Paraneoplastic rhombencephalitis corresponds to a perivascular and interstitial inflammatory process affecting the hindbrain (brainstem and cerebellum) associated with a systemic cancer, small cell lung cancer in the majority of cases. It is considered a subset of autoimmune encephalitis.
Compared with infectious rhombencephalitis, paraneoplastic syndromes are of slower onset, poorer outcome, and are usually associated with a normal MRI findings. Clinical features and an inflammatory CSF with antineuronal antibodies confirms the diagnosis. It has been associated with anti-Yo, anti-Tr, anti-Hu, anti-Ri, anti-Ma, anti-amphiphysin, anti-KLHL11, etc. ref.
Please refer to the article on rhombencephalitis for a general discussion of that entity.
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