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一例急性脑炎综合征的罕见病例:是急性麻疹脑炎还是亚急性硬化性全脑炎?

An unusual case of acute encephalitic syndrome: Is it acute measles encephalitis or subacute sclerosing panencephalitis?

作者信息

Garg Ravindra K, Malhotra Hardeep S, Rizvi Imran, Kumar Neeraj, Jain Amita

机构信息

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India.

Department of Microbiology, King George's Medical University, Lucknow, Uttar Pradesh, India.

出版信息

Neurol India. 2017 Nov-Dec;65(6):1333-1344. doi: 10.4103/0028-3886.217942.

Abstract

Subacute sclerosing panencephalitis is a late complication of measles infection and develops usually 6 to 15 years after the primary measles infection. Fulminant subacute sclerosing panencephalitis is an infrequently encountered form wherein the disease rapidly progresses to death. A six-year old male child presented with fever, abnormal movements of the left side of body followed by weakness of the left side of the body, and involuntary abnormal movements of right upper and lower limbs. On examination, he was drowsy and was unable to communicate. He had right-sided hemiballismus. He also had left-sided hemiparesis and the left plantar reflex was extensor. Cerebrospinal fluid examination revealed elevated protein and cells. In the serum and cerebrospinal fluid, anti-measles IgG antibodies were found to be positive. No other viral marker was noted in the cerebrospinal fluid. Magnetic resonance imaging of the brain showed extensive damage to the right temporal, parietal, and to a lesser extent, the frontal region as well as subcortical structures of these regions. Electroencephalography revealed generalized slowing of waves. Over a period of the next 3 days, the intensity and frequency of choreiform movements markedly reduced and the patient developed periodic generalized myoclonus, which was predominantly present on the right side. The patient succumbed to his illness and died after one month. Fulminant subacute sclerosing panencephalitis may have unusual clinical manifestations such as hemiballismus. In fulminant subacute sclerosing panencephalitis, neuroimaging may show extensive cortical damage.

摘要

亚急性硬化性全脑炎是麻疹感染的一种晚期并发症,通常在原发性麻疹感染后6至15年发病。暴发性亚急性硬化性全脑炎是一种罕见的形式,疾病迅速进展至死亡。一名6岁男童出现发热、身体左侧异常运动,随后左侧身体无力,以及右上肢和下肢的不自主异常运动。检查时,他嗜睡且无法交流。他有右侧偏身投掷症。他还患有左侧偏瘫,左侧跖反射为伸性。脑脊液检查显示蛋白和细胞升高。在血清和脑脊液中,抗麻疹IgG抗体呈阳性。脑脊液中未发现其他病毒标志物。脑部磁共振成像显示右侧颞叶、顶叶广泛受损,额叶及这些区域的皮质下结构受损程度较轻。脑电图显示波幅普遍减慢。在接下来的3天里,舞蹈样动作的强度和频率明显降低,患者出现周期性全身性肌阵挛,主要出现在右侧。患者患病一个月后死亡。暴发性亚急性硬化性全脑炎可能有不寻常的临床表现,如偏身投掷症。在暴发性亚急性硬化性全脑炎中,神经影像学可能显示广泛的皮质损伤。

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