Cerebellar Degeneration, Subacute

Cerebellar Degeneration, Subacute

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Cerebellar Degeneration, Subacute is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Subacute Cerebellar Degeneration

Disorder Subdivisions

  • Alcoholic Cerebellar Degeneration
  • Nutritional Cerebellar Degeneration

General Discussion

Subacute cerebellar degeneration (SCD) is characterized by the deterioration of the area of the brain concerned with muscle coordination and balance (the cerebellum). Less frequently, the area involved may include the area connecting the spinal cord to the brain (the medulla oblongata, the cerebral cortex, and the brain stem). There are two types of subacute cerebellar degeneration: paraneoplastic cerebellar degeneration, which sometimes precedes the diagnosis of cancer, and alcoholic or nutritional cerebellar degeneration, caused by a lack of the vitamin B-1 (thiamine). These two types share symptoms but not the same cause.

Symptoms

Common symptoms of subacute cerebellar degeneration include:



(1) weakened muscle coordination (ataxia) of the limbs (especially of the arms in paraneoplastic cerebellar degeneration, and of the legs in alcoholic or nutritional cerebellar degeneration);



(2) problems in articulation of speech (dysarthria), which are especially noticeable in paraneoplastic cerebellar degeneration;



(3) difficulty in swallowing (dysphagia);



(4) loss of reason (dementia); this occurs in approximately half the patients with paraneoplastic cerebellar degeneration;



(5) involuntary rapid movements of the eyeball in a horizontal or vertical direction (nystagmus); as well as double-vision (diplopia), vertigo (dizziness), paralysis of the eye muscles (ophthalmoplegia), and difficulty in walking if the patient has alcoholic/nutritional cerebellar degeneration.



In addition, patients with SCD lose many of a particular kind of nerve cell (Purkinje cells) throughout the cerebellum. CAT scans may show enlargement of the area of the brain between the spinal cord and the rest of brain (fourth ventricle) as well as areas of the cerebellum. Examination of cerebrospinal fluid may show a high volume 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 against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. In cases in which there is an underlying cancer, the individual's immune system may be responding to its presence by stimulating the body's natural cancer-fighting mechanisms (T-cells, which are a type of white blood cell) to attack normal cells in the nervous system.



Alcoholic/nutritional cerebellar degeneration is associated with 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 poorly and may not get enough thiamine- containing foods. Also, they seem to absorb or utilize the vitamin less efficiently and, therefore, may require larger than normal amounts of thiamine.

Affected Populations

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.

Standard Therapies

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.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1583, 1450.



Fauci AS, Braunwald E, Isselbacher KJ, et al. Eds. Harrison's Principles of Internal Medicine. 14th ed. McGraw-Hill Companies. New York, NY; 1998:2504, 625.



Adams RD, Victor M, Ropper AA. Eds. Principles of Neurology. 6th ed. McGraw-Hill Companies. New York, NY; 1997:1157,686-87.



JOURNAL ARTICLES

Bataller L, Wade DF, Fuller GN, et al. Cerebellar degeneration and autoimmunity to zinc-finger proteins of the cerebellum. Neurology. 2002;59:1985-87.



Leonovicz BM, Gordonm EA, Wass CT. Paraneoplastic syndromes associated with lung cancer: a unique case of concomitant subacute cerebellar degeneration and Lambert-Eaton myasthenic syndrome. Anesth Analg. 2001;93:1557-59.



Levite R, Fishman A, Kesler A, et al. Paraneoplastic cerebellar degeneration heralding fallopian tube adinocarcinoma. Int J Gynecol Cancer. 2001;11:169-71.



Mowzoon N, Bradley WG. Successful immunosuppressant therapy of severe progressive cerebellar degeneration and sensory neuropathy: a case report. J Neurol Sci. 2000;178:63-65.



FROM THE INTERNET

Mehdi A. Paraneoplastic Cerebellar Degeneration. EMedicine. Last Updated: January 30, 2002:9pp.

www.emedicine.com/neuro/topic299.htm



CNS Paraneoplastic Syndromes. The Merck Manual, Sec. 14, Ch. 177, CNS Neoplasms.

www.merck.com/pubs/mmanual/section14/chapter 177/177e.htm



Nutritional CNS Disorders.

www.akronchildrens.org/neuropathology/CHAPTER_EIGHT.html



NINDS Paraneoplastic Syndromes Information Page. Reviewed 03-19-2003. 3pp.

www.ninds.nih.gov/health_and_medical/disorders/paraneoplastic.htm

Resources

March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

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Tel: (914)997-4488

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Tel: (888)663-4637

Email: Askus@marchofdimes.com

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American Autoimmune Related Diseases Association, Inc.

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Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



American Cancer Society, Inc.

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Ste 6000

Atlanta, GA 30303

USA

Tel: (404)320-3333

Tel: (800)227-2345

TDD: (866)228-4327

Internet: http://www.cancer.org



NIH/National Institute on Aging

31 Center Drive, MSC 2292

Building 31

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Bethesda, MD 20892

Tel: (301)496-1752

Fax: (301)496-1072

Tel: (800)222-2225

TDD: (800)222-4225

Email: bapquery@nia.nih.gov

Internet: http://www.nih.gov/nia



Rare Cancer Alliance

1649 North Pacana Way

Green Valley, AZ 85614

USA

Internet: http://www.rare-cancer.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

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