Paraneoplastic Cerebellar Degeneration

Diagnosis:

A combination of clinical features, identification of underlying cancer and occasionally other tests

Clinical features:

Symmetric subacute progressive cerebellar ataxia with dysarthria & nystagmus

Pathology:

Histology: massive loss of Purkinje cells. Dentate nucleus: Pallor of myelin fleece (amiculum). Sometimes mononuclear perivascular infiltrates or microglial nodules

Findings on investigations:

Antibodies:

  • anti-Yo a.k.a. anti-PCA-1 (ovarian & breast cancer), anti-Hu a.k.a. ANNA-1 (lung cancer), Tr-Ab (Hodgkin lymphoma), anti-mGluR1 (Hodgkin’s lymphoma), Anti-Ma1 (lung cancer), anti-CRMP5 a.k.a. anti-CV2 (small cell lung cancer, thymoma)

CSF:

  • Raised IgG index, lymphocytic pleocytosis, increased protein in some

Treatment:

Consider:

  • Plasmapharesis, IVIG, steroids, cyclophosphamide

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