Department of Neurology, Santa Casa de São Paulo School of Medical Sciences, São Paulo, Brazil.
Department of Pediatric Neurology, Santa Casa de São Paulo School of Medical Sciences, São Paulo, Brazil.
Mult Scler Relat Disord. 2019 Nov;36:101379. doi: 10.1016/j.msard.2019.101379. Epub 2019 Aug 28.
We report a case of a 14-year-old girl that presented headache, amaurosis, drowsiness, fever, vomiting and diffuse reduction of muscle strength. She had been diagnosed with ADEM one year before and had a previous diagnosis of Toll-Like 3 receptor deficiency. Cerebrospinal fluid analysis revealed pleocytosis (28/mm, 12/mm red blood cells, 70% lymphocytes cells, 2% monocytes cells, 28% neutrophils), normal total protein (38 pg/mL) and normal glucose level (53/mm). Studies for CSF oligoclonal bands and serum anti-MOG were negative but polymerase chain reaction (PCR) testing was positive for herpes virus 1. In the first ADEM episode, PCR for herpes virus was also positive. Magnetic resonance imaging (MRI) of the brain revealed disseminated hyperintense lesions on T2-weighted and FLAIR images in the white matter of frontal, parietal and temporal lobes, corresponding to extensive asymmetric areas of demyelination that produced mass effect and gadolinium enhancement. Electroencephalography demonstrated irregular diffuse and generalized slow-wave activity with predominance in frontal region. The diagnosis of multiphasic disseminated encephalomyelitis (MDEM) triggered by herpes simplex virus was made. Herpes virus is a neurotropic virus that can cause a wide variety of neurological infection-triggered autoimmune disorders and that is particularly damaging to the central nervous system in situations of impaired immune system. TLR3 is expressed in astrocytes and dendritic cells of the central nervous system and is essential for natural immunity to herpes simplex. TLR3-deficient patients have already been described with herpes simplex encephalitis. TLR3 deficiency may predispose and explain autoimmune and demyelinating manifestations induced by herpes virus. The association of multiphasic disseminated encephalomyelitis triggered by herpes virus in a patient with TLR3 deficiency has not been previously reported in the literature.
我们报告了一例 14 岁女孩，她表现为头痛、视力模糊、嗜睡、发热、呕吐和全身肌肉力量减弱。一年前她被诊断为 ADEM，此前曾被诊断为 Toll-Like 3 受体缺陷。脑脊液分析显示白细胞增多（28/mm，12/mm 红细胞，70%淋巴细胞，2%单核细胞，28%中性粒细胞），总蛋白正常（38pg/mL），血糖水平正常（53/mm）。CSF 寡克隆带和血清抗-MOG 研究均为阴性，但聚合酶链反应（PCR）检测疱疹病毒 1 为阳性。在第一次 ADEM 发作时，PCR 也检测到疱疹病毒阳性。脑部磁共振成像（MRI）显示在额、顶和颞叶的白质中存在 T2 加权和 FLAIR 图像上弥散性高信号病变，对应于广泛的脱髓鞘不对称区域，产生肿块效应和钆增强。脑电图显示不规则弥漫性和广泛的慢波活动，以额叶为主。诊断为单纯疱疹病毒引起的多相播散性脑脊髓炎（MDEM）。单纯疱疹病毒是一种嗜神经病毒，可引起多种神经感染触发的自身免疫性疾病，在免疫系统受损的情况下，对中枢神经系统尤其有害。TLR3 在中枢神经系统的星形胶质细胞和树突状细胞中表达，对单纯疱疹的天然免疫至关重要。已经描述了 TLR3 缺陷的单纯疱疹脑炎患者。TLR3 缺陷可能易患并解释单纯疱疹病毒引起的自身免疫和脱髓鞘表现。TLR3 缺陷患者由单纯疱疹病毒引起的多相播散性脑脊髓炎的关联在文献中尚未报道过。
