$Unique_ID{BRK04108} $Pretitle{} $Title{Pick's Disease} $Subject{Pick's Disease Diffuse Degenerative Cerebral Disease Lobar Atrophy Alzheimer's Disease Huntington's Disease} $Volume{} $Log{} Copyright (C) 1989, 1990 National Organization for Rare Disorders, Inc. 673: Pick's Disease ** IMPORTANT ** It is possible that the main title of the article (Pick's Disease) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Diffuse Degenerative Cerebral Disease Lobar Atrophy Information on the following diseases can be found in the Related Disorders section of this report: Alzheimer's Disease Huntington's Disease General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Pick's Disease is a very rare progressive disease affecting the lobes of the brain. Major symptoms may include changes in intellect, behavior and personality. Symptoms Pick's Disease is characterized by progressive deterioration of intellect with changes in behavior and personality. The memory is usually intact in the early stages of the disease and there is less disorientation than in Alzheimer's disease. This brain disease is very closely related to Alzheimer's disease and can even coexist with Alzheimer's. People with Pick's Disease don't show the tangles and plaques in the brain that are characteristic of Alzheimer's. However, in later stages there is loss of motor control as well as confusion and severe dementia (loss of intellect). Pick's Disease is characterized by atrophy (shrinkage) of the brain, but the atrophy does not occur evenly over the entire brain. Only the lobes of the brain are affected while other areas of the brain appear unaffected. There are also changes in certain nerve cells in the brain and the presence of Pick's inclusion bodies. The reason for this is not known. The disease primarily affects individuals in their 50's, 60's and 70's, but early cases have been reported in people as young as 30 years of age. Diagnosis of Pick's disease is very difficult, and a neurologist should be consulted. Causes Most cases of Pick's Disease have been reported to occur for no apparent reason. However, some cases appear in families and this may indicate some genetic predisposition for the disease. So far researchers have not been able to located a mode of genetic transmission for this disorder. However, research on Alzheimer's disease and related dementias may shed some light on the cause of Pick's Disease. Affected Population Pick's Disease affects females more often than males. The incidence in the United States. indicates a much lower prevalence than Alzheimer's Disease. Studies from the University of Michigan have shown a dozen cases of Pick's Disease in 35 years. However, In Minnesota, Pick's Disease accounts for about 4% of dementias. This may well indicate geographical variance and there may possibly also be an ethnic relationship. Related Disorders Symptoms of the following disorders can be similar to those of Pick's Disease. Comparisons may be useful for a differential diagnosis: Alzheimer's Disease is a progressive disorder of the brain affecting memory, thought and language. Groups of nerve endings in the cortex of people with Alzheimer's degenerate which disrupts the passage of electrochemical signals between the cells. These areas of degeneration are called "plaques". Changes known as "neurofibrillary tangles" also occur in nerve cells of the brain's cortex. The number of plaques and tangles appear to be directly proportional to the disturbance in intellectual function and memory. Recently scientists discovered that Alzheimer's Disease can be inherited through a dominant gene in at least ten percent of affected individuals. Some researchers believe symptoms may be caused by excessive amounts of a protein that builds up in the brains of persons affected by this disorder. There is also abnormally low levels of the brain chemical (neurotransmitter) acetylcholine in patients with this disorder. (For more information on this disorder, choose "Alzheimer" as your search term in the Rare Disease Database). Huntington's Disease is an inherited, progressively degenerative neurological illness. Those affected experience involuntary movements, loss of motor control, changes in gait, loss of memory, and eventual loss of both mental capability and physical control. In general, the symptoms of HD first appear between thirty and fifty years of age. It runs a progressive course, usually over a ten to twenty year period. (For more information on this disorder, choose "Huntington" as your search term in the Rare Disease Database). Dementia can be a symptom of many disorders mimicking Alzheimer's and Pick's disease. To learn more about these illnesses choose "Dementia" as your search term in the Rare Disease Database). Therapies: Standard The diagnosis of Pick's Disease is usually made by a neurologist after taking a careful physical history and examination, a neurological examination, psychometric testing, CAT scan and EEG testing. Patients with progressive dementia often experience frustration, anxiety, and depression resulting from their inability to function at their previous level. These frustrations can be minimized by maintaining a stable home environment and a structured routine that does not place excessive demands on the patient. Sedatives or other drugs that might further dull the patient's intellect should generally be avoided. Other treatment is symptomatic and supportive. Therapies: Investigational The National Institute of Neurological Disorders and Stroke is seeking patients with Pick's Disease and other types of frontal lobe atrophy to participate in a neurobehavioral study. For more information, physicians should contact: Jordan Grafman, Ph.D., Chief Cognitive Neuroscience Section, Medical Neurology Branch NIH/National Institute of Neurological Disorders and Stroke (NINDS) Bldg. 10, Rm. 5C422 Bethesda, MD 20892 (301) 496-0220 This disease entry is based upon medical information available through November 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Pick's Disease, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Alzheimer's Disease and Related Disorders Association, Inc. National Headquarters 919 N. Michigan Ave., Suite 1000 Chicago, IL 60611 (312) 335-8700 (800) 272-3900 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 NIH/National Institute on Aging (NIA) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-1752 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 2150-2153.