Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus

Abstract

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barré 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.

Bickerstaff Brainstem Encephalitis and overlapping Guillain-Barré syndrome in children: Report of two cases and review of the literature

“…In 2 cases, the diagnosis of BBE/GBS was specifically evoked by the authors. 23,25 Seven cases were diagnosed with BBE (one with an atypical form of BBE), 5 with MFS (one being an overlap MFS/BBE) and 1 with an atypical form of GBS. In 2 other cases the diagnosis was of unspecified brainstem encephalitis.…”

First report of Bickerstaff’s brainstem encephalitis caused by Salmonella Dublin: a case report

“…This case is the first report of BBE caused by Salmonella Dublin . There is a previous report of BBE caused by typhoid fever and Salmonella Paratyphi A [ 7 , 8 ].…”

Bickerstaff brainstem encephalitis, acute transverse myelitis, and acute motor axonal neuropathy: diagnostic and treatment challenges in patients with concomitant syndromes. Clinical observation

“…В последующем подобные синдромы стали называться перекрестными, что подразумевало перекрытие симп-томов 2 и более заболеваний предположительно одной природы, таких как СГБ, СЭБ и СМФ [9,17,18]. Се-годня описаны разные сочетания перечисленных со-стояний как у взрослых, так и у детей [5,9,10,[19][20][21]. При обсуждении вопроса о перекрытии симптомов перечисленных форм дизиммунных расстройств обра-щено внимание на клиническую схожесть СЭБ и СМФ.…”

“…Диагностические трудности возникают в случае наличия так называемых перекрестных синдромов (overlap-syndrome), когда у одного и того же больного одномоментно обнаруживаются клинические, биохи-мические, серологические и инструментальные при-знаки, характерные для 2 болезней или синдромов [7,8]. В зарубежной литературе представлены клини-ческие случаи перекрестных синдромов СГБ и СЭБ [5,9,10]. Присоединение к симптомам СЭБ вялого тетра-пареза свидетельствует о возможном параллельном поражении периферических нервов за счет развития overlap-синдрома с СГБ, что утяжеляет течение СЭБ.…”