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2019 | 135 | 144-155
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A rare case of Bickerstaff’s brainstem encephalitis

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Bickerstaff’s brainstem encephalitis (BBE) is a rare, immune-dependent disease of brainstem characterized by progressive ophthalmoplegia, ataxia and central nervous system involvement, which may take the form of impaired consciousness and/or pyramidal signs. BBE requires differentiation with many neurological conditions and it is extremely difficult to make the proper diagnosis. We describe the case of a 63-year-old female patient admitted to the Department of Neurology due to speech changes, balance disorder, diplopia and weakness of the right limbs. On admission, neurological examination revealed depressed gag and palatal reflexes, central facial nerve palsy on the right side of the face and paresis of the right limbs. On the second day, progression of neurological deficit was observed – quantitative disturbances of consciousness, absence of gag and palatal reflexes and bilaterally positive Babinski sign. Head MRI did not confirm the presence of recent ischemic changes in the brain. From the fourth day, the patient's general and neurological condition was very severe. She was unconscious with ophthalmoplegia and narrow, non-reactive pupils. Multimodal therapy was administered during hospitalization, observing a gradual improvement in the patient's condition from the thirteenth day of hospitalization. Due to the ambiguous clinical picture, the following diseases were considered in the differential diagnosis: ischemic stroke of the brainstem region, venous sinus thrombosis, posterior reversible encephalopathy syndrome, reversible cerebral vasoconstriction syndrome, paraneoplastic brainstem encephalitis and Bickerstaff’s brainstem encephalitis. On the thirty-fifth day, the patient was discharged home. The patient maintained verbal contact with clear speech, proper eye movement and persistent four-limb paresis. After 5 months, the patient was re-admitted to the Department of Neurology to assess the neurological condition and undergo medical check-ups.
Physical description
  • Department of Neurology, Medical University of Silesia, Zabrze, Poland
  • Department of Neurology, Medical University of Silesia, Zabrze, Poland
  • Department of Neurology, Medical University of Silesia, Zabrze, Poland
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