• Clinical and Experimental Pediatrics
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• 제57권12호
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• pp.542-545
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• 2014

Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.

• Annals of Clinical Neurophysiology
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• 제11권1호
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• pp.33-36
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• 2009

Bickerstaff's brainstem encephalitis (BBE) is an autoimmune central nervous system disorder. It can occur in more limited forms and may overlap with Guillain-Barr$\acute{e}$ syndrome (GBS). A 49-year-old female presented with rapidly progressive paralytic ileus, urinary retention, deep drowsiness, ophthalmoplegia, dysarthria, ataxia, quadriparesis and hyporeflexia after viral meningitis. She was diagnosed as BBE with GBS and treated with immunoglobulin. She was completely recovered after 1 month. It is a rare case of BBE overlapping with GBS presenting with severe paralytic ileus.

• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.94-97
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• 2006

Bickerstaff's brainstem encephalitis (BBE) is a rare disorder with acute ophthalmoparesis, ataxia, consciousness disturbance, and pyramidal signs of suspected autoimmune origin. A-62-year-old woman developed acute diplopia, dysarthria, gaze-paretic nystagmus and gait ataxia. Her mental status fell subsequently into stupor. Brain MRI and nerve conduction study showed no significantly abnormal findings. Electroencephalography revealed diffuse low voltage slowings. After treating with intravenous immunoglobulin, she demonstrated rapid clinical recovery. This case suggests that immunoglobulin can be an alternative option in BBE treatment, especially when plasmapheresis and corticosteroids are difficult or contraindicated.

• Clinical and Experimental Pediatrics
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• 제57권10호
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• pp.457-460
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• 2014

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barr$\acute{e}$ syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.

• Annals of Clinical Neurophysiology
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• 제15권1호
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• pp.30-33
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• 2013

• Clinical and Experimental Pediatrics
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• 제53권4호
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• pp.607-611
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• 2010

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.

• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.78-80
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• 2006

Miller-Fisher syndrome, Guillain-Barre syndrome with ophthalmoplegia, Bickerstaff s brainstem encephalitis and acute ophthalmoplegia share some clinical features, and common anti-GQ1b IgG antibody and these are introduced as anti-GQ1b antibody syndrome. These syndromes mostly present with paralysis of extraocular muscles and internal ophthalmoplegia rarely occurs. We report a case of acute isolated bilateral internal ophthalmoplegia associated with anti-GQ1b IgG antibody.