白塞性脑炎:临床特征和病因。

IF 4.1 Q1 CLINICAL NEUROLOGY Brain communications Pub Date : 2024-10-04 eCollection Date: 2024-01-01 DOI:10.1093/braincomms/fcae347
Jonathan P Rogers, Tomas Mastellari, Alex J Berry, Kieron Kumar, Ella Burchill, Anthony S David, Glyn Lewis, Andrew Lees, Michael S Zandi
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引用次数: 0

摘要

昏睡性脑炎是一种流行性神经系统疾病,通常伴有严重的睡眠障碍和进行性帕金森病。一个世纪后,我们对这种疾病的了解有赖于开创性的描述、最新的历史研究以及对少量可能的零星病例的研究。围绕感染、环境毒素、紧张症和自身免疫性脑炎提出了一些理论。我们的目的是描述白塞性脑炎的表现,并检验这些诊断和病因理论。我们在英国国立神经病学和神经外科医院的档案中找到了 1918 年至 1946 年间患有昏睡性脑炎的患者。对病例记录进行了研究,以确定疾病的时间性、临床特征和脑脊液结果。从档案中确定了 10% 病例的对照组,根据出院年份、性别和神经科医生进行匹配。临床表现与白塞性脑炎、紧张症和自身免疫性脑炎的现代诊断标准进行了比较。在病例对照设计中,进行了多级逻辑回归,以确定昏睡性脑炎病例是否与发热性疾病和环境暴露有关。研究发现了 614 例白塞性脑炎病例和 65 例对照病例。病例的中位年龄为 29 岁(四分位数间距为 18),中位发病时间为 3.00 年(四分位数间距为 3.52)。97.6%的患者有运动特征,91.0%的患者有颅神经发现,77.4%的患者有眼科特征,66.1%的患者有睡眠障碍,62.1%的患者有胃肠道或营养特征,60.8%的患者有语言障碍,53.9%的患者有精神特征。在接受腰椎穿刺的 167 例病例中,20 例(12.0%)有多血细胞增多。霍华德和利斯脑炎标准的敏感性为 28.5%,特异性为 96.9%。病例中有 195 人(31.8%,95% 置信区间为 28.1-35.6%)在发病前一年内有发热病史,这种情况比对照组更常见(几率比 2.70,95% 置信区间为 1.02-7.20,P = 0.05),但存在很大的报告偏差。没有证据表明职业性接触溶剂或重金属与白塞性脑炎有关。有 276 个病例(45.0%)可能符合可能的自身免疫性脑炎的标准,但只有 3 个病例(0.5%)可能符合可能的 NMDA 受体脑炎的标准。只有 11 个病例(1.8%)符合紧张性精神障碍的标准。昏睡性脑炎作为一种神经精神疾病,具有广泛的临床特征。与传染病或职业接触有关的证据不足。自身免疫性脑炎可能是一种解释,但典型病例与 NMDA 受体脑炎不一致。
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Encephalitis lethargica: clinical features and aetiology.

Encephalitis lethargica, an epidemic neurological illness, typically involved a severe sleep disorder and progressive parkinsonism. A century later, our understanding relies on seminal descriptions, more recent historical research and the study of small numbers of possible sporadic cases. Theories around infection, environmental toxins, catatonia and autoimmune encephalitis have been proposed. We aimed to describe the presentation of encephalitis lethargica and test these diagnostic and aetiological theories. Subjects with encephalitis lethargica were identified in the archives of the National Hospital for Neurology and Neurosurgery, UK between 1918 and 1946. Case notes were examined to establish illness temporality, clinical features and cerebrospinal fluid results. Controls from the archives were identified for 10% of cases, matching on discharge year, sex and neurologist. Clinical presentation was compared to modern diagnostic criteria for encephalitis lethargica, catatonia and autoimmune encephalitis. In a case-control design, a multilevel logistic regression was conducted to ascertain whether cases of encephalitis lethargica were associated with febrile illnesses and with environmental exposures. Six hundred and fourteen cases of encephalitis lethargica and 65 controls were identified. Cases had a median age of 29 years (interquartile range 18) and a median time since symptomatic onset of 3.00 years (interquartile range 3.52). Motor features were present in 97.6%, cranial nerve findings in 91.0%, ophthalmological features in 77.4%, sleep disorders in 66.1%, gastrointestinal or nutritional features in 62.1%, speech disorders in 60.8% and psychiatric features in 53.9%. Of the 167 cases who underwent lumbar puncture, 20 (12.0%) had a pleocytosis. The Howard and Lees criteria for encephalitis lethargica had a sensitivity of 28.5% and specificity of 96.9%. Among the cases, 195 (31.8%, 95% confidence interval 28.1-35.6%) had a history of febrile illness within one calendar year prior to illness onset, which was more common than among the controls (odds ratio 2.70, 95% confidence interval 1.02-7.20, P = 0.05), but there was substantial reporting bias. There was no evidence that occupational exposure to solvents or heavy metals was associated with encephalitis lethargica. Two hundred and seventy-six (45.0%) of the cases might meet criteria for possible autoimmune encephalitis, but only 3 (0.5%) might meet criteria for probable NMDA receptor encephalitis. Only 11 cases (1.8%) met criteria for catatonia. Encephalitis lethargica has a distinct identity as a neuropsychiatric condition with a wide range of clinical features. Evidence for a relationship with infectious or occupational exposures was weak. Autoimmune encephalitis may be an explanation, but typical cases were inconsistent with NMDA receptor encephalitis.

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