bickerstaff脑干脑炎.full.pdf

Bickerstaffs brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain±Barre? syndrome Masaaki Odaka,1 Nobuhiro Yuki,1 Mitsunori Yamada,2 Michiaki Koga,1 Toshihiko Takemi,3 Koichi Hirata1 and Satoshi Kuwabara4 1Department of Neurology, Dokkyo University School of Medicine, Tochigi 2Department of Pathology, Brain Research Institute, Niigata University, Niigata 3Department of Neurology, St Lukes International Hospital, Tokyo and 4Department of Neurology, Chiba University School of Medicine, Chiba, Japan Correspondence to: Nobuhiro Yuki, MD, PhD, Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan E-mail: yuki@dokkyomed.ac.jp Summary 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 Bickerstaffs brainstem ence- phalitis (BBE). One patient had gross ˉaccid 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 clin- ical features, we reviewed detailed clinical pro?les and laboratory ?ndings 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 hyperreˉexia. Ninety- two per cent of the patients involved had had an ante- cedent illness. Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinskis sign (40%) and pupil- lary 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 ˉaccid sym- metrical tetrapares