Ene Luminita, Duiculescu Dan, Radoi Roxana, Lazar Mihaela, Tardei Gratiela, Ungureanu Eugenia, Ruta Simona, Vinters Harry V, Letendre Scott, Grant Igor, Ellis Ronald J, Achim Cristian L
"Dr. Victor Babes" Hospital for Infectious and Tropical Diseases, Bucharest, Romania.
Cantacuzino Institute, Bucharest, Romania.
Front Cell Neurosci. 2023 Apr 4;17:1113935. doi: 10.3389/fncel.2023.1113935. eCollection 2023.
An unusual cluster of myoclonic epilepsy was observed in a Romanian pediatric HIV cohort concurrent with measles outbreaks. We describe this particular form of subacute measles encephalitis (SME) in a group of HIV-infected children and adolescents with severe immunosuppression.
This is a single-center study, starting in 1997 and covering 4 measles outbreaks in Romania. The presumptive diagnosis of subacute myoclonic measles encephalitis (SMME) was based on: (1) epidemiological data, previous measles episode or presumed contact with measles virus (MV), (2) clinical presentation with initial localized myoclonic jerks with rapid extension and subsequent motor deficit with preserved mental status, and (3) neuroimaging studies revealing cortical gray matter lesions. Definitive diagnosis was based on a neuropathological exam and immunohistochemistry of brain tissues, and measles RNA detection in the cerebrospinal fluid (CSF).
Thirty-six patients were diagnosed with a particular form of SME during consecutive measles outbreaks in Romania: 1996-1998 (22); 2005-2008 (12); 2010-2011 (1) and 2016-2018 (1). Most children were born in the late 80s and had parenterally acquired HIV infection in early childhood. Before the episode of SMME, 11 patients had confirmed measles, while the rest, without typical rash, had a respiratory tract infection and/or presumed previous measles contact. In all patients, the clinical onset was sudden, with unilateral myoclonus. MRI findings revealed mainly focal cortical gray matter lesions. Neurologic symptoms progressed rapidly to coma and death in most patients. Three patients survived SMME, they had higher CD4 count at onset, slower progression of neurological symptoms, and benefit of immune recovery with cART. Immunocytochemistry studies revealed MV in the brain with a pattern suggesting an ascending viral neural infection. MV was isolated from CSF in 7 out of 8 patients. Sequence analysis of MV RNA from both nasopharyngeal swabs and CSF was available for one patient with similar N-450 strain characteristics.
During an outbreak of measles, neurological manifestations, especially myoclonus in immunosuppressed patients, can be related to measles even in the absence of an acute episode. This particular form of subacute myoclonic measles encephalitis is an opportunistic fatal disease. Immune recovery due to effective antiretroviral treatment might increase survival.
在罗马尼亚儿科艾滋病毒队列中，在麻疹暴发期间观察到一组不寻常的肌阵挛性癫痫病例。我们描述了在一组严重免疫抑制的艾滋病毒感染儿童和青少年中这种特殊形式的亚急性麻疹脑炎（SME）。
这是一项单中心研究，始于1997年，涵盖罗马尼亚的4次麻疹暴发。亚急性肌阵挛性麻疹脑炎（SMME）的推定诊断基于：（1）流行病学数据、既往麻疹发作或推测与麻疹病毒（MV）接触；（2）临床表现为最初局部肌阵挛性抽搐并迅速扩展，随后出现运动功能障碍但精神状态保留；（3）神经影像学研究显示皮质灰质病变。确诊基于脑组织的神经病理学检查和免疫组织化学，以及脑脊液（CSF）中麻疹RNA检测。
在罗马尼亚连续的麻疹暴发期间，36例患者被诊断为一种特殊形式的SME：1996 - 1998年（22例）；2005 - 2008年（12例）；2010 - 2011年（1例）和2016 - 2018年（1例）。大多数儿童出生于80年代后期，幼儿期通过肠道外途径感染艾滋病毒。在SMME发作前，11例患者确诊为麻疹，其余患者无典型皮疹，有呼吸道感染和/或推测既往接触过麻疹。所有患者临床起病突然，表现为单侧肌阵挛。MRI检查结果主要显示局灶性皮质灰质病变。大多数患者的神经症状迅速进展为昏迷和死亡。3例SMME患者存活，他们起病时CD4计数较高，神经症状进展较慢，且通过抗逆转录病毒治疗实现了免疫恢复。免疫细胞化学研究显示脑中存在MV，其模式提示病毒沿神经逆行感染。8例患者中有7例从CSF中分离出MV。对1例患者的鼻咽拭子和CSF中的MV RNA进行序列分析，具有相似的N - 450株特征。
在麻疹暴发期间，即使在没有急性发作的情况下，免疫抑制患者的神经表现，尤其是肌阵挛，也可能与麻疹有关。这种特殊形式的亚急性肌阵挛性麻疹脑炎是一种机会性致命疾病。有效的抗逆转录病毒治疗导致的免疫恢复可能会提高生存率。
