Shivji Zaitoon M, Al-Zahrani Ibrahim S, Al-Said Yousef A, Jan Mohammed M S
Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Jeddah, Kingdom of Saudi Arabia.
Can J Neurol Sci. 2003 Nov;30(4):384-7. doi: 10.1017/s0317167100003127.
Subacute sclerosing panencephalitis (SSPE) is a rare complication of measles virus infection. The disease is characterized by behavioural abnormalities, intellectual deterioration, motor weakness, and generalized myoclonic jerks progressing to coma and death in one to two years in 80% of the cases. The myoclonic jerks are associated with characteristic generalized slow periodic complexes on electroencephalography (EEG). The symptoms and signs of SSPE are frequently quite variable. The clinical course is equally variable and difficult to predict. The characteristic periodic myoclonus can rarely occur unilaterally particularly in the early stages of the disease. As well, the periodic EEG complexes have been reported unilaterally in up to 3% of cases.
A 12-year-old boy, who was seen at a later stage with atypical manifestation of myoclonic body jerks confined entirely unilaterally, combined with contralateral periodic EEG complexes. One could assume clinically that the more diseased hemisphere was responsible for generating the jerks. However, brain magnetic resonance imaging revealed asymmetric hemispheric changes suggesting that the less neurologically damaged hemisphere is responsible for generating the unilateral myoclonic jerks. This has led to the interpretation that the more severely damaged hemisphere has lost the neuronal connectivity required to generate these periodic myoclonic jerks.
Subacute sclerosing panencephalitis may have asymmetric hemispheric involvement, not only early, but also in the advanced stages of the disease, which can result in unilateral periodic myoclonic jerks.
亚急性硬化性全脑炎（SSPE）是麻疹病毒感染的一种罕见并发症。该疾病的特征为行为异常、智力衰退、运动无力以及全身性肌阵挛性抽搐，80%的病例会在一到两年内发展至昏迷和死亡。肌阵挛性抽搐与脑电图（EEG）上特征性的全身性慢周期性复合波相关。SSPE的症状和体征常常变化很大。临床病程同样多变且难以预测。特征性的周期性肌阵挛很少单侧出现，尤其是在疾病早期。此外，据报道，高达3%的病例中EEG周期性复合波单侧出现。
一名12岁男孩，在疾病后期出现非典型表现，其肌阵挛性身体抽搐完全局限于单侧，同时伴有对侧EEG周期性复合波。临床上可以推测，病变较重的半球是产生抽搐的原因。然而，脑磁共振成像显示半球不对称性改变，提示神经损伤较轻的半球是产生单侧肌阵挛性抽搐的原因。这导致一种解释，即损伤更严重的半球已失去产生这些周期性肌阵挛性抽搐所需的神经元连接。
亚急性硬化性全脑炎可能不仅在疾病早期，而且在疾病晚期都有半球不对称受累情况，这可导致单侧周期性肌阵挛性抽搐。
