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밀러-피셔증후군과 비커스태프 뇌줄기염의 임상적 특징

Clinical Characteristics of Miller-Fisher Syndrome and Bickerstaff Brainstem Encephalitis

검안 및 콘택트렌즈학회지 2020년 19권 3호 p.103 ~ 109
김민하, 서유리, 한승한, 한진우,
소속 상세정보
김민하 ( Kim Min-Ha ) - Yonsei University College of Medicine Gangnam Severance Hospital Department of Ophthalmology
서유리 ( Seo Yu-Ri ) - Yonsei University College of Medicine Yongin Severance Hospital Department of Ophthalmology
한승한 ( Han Sueng-Han ) - Yonsei University College of Medicine Severance Hospital Department of Ophthalmology
한진우 ( Han Jin-U ) - Yonsei University College of Medicine Gangnam Severance Hospital Department of Ophthalmology

Abstract


Purpose: To investigate the clinical characteristics of patients clinically diagnosed with anti-GQ1b antibody syndrome.

Methods: From November 2005 to July 2019, we retrospectively reviewed the medical records of 52 patients diagnosed with Miller-Fisher syndrome, Bickerstaff brainstem encephalitis or anti-GQ1b antibody syndrome. Symptom including acute ophthalmoplegia, ataxia, hyporeflexia and other neurologic features were reviewed. Laboratory results including cerebrospinal fluid (CSF) analysis and anti-ganglioside antibodies were also analyzed.

Results: Among 52 patients, 40 were diagnosed with classic Miller-Fisher syndrome, 3 patients were Guillain-Barre syndrome with ophthalmoparesis, 1 patient was acute ophthalmoparesis without ataxia, 2 patients were acute ataxic neuropathy, and 6 patients were diagnosis with Bickerstaff brainstem encephalitis. Thirty five patients were male (67.3%), the mean age of onset was 39.3 ± 16.7 years, and average follow-up duration was 9.6 ± 15.9 months. Forty-four patients (84.6%) showed preceding infection, and upper respiratory infection was more common than gastrointestinal infection. Forty-nine patients (94.2%) showed ophthalmoplegia, 48 patients (92.3%) showed ataxia and hyporeflexia was presented in 43/51 patients (84.3%). Other than classic symptoms, dysarthria (21/52, 40.4%), dizziness (27/52, 51.9%), and paresthesia (23/52, 44.2%) was observed. Among 48 patients who underwent CSF analysis, 11 patients (22.9%) showed albumino-cytologic dissociation. Only 12 patients (23.1%) had positive anti-ganglioside antibodies.

Conclusions: Anti-GQ1b antibody syndrome is not easy to differentiate from other diseases, careful physical examination and history taking is necessary to make correct diagnosis. The recognition of accompanying symptoms and signs could facilitate early and exact diagnosis of anti-GQ1b antibody syndrome.

키워드

Ataxia; Encephalitis; GQ1b ganglioside; Miller-Fisher syndrome; Ophthalmoparesis

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