Huang C C, Liu C C, Chang Y C, Chen C Y, Wang S T, Yeh T F
Department of Pediatrics, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
N Engl J Med. 1999 Sep 23;341(13):936-42. doi: 10.1056/NEJM199909233411302.
Enterovirus 71 infection causes hand-foot-and-mouth disease in young children, which is characterized by several days of fever and vomiting, ulcerative lesions in the oral mucosa, and vesicles on the backs of the hands and feet. The initial illness resolves but is sometimes followed by aseptic meningitis, encephalomyelitis, or even acute flaccid paralysis similar to paralytic poliomyelitis.
We describe the neurologic complications associated with the enterovirus 71 epidemic that occurred in Taiwan in 1998. At three major hospitals we identified 41 children with culture-confirmed enterovirus 71 infection and acute neurologic manifestations. Magnetic resonance imaging (MRI) was performed in 4 patients with acute flaccid paralysis and 24 with rhombencephalitis.
The mean age of the patients was 2.5 years (range, 3 months to 8.2 years). Twenty-eight patients had hand-foot-and-mouth disease (68 percent), and 6 had herpangina (15 percent). The other seven patients had no skin or mucosal lesions. Three neurologic syndromes were identified: aseptic meningitis (in 3 patients); brain-stem encephalitis, or rhombencephalitis (in 37); and acute flaccid paralysis (in 4), which followed rhombencephalitis in 3 patients. In 20 patients with rhombencephalitis, the syndrome was characterized by myoclonic jerks and tremor, ataxia, or both (grade I disease). Ten patients had myoclonus and cranial-nerve involvement (grade II disease). In seven patients the brain-stem infection produced transient myoclonus followed by the rapid onset of respiratory distress, cyanosis, poor peripheral perfusion, shock, coma, loss of the doll's eye reflex, and apnea (grade III disease); five of these patients died within 12 hours after admission. In 17 of the 24 patients with rhombencephalitis who underwent MRI, T2-weighted scans showed high-intensity lesions in the brain stem, most commonly in the pontine tegmentum. At follow-up, two of the patients with acute flaccid paralysis had residual limb weakness, and five of the patients with rhombencephalitis had persistent neurologic deficits, including myoclonus (in one child), cranial-nerve deficits (in two), and ventilator-dependent apnea (in two).
In the 1998 enterovirus 71 epidemic in Taiwan, the chief neurologic complication was rhombencephalitis, which had a fatality rate of 14 percent. The most common initial symptoms were myoclonic jerks, and MRI usually showed evidence of brainstem involvement.
肠道病毒71型感染可导致幼儿手足口病，其特征为持续数天的发热和呕吐、口腔黏膜溃疡性病变以及手足背部出现水疱。初始疾病可痊愈，但有时会继发无菌性脑膜炎、脑脊髓炎，甚至出现类似于麻痹性脊髓灰质炎的急性弛缓性麻痹。
我们描述了1998年台湾地区肠道病毒71型流行所伴发的神经系统并发症。在三家主要医院，我们确定了41例经培养确诊为肠道病毒71型感染且有急性神经系统表现的儿童。对4例急性弛缓性麻痹患者和24例脑干脑炎患者进行了磁共振成像（MRI）检查。
患者的平均年龄为2.5岁（范围为3个月至8.2岁）。28例患者患有手足口病（68%），6例患有疱疹性咽峡炎（15%）。其他7例患者无皮肤或黏膜病变。确定了三种神经系统综合征：无菌性脑膜炎（3例）；脑干脑炎，即rhombencephalitis（37例）；急性弛缓性麻痹（4例），其中3例急性弛缓性麻痹患者在rhombencephalitis之后出现。在20例rhombencephalitis患者中，该综合征的特征为肌阵挛性抽搐和震颤、共济失调或两者皆有（I级疾病）。10例患者有肌阵挛和脑神经受累（II级疾病）。7例患者脑干感染出现短暂肌阵挛，随后迅速出现呼吸窘迫、发绀、外周灌注不良、休克、昏迷、玩偶眼反射消失及呼吸暂停（III级疾病）；其中5例患者在入院后12小时内死亡。在接受MRI检查 的24例rhombencephalitis患者中，17例T2加权扫描显示脑干有高强度病变，最常见于脑桥被盖部。随访时，2例急性弛缓性麻痹患者有肢体残留无力，5例rhombencephalitis患者有持续的神经功能缺损，包括肌阵挛（1例儿童）、脑神经缺损（2例）和依赖呼吸机的呼吸暂停（2例）。
在1998年台湾地区肠道病毒71型流行中，主要的神经系统并发症是rhombencephalitis，病死率为14%。最常见的初始症状是肌阵挛性抽搐，MRI通常显示有脑干受累的证据。
