Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of Bickerstaff's brainstem encephalitis in childhood

Bickerstaff 뇌간 뇌염 1례

  • Kim, Ji-Youn (Department of Pediatrics, Chonnam National University Medical School) ;
  • Kim, Young-Ok (Department of Pediatrics, Chonnam National University Medical School) ;
  • Son, Young-Jun (Department of Pediatrics, Chonnam National University Medical School) ;
  • Woo, Young-Jong (Department of Pediatrics, Chonnam National University Medical School)
  • 김지윤 (전남대학교 의과대학 소아과학교실) ;
  • 김영옥 (전남대학교 의과대학 소아과학교실) ;
  • 손영준 (전남대학교 의과대학 소아과학교실) ;
  • 우영종 (전남대학교 의과대학 소아과학교실)
  • Received : 2009.09.25
  • Accepted : 2010.03.02
  • Published : 2010.04.15
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Abstract

Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic methods: brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins.

Bickerstaff 뇌간 뇌염은(BBE) 4주 이내의 진행성이며, 비교적 대칭성으로 오는 안근 마비와 실조증, 의식 장애 또는 심부건 반사 항진 등의 임상적 특징을 가지며, 뇌간을 침범하는 타 질환을 배제하였을 때 진단할 수 있는 드문 질환이다. 혈청 또는 뇌척수액의 항 Ganglioside 항체(GM, GD and GQ) 는 때로 BBE의 진단에 도움이 되기도 하며, 뇌 자기 공명 영상, 뇌 척수액 검사, 신경 전도 검사 및 근 전도 검사 등은 진단에 크게 도움이 되지 않는다. 저자들은 안근 마비, 실조증, 언어 운동 장애, 연하 장애, 점진적 사지 마비, 의식 저하 등의 증상을 보이며 혈청과 뇌척수액에서 anti-GM1 항체의 증가를 보여 BBE로 진단하고 면역 글로불린과 스테로이드 치료 후 완치되었던 9세의 여아의 증례를 경험하였기에 문헌 고찰과 함께 이를 보고하는 바이다.

Keywords

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