Autopsy evidence of central and peripheral demyelination in a case of Guillain-Barré syndrome/ Bickerstaff brainstem encephalitis overlap syndrome

Abstract

We report a case of a 59-year-old male who had acute, severe, rapidly progressive ascending weakness, which progressed to difficulty of breathing in a span of twelve hours. Neurophysiologic studies showed acute denervation compatible with acute motor axonal neuropathy. He was started on intravenous immunoglobulin therapy on the first day of hospitalization and completed five days of treatment but still developed decrease in sensorium and bilateral ophthalmoplegia. Imaging studies of the brain and cervical spinal cord showed findings that are non-contributory to the clinical presentation, leading to the consideration of Bickerstaff’s brainstem encephalitis. Pulse therapy with high dose methylprednisolone for five days was given however, despite maximal treatment, he expired on the 12th day of illness. Post-mortem immunohistochemical studies of the pons and sural nerve showed areas of inflammation and demyelination in both areas, suggesting combined central and peripheral demyelination in a single patient. Literature review shows that our patient presents with atypical clinical and diagnostic features, different from Bickerstaff’s brainstem encephalitis and combined central and peripheral demyelination, suggesting another disease entity presenting as acute fulminant neuropathy.