title: Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome.
abstract: Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia. Ninety-two per cent of the patients involved had had an antecedent illness. Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinski's sign (40%) and pupillary abnormality and bulbar palsy (34%) were present. Almost all the patients had a monophasic remitting course and generally a good outcome. Serum anti-GQ1b IgG antibody was positive in 66%, and MRI showed brain abnormality in 30% of the patients. Another striking feature was the association with flaccid symmetrical tetraparesis, seen in 60% of the patients. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: there was perivascular lymphocytic infiltration with oedema and glial nodules. Electrodiagnostic study results suggested peripheral motor axonal degeneration. Limb weakness in the BBE cases studied was considered the result of overlap with the axonal subtype of Guillain-Barré syndrome. These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE. A considerable number of BBE patients have associated axonal Guillain-Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.
authors.affiliation: Department of Neurology, Dokkyo University School of Medicine, Shimotsuga, Tochigi, Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Odaka
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Yuki
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Yamada
authors.forename: Michiaki
authors.initials: M
authors.lastname: Koga
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Takemi
authors.forename: Koichi
authors.initials: K
authors.lastname: Hirata
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuwabara
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001259:Ataxia / Q000209:etiology / Q000276:immunology / Q000473:pathology; D001323:Autoantibodies / Q000097:blood; D001933:Brain Stem / Q000276:immunology / Q000473:pathology*; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004576:Electromyography; D004660:Encephalitis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D005260:Female; D005732:Gangliosides / Q000276:immunology; D020275:Guillain-Barre Syndrome / Q000276:immunology / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009886:Ophthalmoplegia / Q000209:etiology / Q000276:immunology / Q000473:pathology; D011782:Quadriplegia / Q000209:etiology / Q000276:immunology / Q000473:pathology
chemical_list: D001323:Autoantibodies; D005732:Gangliosides; D007074:Immunoglobulin G; C039129:GQ1b ganglioside