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