Gagnon Alexis, Bouchard Remi W
Department of Neurological Sciences, Centre Hospitalier Affilié Universitaire de Québec-Hôpital de l'Enfant-Jésus, Quebec, Quebec, Canada.
Arch Neurol. 2003 Aug;60(8):1160-1. doi: 10.1001/archneur.60.8.1160.
Subacute sclerosing panencephalitis (SSPE) is a rare, slow viral infection caused by a defective measles virus. It is characterized by progressive mental deterioration associated with motor impairment and prominent myoclonus. In about 10% of all cases, the disease can progress rapidly and lead to death within a few months. The oldest previously reported fulminating case was in a 25-year-old man.
To emphasize the relationship between retinal involvement and acute SSPE by reporting the case of a 49-year-old man with clinical, laboratory, and pathological evidence of acute SSPE.
Hôpital de l'Enfant-Jésus, Quebec, Quebec.
This man was referred to the Department of Neurological Sciences on March 21, 2001, because of recent behavioral changes and progressive cognitive impairment over the past few months. Medical history was unremarkable except for an episode of measles in his childhood. Neurological examination showed bilateral myoclonic jerks. Ophthalmic examination revealed bilateral macular swelling and papilledema. Electroencephalography showed periodic sharp and slow-wave discharges. Magnetic resonance imaging showed bilateral diffuse T2-signal hyperintensities in both periventricular and subcortical white matter. Cerebrospinal fluid antimeasles antibody titers were highly positive. An Omaya reservoir was inserted and therapy using a combination of high-dose intrathecal interferon alfa and oral isoprinosine were administered for 6 weeks. Despite transient subjective improvement in the patient's condition, it continued to deteriorate, he became bedridden, and he died on June 26, 2001.
To our knowledge, this patient is the oldest case of SSPE reported in the literature. This patient and other patients with acute SSPE associated with bilateral macular swelling described in the literature raised the possibility of measles virus-acquired virulent neurotropism in the retina before invading the central nervous system.
亚急性硬化性全脑炎（SSPE）是一种由缺陷型麻疹病毒引起的罕见的慢病毒感染。其特征为与运动障碍和明显肌阵挛相关的进行性精神衰退。在所有病例中，约10%的疾病可迅速进展并在数月内导致死亡。此前报道的最年长的暴发性病例是一名25岁男性。
通过报告一名有急性SSPE临床、实验室和病理证据的49岁男性病例，强调视网膜受累与急性SSPE之间的关系。
魁北克省魁北克市圣婴耶稣医院。
该男子于2001年3月21日因近期行为改变和过去几个月进行性认知障碍被转诊至神经科学科。除童年时有一次麻疹发作外，病史无异常。神经检查显示双侧肌阵挛性抽搐。眼科检查发现双侧黄斑肿胀和视乳头水肿。脑电图显示周期性尖波和慢波放电。磁共振成像显示双侧脑室周围和皮质下白质T2信号弥漫性增高。脑脊液抗麻疹抗体滴度呈高度阳性。插入了一个奥马亚贮液器，并使用高剂量鞘内注射干扰素α和口服异丙肌苷联合治疗6周。尽管患者病情有短暂的主观改善，但仍继续恶化，他卧床不起，并于2001年6月26日死亡。
据我们所知，该患者是文献中报道的最年长的SSPE病例。该患者及文献中描述的其他伴有双侧黄斑肿胀的急性SSPE患者，提示麻疹病毒在侵入中枢神经系统之前在视网膜获得了强嗜神经性。
