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.
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.
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.
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.
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.
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: [email protected]
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
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 and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100
