Paraneoplastic cerebellar degeneration is a type of paraneoplastic syndrome, and manifestation of autoimmune encephalitis, characterised by the progressive onset of cerebellar dysfunction not explained by tumoural invasion, metastasis, or treatment side effects.
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Clinical presentation
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.
Pathology
Cerebellar degeneration has been reported in patients with multiple different primary malignancies, including small cell lung cancer (most common), ovarian malignancy, breast 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.
Radiographic features
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.
Treatment and prognosis
The management is focused on treatment of the underlying malignancy, and supportive therapies.