Santoro Jonathan D
Division of Child Neurology, Department of Neurology, Stanford University, Palo Alto, CA, USA.
Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
J Child Neurol. 2019 Mar;34(3):153-160. doi: 10.1177/0883073818820488. Epub 2019 Jan 3.
Bickerstaff brainstem encephalitis is a rare, immune-mediated disorder of the brainstem and peripheral nervous system. Published knowledge of pediatric Bickerstaff brainstem encephalitis focuses on the acute phase of the disease process. This study evaluated long-term neurologic and immune sequelae of Bickerstaff brainstem encephalitis in children. A single-center retrospective chart review was performed. Clinical data, neuroimaging, polysomnograms, and serum data were reviewed. Five patients were included in this study. Four patients had no neurologic residua, and 1 patient continued to have mild bulbar dysfunction. There was neither recurrence of symptoms nor development of other neurologic or immunologic disorders at a median of 3 years after diagnosis. Review of systems was largely negative, although 2 patients endorsed symptoms consistent with mild orthostatic hypotension for 1 year after diagnosis, but these findings were not sustained. Four of 5 patients endorsed sleep dysregulation. Three patients met criteria for pediatric obstructive sleep apnea. Prognosis following pediatric Bickerstaff brainstem encephalitis is excellent although posttreatment autonomic and sleep dysregulation may reflect residua from acute phase inflammation in the peripheral nervous system and connections of the reticular activating formation of the brainstem, although this was time limited. Further prospective, multicenter, analysis is warranted.
比克斯特法夫脑干脑炎是一种罕见的、免疫介导的脑干和周围神经系统疾病。已发表的关于儿童比克斯特法夫脑干脑炎的知识主要集中在疾病过程的急性期。本研究评估了儿童比克斯特法夫脑干脑炎的长期神经和免疫后遗症。进行了一项单中心回顾性病历审查。审查了临床数据、神经影像学、多导睡眠图和血清数据。本研究纳入了5例患者。4例患者无神经残留症状，1例患者仍有轻度延髓功能障碍。诊断后中位3年时，既没有症状复发，也没有出现其他神经或免疫疾病。系统回顾大多为阴性，尽管2例患者在诊断后1年内认可与轻度体位性低血压一致的症状，但这些发现未持续存在。5例患者中有4例认可睡眠失调。3例患者符合小儿阻塞性睡眠呼吸暂停的标准。儿童比克斯特法夫脑干脑炎后的预后良好，尽管治疗后自主神经和睡眠失调可能反映了急性期外周神经系统炎症以及脑干网状激活系统连接的残留影响，尽管这种情况是有时间限制的。有必要进行进一步的前瞻性多中心分析。
