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Paraneoplastic cerebellar degeneration

II. Clinical and immunologic findings in 21 patients with Hodgkin's disease

Departments of Neurology (Drs. Hammack and Posner) and Pathology (Dr. Rosenblum), and the George C. Cotzias Laboratory of Neuro-Oncology (Drs. Hammack and Posner, and H. Kotanides), Memorial Sloan-Kettering Cancer Center, and the Department of Neurology and Neuroscience (Drs. Hammack and Posner), Cornell University Medical College, New York, NY.

We reviewed clinical findings and serologic data on 18 men and three women with paraneoplastic cerebellar degeneration (PCD) associated with Hodgkin's disease (HD). The patients were 20 to 77 years old (median, 44). The lymphoma preceded neurologic symptoms by 1 to 54 months in 17/21 patients, but stage or activity did not correlate with severity of neurologic disease; six developed PCD while in HD remission. PCD evolved subacutely (over weeks to months) and was pancerebellar in most. Ten had downbeat nystagmus. Thirteen stabilized in a disabled state (wheelchair- or bed-bound), five stabilized ambulatory, and three, who had progressed to a nonambulatory state, recovered. The clinical findings were usually only cerebellar but one patient had an encephalopathy, three long-tract signs, and two sensory neuropathy. Plasmapheresis (seven patients) and corticosteroids or other immunosuppressant medication (eight patients) did not help; one improved dramatically after treatment with clonazepam. Two patients improved spontaneously. Six patients had serum antibodies that reacted specifically with Purkinje cells. The pattern was distinct from that of PCD with gynecologic cancer (anti-Yo) or small-cell lung cancer (anti-Hu). Western blotting failed to identify a discrete Purkinje cell antigen. Seropositive patients did not differ clinically from their seronegative counterparts. HD-associated PCD is more common in men and in a younger age group than in PCD with other malignancies.

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

Supported in part by NIH grants #NS26064 and the Charles A. Dana Foundation.

Presented in part at the 43rd annual meeting of the American Academy of Neurology, Boston, MA, April 1991.

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

Avenue, New York, NY 10021. Dr. Hammacks current address is Mayo Clinic, Rochester, MN.

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