$Unique_ID{BRK03580} $Pretitle{} $Title{Cerebellar Degeneration, Subacute} $Subject{Cerebellar Degeneration Subacute Subacute Cerebellar Degeneration Alcoholic Nutritional Cerebellar Degeneration Multiple Sclerosis Wernicke Encephalopathy Paraneoplastic Cerebellar Degeneration} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 795: Cerebellar Degeneration, Subacute ** IMPORTANT ** It is possible that the main title of the article (Subacute Cerebellar Degeneration) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Subacute Cerebellar Degeneration Disorder Subdivisions: Alcoholic or Nutritional Cerebellar Degeneration Information on the following disorders can be found in the Related Disorders section of this report: Multiple Sclerosis Wernicke Encephalopathy Paraneoplastic Cerebellar Degeneration 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. Subacute Cerebellar Degeneration is characterized by deterioration of the cerebellum (an area of the brain concerned especially with muscle coordination and balance). It may also involve the area connecting the spinal cord to the brain such as the medulla oblongata, the cerebral cortex, and possibly the brain stem. There are two subtypes of this disorder. The first is Paraneoplastic Cerebellar Degeneration which sometimes precedes cancer. The second is Alcoholic or Nutritional Cerebellar Degeneration which is caused by a lack of the vitamin B-1 (thiamine) and is not related to cancer. Symptoms Common symptoms of Subacute Cerebellar Degeneration include: impaired muscle coordination (ataxia) of the limbs (especially of the arms in Paraneoplastic Cerebellar Degeneration, and of the legs in Alcoholic or Nutritional Cerebellar Degeneration) difficulty in articulation of speech (dysarthria), which is especially noticeable in Paraneoplastic Cerebellar Degeneration difficulty in swallowing (dysphagia) loss of reason (dementia), occurring in approximately half the patients with Paraneoplastic Cerebellar Degeneration involuntary rapid movements of the eyeball in a horizontal or vertical direction (nystagmus) double-vision (diplopia) vertigo (dizziness) paralysis of the eye muscles (ophthalmoplegia) difficulty in walking if the patient has Alcoholic/Nutritional Cerebellar Degeneration In Subacute Cerebellar Degeneration, there is a loss of nerve cells (Purkinje cells) throughout the cerebellum. A CT scan may show enlargement of the fourth ventricle which is the area in the brain between the spinal cord and the rest of brain, as well as areas of the cerebellum. Examination of cerebrospinal fluid is usually normal except that it may show a high amount of lymph cells (white blood cells formed in lymphoid tissue) and an elevated protein level. Causes The causes of Subacute Cerebellar Degeneration are as follows. Paraneoplastic Cerebellar Degeneration may be an autoimmune disorder. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack healthy tissue. Alcoholic/Nutritional Cerebellar Degeneration is associated with a thiamine deficiency. Secondary thiamine deficiency results from increased requirements for thiamine, and from impaired absorption or impaired utilization of the vitamin. Alcoholics tend to eat few thiamine containing foods, they may absorb or utilize the vitamin poorly, and they normally require larger than normal amounts of thiamine. Affected Population In Paraneoplastic Cerebellar Degeneration, the average age of onset is 50, with males affected more often than females. This form of cerebellar degeneration may precede cancer. Alcoholic or Nutritional Cerebellar Degeneration affects alcoholics and people with thiamine deficiency. It is not related to cancer and is more common than the paraneoplastic type. Related Disorders Symptoms of the following disorders can be similar to those of Subacute Cerebellar Degeneration. Comparisons may be useful for a differential diagnosis: Multiple Sclerosis is a chronic disease of the central nervous system, which may be progressive, relapsing and remitting, or stable. MS is characterized by small lesions called plaques that form randomly throughout the brain and spinal cord. These patches prevent proper transmission of nervous system signals and thus result in a variety of neurological symptoms including visual difficulties (blind spots, double vision, nystagmus), impairment of speech, abnormal skin sensations (paresthesias) or numbness, walking disturbance and difficulties with bladder or bowel function. (For more information on this disorder, choose "Multiple Sclerosis" as your search term in the Rare Disease Database). Wernicke Encephalopathy is a degenerative brain disorder characterized by a deficiency of thiamine. It is marked by loss of coordination (ataxia) and apathy, confusion, disorientation or delirium. Various vision dysfunctions may also develop. This disorder often occurs in conjunction with Korsakoff Syndrome which involves a Vitamin B1 (thiamine) deficiency usually caused by alcoholism. Wernicke Encephalopathy can be severely disabling and life threatening if it is not recognized and treated early. (For more information, on this disorder, choose "Korsakoff" or "Wernicke" as your search term in the Rare Disease Database). Paraneoplastic Cerebellar Degeneration is associated with and often may precede the development of several types of cancer. It occurs most often in patients with lung cancer (especially small-cell carcinoma), but may also occur in patients with cancers of the ovary, breast, stomach, or uterus, as well as in patients with Hodgkin's Disease. Hodgkin's Disease is a form of cancer of the lymphatic system, especially the lymph nodes. Tumors occur in the lymph nodes (places where lymphatic vessels unite) and/or the area around the nodes. Fever, night sweats, and weight loss may occur along with swollen lymph nodes. (For more information on this disorder, choose "Hodgkin's Disease" as your search term in the Rare Disease Database). Therapies: Standard Paraneoplastic Cerebellar Degeneration may improve after successful treatment of the underlying cancer. For Alcoholic/Nutritional Cerebellar Degeneration, thiamine is given along with other B vitamins, usually relieving the condition if the patient stops drinking alcohol and resumes a normal diet. In cases of X-linked inherited Subacute Cerebellar Degeneration, genetic counseling may be of benefit for patients and their families. Other treatment is symptomatic and supportive. Therapies: Investigational The National Cancer Institute conducts clinical trials on new drugs being tested for Hodgkin's Disease and other forms of cancer. To learn about the locations of these investigations, contact the Cancer Information Service, 1- 800-4-CANCER. This disease entry is based upon medical information available through March 1991. 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 Subacute Cerebellar Degeneration, please contact: National Organization for Rare Disorders P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 NIH/National Cancer Institute 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give the public, cancer patients and families, and health professionals quick and easy access to many types of information vital to patients with this and many other types of cancer. To gain access to this service, call: Cancer Information Service (CIS) 1-800-4-CANCER In Washington, DC and suburbs in Maryland and Virginia, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect) For genetic information and genetic counseling referrals: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 997, 999, 2250. MENDELIAN INHERITANCE IN MAN, 8th Ed.: Victor A. McKusick; Johns Hopkins University Press, 1986. Pp. 1262. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck, Sharp, and Dohme Laboratories, 1982. Pp. 1365, 1411-1412. CHARACTERIZATION OF A cDNA ENCODING A 34-kDA PURKINJE NEURON PROTEIN RECOGNIZED BY SERA FROM PATIENTS WITH PARANEOPLASTIC CEREBELLAR DEGENERATION. H.M. Furneaux, et al.; Proc Natl Acad Sci USA (April 1989; issue 86 (8)). Pp. 2873-2877. A QUANTITATIVE HISTOLOGICAL STUDY OF THE CEREBELLAR VERMIS IN ALCOHOLIC PATIENTS. S.C. Phillips, et al.; Brain (April 1987; issue 110 (Pt 2)). Pp. 301-314.