Furby A, Vallée L, Rousseaux M, Nuyts J P, Destée A
Service de Clinique Neurologique, C.H.U., Lille.
Rev Neurol (Paris). 1990;146(3):191-5.
Long survival in subacute sclerosing panencephalitis (SSPE), including total disappearance of clinical signs, is rare. Two cases are reported. They concern a girl and a boy who, at age 13 and 15, developed SSPE and are still in remission 6 and 5 years later. After a typical onset and course over periods of 12 and 18 months, clinical improvement was observed and periodic EEG complexes disappeared. However, the electrophoretic oligoclonal pattern of CSF proteins and the elevated measles titers persisted (in one case specific CSF IgM were still increased 6 years after the onset). MRI showed asymmetrical areas of high-intensity signal in both white and gray matter, predominant in the temporal, parietal and occipital regions. The age at which SSPE begins and the interval between measles and SSPE onset are not prognostic factors. On the other hand, in reported cases with lasting remission SSPE did not progress beyond Jabbour's stage II. The second typical feature of these long-term improvements is disappearance of EEG periodic complexes and emergence of a normal basic background activity. No other prognostic factor has been reported.
亚急性硬化性全脑炎(SSPE)患者长期存活,包括临床症状完全消失的情况十分罕见。本文报告了两例病例。其中一例为女孩,另一例为男孩,他们分别在13岁和15岁时患上SSPE,6年和5年后仍处于缓解期。在经历了12个月和18个月的典型发病过程后,临床症状出现改善,周期性脑电图复合波消失。然而,脑脊液蛋白的电泳寡克隆图谱以及麻疹抗体滴度仍持续存在(其中一例在发病6年后脑脊液特异性IgM仍升高)。磁共振成像(MRI)显示,白质和灰质均有不对称的高强度信号区域,主要集中在颞叶、顶叶和枕叶区域。SSPE起病年龄以及麻疹与SSPE发病之间的间隔时间并非预后因素。另一方面,在报告的持续缓解病例中,SSPE病情未进展至贾布尔(Jabbour)II期以上。这些长期改善的第二个典型特征是脑电图周期性复合波消失,出现正常的基本背景活动。尚未有其他预后因素的报道。