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Neurology 2002;59:1985-1987
© 2002 American Academy of Neurology
Brief Communications

Cerebellar degeneration and autoimmunity to zinc-finger proteins of the cerebellum

L. Bataller, MD, D.F. Wade, BS, G.N. Fuller, MD, M.R. Rosenfeld, MD PhD and J. Dalmau, MD PhD

From the Departments of Neurology (Drs. Bataller, Rosenfeld, and Dalmau) and Microbiology and Immunology (Drs. Rosenfeld and Dalmau, and D.F. Wade), University of Arkansas for Medical Sciences, Little Rock, AR; Department of Pathology (Dr. Fuller), MD Anderson Cancer Center, Houston, TX; and Central Arkansas Veterans Health Care System (Dr. Rosenfeld), Little Rock, AR.

Address correspondence and reprint requests to Dr. Josep Dalmau, Department of Neurology, 3 W. Gates, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104; e-mail: jdalmau{at}aol.com

The serum of a patient with subacute cerebellar dysfunction was used to probe a cDNA expression library and isolate two genes: Zic1 (zinc-finger of the cerebellum) and Zic4. The patient had intrathecal synthesis of Zic antibodies, suggesting that the Zic proteins were autoantigens of the neurologic disorder. The Zic proteins are involved in cerebellar development and are reported as being preferentially expressed by medulloblastomas. It was found that the expression of Zic proteins is enriched in, but not limited to, medulloblastomas and primitive neuroectodermal tumors.




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L. Bataller, D. F. Wade, F. Graus, H. D. Stacey, M. R. Rosenfeld, and J. Dalmau
Antibodies to Zic4 in paraneoplastic neurologic disorders and small-cell lung cancer
Neurology, March 9, 2004; 62(5): 778 - 782.
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