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Alternative names Return to top
Lobar sclerosis; Circumscribed brain atrophy; Fronto-temporal dementia; Arnold Pick's disease; Focal cerebral atrophyDefinition Return to top
Pick's disease produces deterioration in mental function caused by loss of brain tissue in discrete areas (focal lesions). It is marked by the presence of abnormalities in brain cells (Pick's bodies) which can be found in the affected areas and elsewhere in the brain.
Causes, incidence, and risk factors Return to top
Pick's disease is a rare disorder similar to senile dementia/Alzheimer's type. What differentiates it from Alzheimer's disease is that it seems to affect predominantly circumscribed areas of the brain, not all regions. Alzheimer's disease is a more diffuse process that can affect any part of the brain as it progresses.
Pick's disease affects about 1 out of 100,000 people. It is more common in women than men. It may occur in people as young as 20, but usually begins between ages 40 and 60. The average age of onset is 54.
The onset is usually slow and insidious. The disorder involves shrinking of the tissues (atrophy) of the frontal and temporal lobes of the brain, "fronto-temporal dementia." The neurons (nerve cells) in the affected areas contain abnormal material (Pick's bodies). These are tangles made of tau protein. The exact cause is unknown.
The symptoms may be similar to Alzheimer's, with aphasia (loss of language abilities), agnosia (loss of ability to recognize objects or people), and apraxia (loss of skilled movement abilities). Behavioral changes are prominent with loss of inhibition and change in personality, as opposed to Alzheimer's Disease where memory loss is often the primary feature.
Risk factors may include a having a personal or family history of Pick's disease or senile dementia, though the genetic basis of the disease has not yet been determined.
Symptoms Return to top
Movement/coordination difficulties (apraxia) -- may be one of the earliest symptomsSigns and tests Return to top
The health care provider bases the initial diagnosis on history and symptoms, signs, and tests, and by ruling out other causes of dementia including dementia due to metabolic causes. Neurologic examination may reveal signs that vary according to the which part of the brain is afffected. Temporal and frontal lobe signs are most common, with resulting behavioral and language changes.
There may be other abnormalities, including frontal release signs (presence of abnormal reflexes) and exacerbation of muscle stretch reflexes. Psychologic studies and tests of sensation, cognitive function, and motor function may be abnormal.
It is important to note that the definitive diagnosis can only be made by brain biopsy since the demonstration of Pick bodies must be done with a microscope by an experienced neuropathologist. However, ongoing efforts are directed at developing a biological marker that would allow a firm diagnosis without the need for such an invasive procedure.
Treatment Return to top
There is no proven effective treatment for Pick's disease. Monitoring and assistance with self-care may be required depending on symptoms exhibited and progression of the disorder.Expectations (prognosis) Return to top
The probable outcome is poor. The disorder progresses steadily and relatively rapidly. Total disability occurs early. Commonly, Pick's disease results in death within 2 to 10 years, usually from infection and occasionally from general failure of body systems.Complications Return to top
Calling your health care provider Return to top
Call your health care provider if symptoms of Pick's disease develop.Prevention Return to top
There is no known prevention for this rare disorder. Update Date: 4/22/2004 Updated by: Joseph V. Campellone, M.D., Division of Neurology, Cooper Hospital/University Medical Center, Camden, NJ. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |