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NEUROLOGY 1988;38:1642
© 1988 American Academy of Neurology

Delayed immune response in chorea-amyotrophy with spherocytosis

Hans L. Lagrèze, MD, Steven E. Kornguth, PhD, Benjamin Rix Brooks, MD and Ross L. Levine, MD

Neurology Service, William S. Middleton Memorial Hospital, and the Departments of Neurology and Physiologic Chemistry (Dr. Kornguth), University of Wisconsin Medical School, Madison, WI.

Progressive CSF lymphocytic pleocytosis and intrathecal IgG-synthesis occurred late in familial amyotrophy, neuropathy, chorea, and dementia with spherocytosis. Immunoblotting showed a serum and CSF antibody apparently directed against glial fibrillary acidic protein. A secondary autoimmune response was probably triggered during the evolution of the neurodegenerative process.

Address correspondence and reprint requests to Dr. Lagrèze, Universitäts-Nervenklinik, Sigmund-Freud-Str. 25, 5300 Bonn 1, West Germany.

Received February 11, 1988. Accepted for publication in final form April 13, 1988.






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