Document Detail

Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics.
MedLine Citation:
PMID:  22851608     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Information regarding the epidemiological background of Bickerstaff brainstem encephalitis (BBE) is limited.
METHODS: We conducted a nationwide survey of BBE in the Japanese population in two steps: the first aimed to identify patients with brainstem encephalitis for the specified 3 year period and the second to evaluate whether the clinical picture met our diagnostic criteria for BBE.
RESULTS: The number of patients with brainstem encephalitis was estimated as 704 (95% CI 478 to 930) over the 3 years. The annual onset of BBE was roughly estimated as 100 cases, which accounted for 43% of brainstem encephalitis. BBE was slightly male predominant and often young onset. Among brainstem encephalitis patients, BBE was characterised by antecedent infectious symptoms, oropharyngeal palsy and sensory disturbance at the distal extremities with absent or decreased tendon reflexes, in addition to a triad of symptoms (external ophthalmoplegia, ataxia and impaired level of consciousness) and shorter duration to the peak, with good outcome. Anti-GQ1b antibodies were present in 75% of cases. Several BBE patients with atypical clinical features or without anti-GQ1b antibodies were also identified. These cases often had marked CSF pleocytosis, abnormal brain MRI findings and a longer duration to peak symptoms, sometimes with considerable residual deficits.
CONCLUSIONS: BBE is a rare disorder but accounts for a major proportion of brainstem encephalitis. BBE consists of typical and atypical cases. Typical BBE has similar neurological and serological features to Fisher syndrome and shows good recovery whereas atypical BBE is characterised by delayed recovery, negative anti-GQ1b antibodies, and abnormal CSF and brain MRI findings with other possible pathogeneses.
Michiaki Koga; Susumu Kusunoki; Kenichi Kaida; Ritei Uehara; Yosikazu Nakamura; Tatsuo Kohriyama; Takashi Kanda
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-07-31
Journal Detail:
Title:  Journal of neurology, neurosurgery, and psychiatry     Volume:  83     ISSN:  1468-330X     ISO Abbreviation:  J. Neurol. Neurosurg. Psychiatr.     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-11-15     Completed Date:  2013-01-16     Revised Date:  2013-11-06    
Medline Journal Info:
Nlm Unique ID:  2985191R     Medline TA:  J Neurol Neurosurg Psychiatry     Country:  England    
Other Details:
Languages:  eng     Pagination:  1210-5     Citation Subset:  IM    
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Minami-Kogushi 1-1-1, Ube, Yamaguchi 755-8505, Japan.
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MeSH Terms
Aged, 80 and over
Brain Stem / pathology*
Child, Preschool
Encephalitis / epidemiology*,  pathology*,  therapy
Gangliosides / analysis,  immunology
Health Surveys
Japan / epidemiology
Magnetic Resonance Imaging
Middle Aged
Nervous System Diseases / physiopathology
Physicians, Primary Care
Treatment Outcome
Young Adult
Reg. No./Substance:
0/Gangliosides; 68652-37-9/GQ1b ganglioside

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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