Rosch Richard E, Farquhar Michael, Gringras Paul, Pal Deb K
Wellcome Trust Centre for Neuroimaging, Institute of Neurology, University College London, London, UK; Centre for Developmental Cognitive Neuroscience, Institute of Child Health, University College London, London, UK.
Children's Sleep Medicine Department, Evelina London Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust , London , UK.
Front Neurol. 2016 Aug 10;7:130. doi: 10.3389/fneur.2016.00130. eCollection 2016.
Narcolepsy with cataplexy is a rare, but important differential diagnosis for daytime sleepiness and atonic paroxysms in an adolescent. A recent increase in incidence in the pediatric age group probably linked to the use of the Pandemrix influenza vaccine in 2009, has increased awareness that different environmental factors can "trigger" narcolepsy with cataplexy in a genetically susceptible population. Here, we describe the case of a 13-year-old boy with narcolepsy following yellow fever vaccination. He carries the HLA DQB1*0602 haplotype strongly associated with narcolepsy and cataplexy. Polysomnography showed rapid sleep onset with rapid eye movement (REM) latency of 47 min, significant sleep fragmentation and a mean sleep latency of 1.6 min with sleep onset REM in four out of four nap periods. Together with the clinical history, these findings are diagnostic of narcolepsy type 1. The envelope protein E of the yellow fever vaccine strain 17D has significant amino acid sequence overlap with both hypocretin and the hypocretin receptor 2 receptors in protein regions that are predicted to act as epitopes for antibody production. These findings raise the question whether the yellow fever vaccine strain may, through a potential molecular mimicry mechanism, be another infectious trigger for this neuro-immunological disorder.
发作性睡病伴猝倒症是青少年日间嗜睡和弛缓性发作的一种罕见但重要的鉴别诊断。儿科年龄组发病率最近有所上升,这可能与2009年使用大流行甲型H1N1流感疫苗有关,这提高了人们对不同环境因素可在遗传易感人群中“引发”发作性睡病伴猝倒症的认识。在此,我们描述了一名13岁男孩在接种黄热病疫苗后发生发作性睡病的病例。他携带与发作性睡病和猝倒症密切相关的HLA DQB1*0602单倍型。多导睡眠图显示睡眠快速开始,快速眼动(REM)潜伏期为47分钟,睡眠严重碎片化,平均睡眠潜伏期为1.6分钟,四个午睡期均在睡眠开始时出现快速眼动。结合临床病史,这些发现可诊断为1型发作性睡病。黄热病疫苗株17D的包膜蛋白E在预测为抗体产生表位的蛋白质区域与下丘脑分泌素和下丘脑分泌素受体2均有显著的氨基酸序列重叠。这些发现提出了一个问题,即黄热病疫苗株是否可能通过潜在的分子模拟机制成为这种神经免疫疾病的另一种感染性触发因素。