos, with accelerated pulse rate, profuse sweating, nervous symptoms, psychic disturbances, emaciation, and elevated basal metabolism.

Greenfield's disease  former name for the late infantile form of metachromatic leukodystrophy.

Gull's disease  atrophy of the thyroid gland with myxedema.

Günther disease  congenital erythropoietic porphyria.

H disease  Hartnup d.

Hailey-Hailey disease  benign familial pemphigus.

Hallervorden-Spatz disease  an autosomal recessive disorder caused by decreased numbers of myelin sheaths of the globus pallidus and substantia nigra, with accumulation of iron pigment, progressive rigidity beginning in the legs, choreoathetoid movements, dysarthria, and mental deterioration.

Hand's disease  Hand-Schüller-Christian d.

hand-foot-and-mouth disease  a mild, highly infectious viral disease of children, with vesicular lesions in the mouth and on the hands and feet.

Hand-Schüller-Christian disease  a chronic, progressive form of multifocal Langerhans cell histiocytosis, sometimes with accumulation of cholesterol, characterized by the triad of calvarial bone defects, exophthalmos, and diabetes insipidus.

Hansen's disease  leprosy.

Hartnup disease  a hereditary disorder of intestinal and renal transport of neutral α-amino acids, marked by a pellagra-like skin rash, with transient cerebellar ataxia, constant renal aminoaciduria, and other biochemical abnormalities.

Hashimoto's disease  a progressive disease of the thyroid gland with degeneration of its epithelial elements and replacement by lymphoid and fibrous tissue.

heavy chain diseases  a group of malignant neoplasms of lymphoplasmacytic cells marked by the presence of immunoglobulin heavy chains or heavy chain fragments; they are classified according to heavy chain type, e.g., alpha chain disease.

Heine-Medin disease  the major form of poliomyelitis.

hemoglobin disease  any of various hereditary molecular diseases characterized by abnormal hemoglobins in the red blood cells; the homozygous form is manifested by hemolytic anemia.

hemolytic disease of the newborn  erythroblastosis fetalis.

hemorrhagic disease of the newborn  a self-limited hemorrhagic disorder of the first few days of life, due to deficiency of vitamin K–dependent coagulation factors II, VII, IX, and X.

Hers' disease  glycogen storage d., type VI.

Heubner-Herter disease  the infantile form of celiac disease.

hip-joint disease  tuberculosis of the hip joint.

Hippel's disease  von Hippel's d.

Hirschsprung's disease  congenital megacolon.

His disease , His-Werner disease trench fever.

Hodgkin's disease  a form of malignant lymphoma marked clinically by painless, progressive enlargement of lymph nodes, spleen, and general lymphoid tissue; other symptoms may include anorexia, lassitude, weight loss, fever, pruritus, night sweats, and anemia. Reed-Sternberg cells are characteristically present. Four types have been distinguished on the basis of histopathologic criteria.

hoof-and-mouth disease  foot-and-mouth d.

hookworm disease  infection with the hookworm Ancylostoma duodenale or Necator americanus, whose larvae enter the body through the skin or in contaminated food or water and migrate to the small intestine where, as adults, they attach to the mucosa and ingest blood; symptoms may include abdominal pain, diarrhea, colic or nausea, and anemia.

hyaline membrane disease  a type of respiratory distress syndrome of the newborn in which there is formation of a hyaline-like membrane lining the terminal respiratory passages; extensive atelectasis is attributed to lack of surfactant.

hydatid disease  an infection, usually of the liver, due to larval forms of tapeworms of the genus Echinococcus, marked by development of expanding cysts.

hypophosphatemic bone disease  an inherited disorder resembling a mild form of X-linked hypophosphatemia, similarly due to a defect in renal tubular function but usually showing osteomalacia without radiographic evidence of rickets.

immune complex disease  local or systemic disease caused by the formation of circulating immune complexes and their deposition in tissue, due to activation of complement and to recruitment and activation of leukocytes in type III hypersensitivity reactions.

infectious disease  one due to organisms ranging in size from viruses to parasitic worms; it may be contagious in origin, result from nosocomial organisms, or be due to endogenous microflora from the nose and throat, skin, or bowel.

inflammatory bowel disease  any idiopathic inflammatory disease of the bowel, such as Crohn's disease and ulcerative colitis.

intercurrent disease  one occurring during the course of another disease with which it has no connection.

iron storage disease  hemochromatosis.

ischemic bowel disease  ischemic colitis.

ischemic heart disease  (IHD) any of a group of acute or chronic cardiac disabilities resulting from insufficient supply of oxygenated blood to the heart.

Janský-Bielschowsky disease  the late infantile form of neuronal ceroid lipofuscinosis, occurring between two and four years of age, characterized by abnormal accumulation of lipofuscin; beginning as myoclonic seizures and progressing to neurologic and retinal deterioration and death by age 8 to 12.

jumping disease  any of several culture-specific disorders characterized by exaggerated responses to small stimuli, muscle tics including jumping, obedience even to dangerous suggestions, and sometimes coprolalia or echolalia.

juvenile Paget disease  hyperostosis corticalis deformans juvenilis.

Kashin-Bek (Kaschin-Beck) disease  a disabling degenerative disease of the peripheral joints and spine, endemic in northeastern Asia; believed to be caused by ingestion of cereal grains infected with the fungus Fusarium sporotrichiella.

Katayama disease  schistosomiasis japonica.

Kawasaki disease  a febrile illness usually affecting infants and young children, with conjunctival injection, changes to the oropharyngeal mucosa, changes to the peripheral extremities including edema, erythema, and desquamation, a primarily truncal polymorphous exanthem, and cervical lymphadenopathy. It is often associated with vasculitis of the large coronary vessels.

Kienböck's disease  slowly progressive osteochondrosis of the lunate bone; it may affect other wrist bones.

kinky hair disease  Menkes' syndrome.

Köhler's bone disease 

1. osteochondrosis of the tarsal navicular bone in children.

2. thickening of the shaft of the second metatarsal bone and changes about its articular head, with pain in the second metatarsophalangeal joint on walking or standing.

Krabbe's disease  a lysosomal storage disease beginning in infancy, due to deficiency of β-galactosidase. Pathologically, there is rapidly progressive cerebral demyelination and large globoid bodies (swollen with accumulated cerebroside) in the white substance.

Kufs' disease  the adult form of neuronal ceroid lipofuscinosis, with onset prior to age 40; characterized by progressive neurologic deterioration but not blindness, excessive storage of lipofuscin, and shortened life expectancy;

Kümmell's disease  compression fracture of vertebra, with symptoms a few weeks after injury, including spinal pain, intercostal neuralgia, lower limb motor disturbances, and kyphosis.

Kyasanur Forest disease  a fatal viral disease of monkeys in the Kyasanur Forest of India, communicable to humans, in whom it produces hemorrhagic symptoms.

Kyrle's disease  a chronic disorder of keratinization marked by keratotic plugs that develop in hair follicles and eccrine ducts, penetrating the epidermis and extending down into the corium, causing foreign-body reaction and pain.

Lafora's disease  see under epilepsy.

Leber's disease 

1. Leber's hereditary optic neuropathy.

2. Leber's congenital amaurosis.

legionnaires' disease  an often fatal bacterial infection caused by Legionella pneumophila, not spread by person-to-person contact, characterized by high fever, gastrointestinal pain, headache, and pneumonia; there may also be involvement of the kidneys, liver, and nervous system.

Leiner's disease  a disorder of infancy characterized by generalized seborrhea-like dermatitis and erythroderma, severe intractable diarrhea, recurrent infections, and failure to thrive.

Leriche disease  post-traumatic osteoporosis.

Letterer-Siwe disease  a Langerhans cell histiocytosis of early childhood, of autosomal recessive inheritance, characterized by cutaneous lesions resembling seborrheic dermatitis, hemorrhagic tendency, hepatosplenomegaly, lymphadenitis, and progressive anemia. If untreated it is rapidly fatal. Called also acute disseminated Langerhans cell histiocytosis.

Libman-Sacks disease  see under endocarditis.

Lindau's disease , Lindau-von Hippel disease von Hippel-Lindau d.

Little's disease  congenital spastic stiffness of the limbs, a form of cerebral palsy due to lack of development of the pyramidal tracts.

Lobstein's disease  see osteogenesis imperfecta.

Lou Gehrig disease  amyotrophic lateral sclerosis.

Lowe disease  oculocerebrorenal syndrome.

Lutz-Splendore-Almeida disease  paracoccidioidomycosis.

Lyme disease  a recurrent multisystemic disorder caused by the spirochete Borrelia burgdorferi, the vectors being the ticks Ixodes scapularis and I. pacificus; usually initially characterized by lesions of erythema chronicum migrans, followed by various manifestations including arthritis of the large joints, myalgia, and neurologic and cardiac abnormalities.

lysosomal storage disease  an inborn error of metabolism with (1) a defect in a specific lysosomal enzyme; (2) intracellular accumulation of an unmetabolized substrate; (3) clinical progression affecting multiple tissues or organs; (4) considerable phenotypic variation within a disease.

MAC disease  complex d.

McArdle disease  glycogen storage d., type V.

mad cow disease  bovine spongiform encephalopathy.

Madelung's disease 

1. see under deformity.

2. see under neck.

maple bark disease  hypersensitivity pneumonitis in logging and sawmill workers due to inhalation of spores of a mold, Cryptostroma corticale, growing under the maple bark.

maple syrup urine disease  (MSUD) a hereditary enzyme defect in metabolism of branched chain amino acids, marked clinically by mental and physical retardation, severe ketoacidosis, feeding difficulties, and a characteristic maple syrup odor in the urine and on the body.

Marburg virus disease  a severe, often fatal, viral hemorrhagic fever first reported in Marburg, Germany, among laboratory workers exposed to African green monkeys.

Marchiafava-Micheli disease  paroxysmal nocturnal hemoglobinuria.

Marie-Bamberger disease  hypertrophic pulmonary osteoarthropathy.

Marie-Strümpell disease  ankylosing spondylitis.

Marie-Tooth disease  Charcot-Marie-Tooth d.

Mediterranean disease  thalassemia major.

medullary cystic disease  familial juvenile nephronophthisis.

Meniere's disease  deafness, tinnitus, and dizziness, in association with nonsuppurative disease of the labyrinth.

mental disease  see under disorder.

Merzbacher-Pelizaeus disease  Pelizaeus-Merzbacher d.

metabolic disease  one caused by a disruption of a normal metabolic pathway because of a genetically determined enzyme defect.

Meyer's disease  adenoid vegetations of the pharynx.

Mikulicz's disease  benign, self-limited lymphocytic infiltration and enlargement of the lacrimal and salivary glands of uncertain etiology.

Milroy disease  hereditary permanent lymphedema of the legs due to lymphatic obstruction.

Minamata disease  a severe neurologic disorder due to alkyl mercury poisoning, with permanent neurologic and mental disabilities or death; once prevalent among those eating contaminated seafood from Minamata Bay, Japan.

minimal change disease  subtle alterations in kidney function demonstrable by clinical albuminuria and the presence of lipid droplets in cells of the proximal tubules, seen primarily in young children.

mixed connective tissue disease  a combination of scleroderma, myositis, systemic lupus erythematosus, and rheumatoid arthritis, and marked serologically by the presence of antibody against extractable nuclear antigen.

Möbius disease  ophthalmoplegic migraine.

molecular disease  any disease in which the pathogenesis can be traced to a single molecule, usually a protein, which is either abnormal in structure or present in reduced amounts.

Mondor's disease  phlebitis affecting the large subcutaneous veins normally crossing the lateral chest wall and breast from the epigastric or hypochondriac region to the axilla.

Monge's disease  chronic mountain sickness.

Morquio's disease , Morquio-Ullrich disease see under syndrome.

motor neuron disease , motor system disease any disease of a motor neuron, including spinal muscular atrophy, progressive bulbar paralysis, amyotrophic lateral sclerosis, and lateral sclerosis.

Mycobacterium avium complex disease  MAC disease; systemic disease caused by infection with organisms of the Mycobacterium avium-intracellulare complex in patients with human immunodeficiency virus infection.

Newcastle disease  a viral disease of birds, including domestic fowl, transmissible to humans, characterized by respiratory, gastrointestinal or pulmonary, and encephalitic symptoms.

new variant Creutzfeldt-Jakob disease  (nvCJD) a variant of Creutzfeldt-Jakob disease having a younger age of onset than is seen in Creutzfeldt-Jakob disease, and caused by the same agent that causes bovine spongiform encephalopathy.

Nicolas-Favre disease  lymphogranuloma venereum.

Niemann's disease , Niemann-Pick disease a lysosomal storage disease due to sphingomyelin accumulation in the reticuloendothelial system; there are five types distinguished by age of onset, amount of central nervous system involvement, and degree of enzyme deficiency.

nil disease  minimal change d.

Norrie's disease  an X-linked disorder consisting of bilateral blindness from retinal malformation, mental retardation, and deafness.

notifiable disease  one required to be reported to federal, state, or local health officials when diagnosed, because of infectiousness, severity, or frequency of occurrence.

oasthouse urine disease  methionine malabsorption syndrome.

obstructive small airways disease  chronic bronchitis with irreversible narrowing of the bronchioles and small bronchi with hypoxia and often hypercapnia.

occupational disease  disease due to various factors involved in one's employment.

Oguchi's disease  a form of hereditary night blindness and fundus discoloration following light adaptation.

organic disease  one associated with demonstrable change in a bodily organ or tissue.

Osgood-Schlatter disease  osteochondrosis of the tuberosity of the tibia.

Osler's disease 

1. polycythemia vera.

2. hereditary hemorrhagic telangiectasia.

Owren's disease  parahemophilia.

Paget's disease 

1. (of bone) osteitis deformans.

2. (of breast) an intraductal inflammatory carcinoma of the breast, involving the areola and nipple.

3. an extramammary counterpart of Paget's disease (2), usually involving the vulva, and sometimes other sites, as the perianal and axillary regions.

Parkinson's disease  a slowly progressive form of parkinsonism, usually seen late in life, marked by masklike facies, tremor of resting muscles, slowing of voluntary movements, festinating gait, peculiar posture, muscular weakness, and sometimes excessive sweating and feelings of heat.

Parrot's disease  see under pseudoparalysis.

parrot disease  psittacosis.

Parry's disease  Graves' d.

Pelizaeus-Merzbacher disease  a progressive familial form of leukoencephalopathy, marked by nystagmus, ataxia, tremor, parkinsonian facies, dysarthria, and mental deterioration.

Pellegrini's disease , Pellegrini-Stieda disease calcification of the medial collateral ligament of the knee due to trauma.

pelvic inflammatory disease  (PID) any pelvic infection involving the upper female genital tract beyond the cervix.

periodontal disease  any disease or disorder of the periodontium.

Perthes' disease  osteochondrosis of capitular femoral epiphysis.

Peyronie's disease  induration of the corpora cavernosa of the penis, producing a painful fibrous chordee and penile curvature.

Pfeiffer's disease  infectious mononucleosis.

Pick's disease 

1. progressive atrophy of the cerebral convolutions in a limited area (lobe) of the brain, with clinical manifestations and course similar to Alzheimer's disease.

2. Niemann-Pick d.

polycystic kidney disease , polycystic disease of kidneys either of two unrelated heritable disorders marked by cysts in both kidneys: the autosomal dominant or adult form is more common, appears in adult life, and is marked by loss of renal function that can be either rapid or slow; the autosomal recessive or infantile form is more rare, may be congenital or may appear later in childhood, and almost always progresses to renal failure.

polycystic renal disease  polycystic kidney d..

Pompe's disease  glycogen storage d., type II.

Pott's disease  spinal tuberculosis.

primary electrical disease  serious ventricular tachycardia, and sometimes ventricular fibrillation, in the absence of recognizable structural heart disease.

prion disease  any of a group of fatal, transmissible neurodegenerative diseases, which may be sporadic, familial, or acquired, caused by abnormalities of prion protein metabolism resulting from mutations in the prion protein gene or from infection with pathogenic forms of the protein.

pulseless disease  Takayasu's arteritis.

Raynaud's disease  a primary or idiopathic vascular disorder, most often affecting women, marked by bilateral attacks of Raynaud's phenomenon.

Recklinghausen's disease 

1. neurofibromatosis.

2. (of bone) osteitis fibrosa cystica generalisata.

Refsum's disease  an inherited disorder of lipid metabolism, characterized by accumulation of phytanic acid, chronic polyneuritis, retinitis pigmentosa, cerebellar ataxia, and persistent elevation of protein in cerebrospinal fluid.

remnant removal disease  familial dysbetalipoproteinemia.

reversible obstructive airway disease  a condition characterized by bronchospasm reversible by intervention, as in asthma.

rheumatic heart disease  the most important manifestation and sequel to rheumatic fever, consisting chiefly of valvular deformities.

rheumatoid disease  a systemic condition best known by its articular involvement (rheumatoid arthritis) but emphasizing nonarticular changes, e.g., pulmonary interstitial fibrosis, pleural effusion, and lung nodules.

Ritter's disease  dermatitis exfoliativa neonatorum.

Roger's disease  a ventricular septal defect; the term is usually restricted to small, asymptomatic defects.

runt disease  a graft-versus-host disease produced by immunologically competent cells in a foreign host that is unable to reject them, resulting in gross retardation of host development and in death.

Salla disease  an inherited disorder of sialic acid metabolism characterized by accumulation of sialic acid in lysosomes and excretion in the urine, mental retardation, delayed motor development, and ataxia.

Sandhoff's disease  a type of GM2 gangliosidosis resembling Tay-Sachs disease, seen in non-Jews, marked by a progressively more rapid course, and due to a defect in hexosaminidase, both isozymes A and B.

Schamberg's disease  a slowly progressive purpuric and pigmentary disease of the skin affecting chiefly the shins, ankles, and dorsa of the feet.

Schilder's disease  subacute or chronic leukoencephalopathy in children and adolescents, similar to adrenoleukodystrophy; massive destruction of the white substance of the cerebral hemispheres leads to blindness, deafness, bilateral spasticity, and mental deterioration.

Schönlein's disease  see under purpura.

secondary disease 

1. one subsequent to or as a consequence of another disease.

2. one due to introduction of incompatible, immunologically competent cells into a host rendered incapable of rejecting them by heavy exposure to ionizing radiation.

self-limited disease  one that runs a limited and definite course.

serum disease  see under sickness.

severe combined immunodeficiency disease  (SCID) see under immunodeficiency .

sexually transmitted disease  venereal disease; any of a diverse group of infections transmitted by sexual contact; in some this is the only important mode of transmission, and in others transmission by nonsexual means is possible.

sickle cell disease  any disease associated with the presence of hemoglobin S.

Simmonds' disease  see panhypopituitarism.

sixth disease  exanthema subitum.

small airways disease  chronic obstructive bronchitis with irreversible narrowing of the bronchioles and small bronchi. See also obstructive small airways d.

Smith-Strang disease  methionine malabsorption syndrome.

Spielmeyer-Vogt disease  Vogt-Spielmeyer d.

Steinert's disease  myotonic dystrophy.

Still's disease  juvenile rheumatoid arthritis.

storage disease  a metabolic disorder in which a specific substance (a lipid, a protein, etc.) accumulates in certain cells in unusually large amounts.

storage pool disease  a blood coagulation disorder due to failure of the platelets to release adenosine diphosphate (ADP) in response to aggregating agents; characterized by mild bleeding episodes, prolonged bleeding time, and reduced aggregation response to collagen or thrombin.

Strümpell's disease 

1. hereditary lateral sclerosis with the spasticity mainly limited to the legs.

2. cerebral poliomyelitis.

Strümpell-Leichtenstern disease  hemorrhagic encephalitis.

Strümpell-Marie disease  ankylosing spondylitis.

Sutton's disease 

1. halo nevus.

2. periadenitis mucosa necrotica recurrens.

3. granuloma fissuratum.

Swift's disease , Swift-Feer disease acrodynia.

Takayasu's disease  see under arteritis .

Tangier disease  a familial disorder characterized by a deficiency of high-density lipoproteins in the blood serum, with storage of cholesteryl esters in tissues.

Tarui's disease  glycogen storage d., type VII.

Tay-Sachs disease  (TSD) the most common GM2 gangliosidosis, seen almost exclusively in northeastern European Jews, characterized by infantile onset, doll-like facies, cherry-red macular spot, early blindness, hyperacusis, macrocephaly, seizures, hypotonia, and death in early childhood.

Thomsen's disease  myotonia congenita.

thyrotoxic heart disease  heart disease associated with hyperthyroidism, marked by atrial fibrillation, cardiac enlargement, and congestive heart failure.

transmissible neurodegenerative disease  prion d..

trophoblastic disease  gestational trophoblastic neoplasia.

tsutsugamushi disease  scrub typhus.

tunnel disease  decompression sickness.

uremic bone disease  renal osteodystrophy.

venereal disease  sexually transmitted d.

venoocclusive disease of the liver  symptomatic occlusion of the small hepatic venules caused by ingestion of Senecio tea or related substances, by certain chemotherapy agents, or by radiation.

vinyl chloride disease  acro-osteolysis resulting from exposure to vinyl chloride, characterized by Raynaud's phenomenon and skin and bony changes on the limbs.

Vogt-Spielmeyer disease  the juvenile form of neuronal ceroid lipofuscinosis with onset between ages 5 and 10 years; characterized by rapid cerebroretinal degeneration, excessive neuronal storage of lipofuscin, and death within 10 to15 years.

Volkmann's disease  congenital deformity of the foot due to tibiotarsal dislocation.

von Hippel's disease  hemangiomatosis confined principally to the retina; when associated with hemangioblastoma of the cerebellum, it is known as von Hippel-Lindau d.

von Hippel-Lindau disease  a hereditary condition marked by hemangiomas of the retina and hemangioblastomas of the cerebellum, sometimes with similar lesions of the spinal cord and cysts of the viscera; there may be neurologic symptoms such as seizures and mental retardation.

von Willebrand's disease  an autosomal dominant bleeding disorder characterized by prolonged bleeding time, deficiency of von Willebrand's factor, and often impairment of adhesion of platelets on glass beads, associated with epistaxis and increased bleeding after trauma or surgery, menorrhagia, and postpartum bleeding.

Waldenström's disease  osteochondrosis of the capitular femoral epiphysis.

Weber-Christian disease  nodular nonsuppurative panniculitis.

Werlhof's disease  idiopathic thrombocytopenic purpura.

Wernicke's disease  see under encephalopathy.

Westphal-Strümpell disease  hepatolenticular degeneration.

Whipple's disease  a malabsorption syndrome marked by diarrhea, steatorrhea, skin pigmentation, arthralgia and arthritis, lymphadenopathy, central nervous system lesions, and infiltration of the intestinal mucosa with macrophages containing PAS-positive material.

Whitmore's disease  melioidosis.

Wilson's disease  an inherited, progressive disorder of copper metabolism, with accumulation of copper in liver, brain, kidney, cornea, and other tissues; it is characterized by cirrhosis in the liver, degenerative changes in the brain, and a pigmented ring at the outer margin of the cornea.

Wolman's disease  a lysosomal storage disease due to deficiency of the lysosomal sterol esterase, occurring in infants, and associated with hepatosplenomegaly, adrenal steatorrhea, calcification, abdominal distention, anemia, and inanition.

woolsorter's disease  inhalational anthrax.

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Alz·hei·mer's disease (ältsh-mrz, lts-, ôlts-)

n.

A degenerative disease of the brain, associated with the development of abnormal tissues and protein deposits in the cerebral cortex and characterized by confusion, disorientation, memory failure, speech disturbances, and the progressive loss of mental capacity.

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Alzheimer's disease (AD)

[ôl′zīmərz]

Etymology: Alois Alzheimer, German neurologist, 1864-1915

a condition characterized by progressive mental deterioration, often with confusion, memory failure, disorientation, restlessness, agnosia, speech disturbances, inability to carry out purposeful movement, and hallucinosis. The patient may become hypomanic, refuse food, and lose sphincter control without focal impairment. The disease sometimes begins in middle life with slight defects in behavior and memory, usually an inability to incorporate new knowledge with old knowledge, but the symptoms can worsen dramatically after the age of 70. When the symptoms are severe, patients are unable to perform activities of daily living or orient to surroundings and do not recognize loved ones. Although it occurs with equal frequency in men and women, the familial risk is four times that of the general population. Typical pathologic features are miliary amyloid plaques in the cortex and fibrillary degeneration (tangles) in layers containing pyramidal ganglion cells. The cerebral cortex atrophies with widening of the cerebral sulci, especially in the frontal and temporal regions. Diagnostic criteria consist of a failure in at least three cognitive functions, including memory, use of language, visuospatial skills, personality, and calculating skills. Treatment may involve medications that are thought to decrease the rate of decline. Also called senile dementia-Alzheimer type (SDAT).

nursing considerations Nursing care is initially concerned primarily with promoting activity and sleep and preventing agitation, violence, and injury. Death usually occurs 8 to 12 years after the first symptoms appear. Late-stage nursing care is focused on palliative measures.

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Alzheimer's disease,

n.pr a presenile dementia characterized by con-fusion, memory failure, disorientation, restlessness, agnosia, hallucinosis, speech disturbances, and the inability to carry out purposeful movement. The disease usually begins in later middle life with slight defects in memory and behavior that become progressively more severe. Also known as primary progressive aphasia.

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Alzheimer's disease

Alzheimer's dementia Neurology A progressive, neurodegenerative disease, which is the most common cause of dementia, and characterized by progressive mental deterioration accompanied by disorientation, memory and language defects, confusion, leading to progressive dementia, which may be accompanied by dysphasia, and apraxia; in the DSM-IV, AD '…is …a diagnosis of exclusion, and all other causes (of) cognitive defects … must first be ruled out.' AD affects 3% in those aged 65–74; 18% aged 75–84; 47% > age 85; AD has been linked to a form of apolipoprotein E, those with 1 defective APOE ε4 gene–located on chromosome 19 are at an ↑ risk of AD, and those with 2 copies have AD of early onset; A68 protein is present in AD brain homogenates, and linked to formation of neuritic plaques and neurofibrillary tangles–68 kD; one Alzheimer's amyloid precursor–APP may inhibit serine proteases, and is generated by alternative enzyme splicing, releasing an intact β fragment, possibly explaining the deposition of these fibrils in AD brains Management Tetrahydroaminoacridine-THA, aka tacrine, an acetylcholinesterase inhibitor, may improve the quality of life in AD Pts. See Tacrine.

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Patient discussion about Alzheimer's.

Q. how do you know if you have early onset of alzheimers? i'm 47. i do have extreme tremors at times and memory l i was told this could be what i have by a psychiatrist. What else can cause me to have these symptoms at my age and how do i know?

A. Some early signs of Alzheimer's disease are:
Trouble remembering recent events.
Problems remembering namesof people and places.
Trouble solving simple math problems.

Alzheimer's disease usually affects people over 60. There are some illnesses and problems that may look like Alzheimer’s, but are caused by other problems.
These include:

Bad reaction to certain medicines
Depression
Not eating enough healthy foods, or too few vitamins and minerals in your body
Brain tumors
Blood vessel disease
Thyroid problems

Some of these illnesses can be treated. Once treated, your confusion and memory loss should go away. The point is to get checked out by your doctor. Don’t let fear stop you from seeing your doctor. You can get help.

Q. Can you tell me more about Brain games? There are many new brain games now advertised by Nintendo and others. Are they doing anything to delay Alzheimer’s

A. This is a question that is often asked by individuals that are tuned in to innovations. Indeed, many of the software programs marketed today are designed to exercise a broad range of thinking and information processing brain skills. They provide an opportunity to activate different areas of the brain that people typically do not use in their routine daily life. It is important to seek out opportunities for mental challenge and stimulation. When looking for such a program, look for a program exercising a broad set of skills and check that the company has a strong scientific advisory board behind it. Also, try the exercises to see that you are having fun while exercising. Otherwise, you are unlikely to continue to do them. I liked My Vigorous Mind because it was very easy to use, they have many activities and it is fun. Lumosity is another fun program and MindFit is interesting as it tailors a training program for each user.

Q. I want to improve my memory. I do a Sudoku every day and crossword puzzles. Do I need to do anything else?

A. I often encounter this question, and I wanted to share it with you. These are very good activities that stimulate the brain. You want to engage in additional mental activities that require other types of thinking in order to engage more of your mental capacity. Most importantly, maintaining brain health also depends on other components of healthy life style, such as being physically active, keeping a healthy diet, and reducing stress levels.