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.
-  Koga M. A nationwide survey of patients with Bickerstaff brainstem encephalitis: diversity of underlying mechanism. Rinsho Shinkeigaku 53(11) (2013) 1322-1324.
-  Koga M. Bickerstaff brainstem encephalitis: epidemiology, diagnosis, and therapy. Nihon Rinsho 71(5) (2013) 898-903.
-  Horton E, Krishnamoorthy S, Reynolds L. Bickerstaff's encephalitis. BMJ Case Rep 2014. doi: 10.1136/bcr-2014-205336
-  Bickerstaff E. Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis. Br Med J 1(5032) (1957) 1384-1387.
-  Bickerstaff E, Cloake PCP. Mesencephalitis and rhombencephalitis. Br Med J 2(4723) (1951) 77-81.
-  Chiba A, Kusunoki S, Shimizu T, Kanazawa I. Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol 31(6) (1992) 677-679.
-  Shahrizaila N, Yuki N. Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry 84(5) (2013) 576-83.
-  Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barre´ syndrome: a systematic review and meta-analysis. Neuroepidemiology 36(2) (2011) 123-33.
-  Rybacka-Mossakowska J, Wyciszkiewicz A, Michalak S. Diagnostic and therapeutic problems in patients with encephalomyelitis. Anesthesiology and Rescue 8 (2014) 321-327.
-  Ducros A. Reversible cerebral vasoconstriction syndrome. Hand Clin Neurol 121 (2014) 1725-41.
-  Dałek G, Wasilewski G, Tutaj A. Reversible posterior encephalopathy syndrome in the course of eclampsia in pregnant women - a report of three cases. Polish Neurological Overview 5(4) (2009) 201-207.
-  Ito M, Matsuno K, Sakumoto Y, Hirata K, Yuki N. Ataxic Guillain-Barre´ syndrome and acute sensory ataxic neuropathy form a continuous spectrum. J Neurol Neurosurg Psychiatry 82 (2011) 294e9.
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