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Cerebellar Degeneration, Subacute

Synonyms of Cerebellar Degeneration, Subacute

  • 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.

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.)

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

Organizations related to Cerebellar Degeneration, Subacute

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

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2007/09/23 00:00:00 GMT+0

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