Paraneoplastic cerebellar degeneration (PCD)

What is paraneoplastic cerebellar degeneration (PCD)? 

Paraneoplastic cerebellar degeneration is the most common remote effect of neoplasm affecting the brain, associated with lung (especially small-cell), ovarian, and breast neoplasms as well as Hodgkin’s disease.

A prodrome (a viral-like illness, dizziness, nausea, or vomiting) may be followed by rapid development and progression of gait unsteadiness, ataxia, diplopia, dysarthria, and dysphagia over a few weeks to months.

Pathology reveals extensive loss of Purkinje cells and inflammatory infiltrates.

Neuroimaging is typically normal early, with delayed progressive cerebellar atrophy.

Associated antibodies include (1) anti-Hu or ANNA1 (found predominantly in small-cell lung cancer); (2) anti-Yo or PCA1 (ovarian and breast cancers); and (3) anti-Tr (Hodgkin’s lymphoma).

Treatment includes steroids, plasmapheresis, intravenous immunoglobulin, tacrolimus, and rituximab, with varying success, or complete removal of the underlying malignancy.

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