Towards a better recognition of paraneoplastic brainstem encephalitis

Clinical and immunological features in paraneoplastic cochleovestibulopathy

A paraneoplastic aetiology should be considered in patients who develop symptoms of brainstem dysfunction over a period of weeks, even if their brain MRI does not show any evidence of inflammation.1 The detection of serum or cerebrospinal fluid onconeural antibodies is a critically important test to confirm the diagnosis. However, the frequency of paraneoplastic brainstem encephalitis is low and considering that indiscriminate antibody evaluation reduces the positive diagnostic value, careful selection of patients for onconeural antibody testing remains a crucial issue.2

When should we suspect a paraneoplastic brainstem encephalitis? When we recognise particular neurological symptoms typically observed in patients with paraneoplastic brainstem encephalitis and other more common disorders are reasonably ruled out. Some neurological symptoms also predict the type of associated onconeural antibody. For example, isolated or predominant central hypoventilation (Ondine’s curse) is indicative of anti-Hu-associated brainstem encephalitis, whereas jaw…

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