OBJECTIVES: Bickerstaff brainstem encephalitis (BBE) is an uncommon disease characterized by the occurrence of ataxia, ophthalmoplegia, sensorial disturbance, and pyramidal tract signs such as hyperreflexia and toe extensor response. In Asia, preponderance of cases has been reported in Japan. It has not been previously reported in the Philippines. This will describe the clinical symptoms, laboratory features and diagnostic criteria and treatment of this rare disease.
CASE REPORT: A 31- year old Vietnamese-American from Makati City presented with a 3-week history of diarrhea and flu-like symptoms followed by dizziness, gait instability, diplopia, generalized weakness and rapidly progressive sensorial deterioration. Neurologic findings included ataxia, bilateral external ophthalmoplegia, ocular bobbing, hyperreflexia, bilateral toe extensor response and altered sensorium. Cranial MRI, Complete CSF Analysis, EMG-NCV and RNS were unremarkable. Brainstem auditory evoked potential indicated failed conduction between the lower and upper pons. Blink reflex study indicated failed conduction along the medullary interneuronal pathway. Plasmapheresis and intravenous corticosteroid therapy were instituted. Patient's condition improved from a comatose state and was discharged ambulatory with minimal residual neurologic deficits after 38 days of hospitalization. Serum Anti-ganglioside antibody assay were positive for GM1 b IgG and GQ 1bG.
CONCLUSION: The clinical characteristics, laboratory features and course of this patient were consistent with Bickerstaff's Brainstem Encephalitis.