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NEUROLOGY 1992;42:1944
© 1992 American Academy of Neurology

Paraneoplastic cerebellar degeneration

III. Cerebellar degeneration, cancer, and the Lambert-Eaton myasthenic syndrome

P. D. Clouston, MB, BS, FRACP, C. B. Saper, MD, PhD, T. Arbizu MD, I. Johnston BSc, B. Lang PhD, J. Newsom-Davis MD, FRCP and J. B. Posner, MD

Departments of Neurology (Drs. Clouston and Posner), Memorial Sloan-Kettering Cancer Center and Cornell University Medical College, New York, Ny, Departments of Pharmacological and Physiological Sciences and Neurology (Dr. Saper), University of Chicago Hospitals, Chicago, IL; C.S. "Principes de España" (Dr. Arbizu), Barcelona, Spain; and the Neurosciences Group (Drs. Lang and Newsom-Davis, and I. Johnston), Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK.

We studied nine patients with a subacute onset of a pancerebellar syndrome. Six had known cancer (three small-cell carcinoma of the lung [SCLC], one metastatic small-cell carcinoma, one small-cell carcinoma of the prostate, and one non-Hodgkin's lymphoma). Six of eight who had neurophysiologic testing, including the three patients without detectable cancer, had coexistent Lambert-Eaton myasthenic syndrome (LEMS). In two of the patients, LEMS was discovered only by neurophysiologic testing. We looked for anti-Purkinje cell autoantibodies in all patients' sera and in four patients' CSF. We also looked for autoantibodies to voltage-gated calcium channels (VGCCs) in seven patients' sera and two patients' CSF, using the 125I-{omega}-conotoxin radioimmunoassay. We were unable to detect anti-Purkinje cell autoantibodies in any patients' serum or CSF. However, there were raised titers of anti-VGCC autoantibodies in five of seven patients' serum, including one patient with SCLC who did not have LEMS, and in the CSF of one of two patients. We conclude that the frequency of presentation of a pancerebellar syndrome with LEMS is higher than expected by chance and is usually associated with cancer. In some of these patients, LEMS may be clinically occult. The presence of LEMS and raised titers of anti-VGCC autoantibodies in some patients with subacute cerebellar degeneration is suggestive of an autoimmune etiology even though anti-Purkinje cell antibodies could not be detected. Anti-VGCC autoantibodies are not confined to LEMS. They may be found at high titer in CSF as well as serum.

Address correspondence and reprint requests to Dr. Jerome B. Posner, Department of Neurology, Memorial Sloan-Kettering Cancer Center, 1275 York

Supported in part by the Sir Jules Thorn Trust and by NIH Grant #NS26064

Received January 17, 1992. Accepted for publication in final form March 24, 1992

Avenue, New York NY 10021. Dr. Clouston's current address is Department of Neurology, Massachusetts General Hospital, Boston, MA.




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