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$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.