Paraneoplastic cerebellar degeneration

Edward J Dropcho MD (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released February 22, 1994; last updated March 15, 2015; expires March 15, 2018

This article includes discussion of paraneoplastic cerebellar degeneration and PCD. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Paraneoplastic cerebellar degeneration is an uncommon but often devastating complication of a number of carcinomas and other neoplasms in adults. The syndrome is likely caused by a dysregulated autoimmune response directed against shared neuronal-tumor antigens. Many patients have circulating anti-neuronal autoantibodies that are valuable diagnostic markers. The immunopathogenesis of neuronal injury in paraneoplastic cerebellar degeneration is poorly understood. The author reviews the clinical features, autoimmune aspects, and practical management of this important condition.

Key points

 

• Paraneoplastic cerebellar degeneration can occur in association with virtually any neoplasm, but is most often linked to small cell lung cancer, breast carcinoma, ovarian carcinoma, and Hodgkin lymphoma.

 

• Paraneoplastic cerebellar degeneration may present as an isolated subacute pancerebellar syndrome, or it may be part of a multifocal encephalomyeloneuritis.

 

• Most, but not all, patients with paraneoplastic cerebellar degeneration have one of several circulating onconeural antibodies, which, if present, are a valuable diagnostic marker and can guide the search for an underlying neoplasm.

 

• Paraneoplastic cerebellar degeneration is generally a disabling condition; unfortunately, most patients do not show significant neurologic improvement despite successful tumor treatment and/or immunosuppressive therapies.

Historical note and terminology

The occurrence of subacute cerebellar degeneration in patients with systemic cancer was noted more than 60 years ago (Parker and Kernohan 1933). An etiologic association between cancer and cerebellar degeneration was first clearly postulated by Lord Russell Brain in 1951 (Brain et al 1951).

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