Paraneoplastic cerebellar degeneration

Last revised by Rohit Sharma on 3 Mar 2024

Paraneoplastic cerebellar degeneration is a type of paraneoplastic syndrome, and manifestation of autoimmune encephalitis, characterized by the progressive onset of cerebellar dysfunction not explained by tumoral invasion, metastasis, or treatment side effects.

Cerebellar dysfunction may manifest as dizziness, nausea and/or vomiting, ataxia, diplopia, dysarthria, or dysphagia 1. Rapidly progressing gait unsteadiness is usually the key symptom that prompts neuroimaging.

Cerebellar degeneration has been reported in patients with multiple different primary malignancies, including small cell lung cancer (most common), ovarian malignancybreast carcinoma, and Hodgkin lymphoma 1.

There are many implicated pathogenic antibodies that may be produced by the primary malignancy to cause cerebellar degeneration, including anti-Hu, anti-Ma, anti-Yo, anti-Tr, and anti-Zic4 3.

Brain imaging with CT or MRI may show either normal cerebellum, particularly in initial presentation, to eventual diffuse cerebellar atrophy. Cerebellar hemispheric enlargement and corticomeningeal enhancement have been reported 2

The management is focused on treatment of the underlying malignancy, and supportive therapies.

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