Cole A J, Andermann F, Taylor L, Olivier A, Rasmussen T, Robitaille Y, Spire J P
Montreal Neurological Hospital and Institute, McGill University, Quebec, Canada.
Neurology. 1988 Jan;38(1):31-8. doi: 10.1212/wnl.38.1.31.
The syndrome of acquired verbal auditory agnosia in childhood with mutism and epileptic discharges has been described in over 100 cases. An encephalitic etiology has often been postulated but never proved. We report two patients with this syndrome who were treated surgically. Despite careful search, no pathologic evidence of encephalitis was found. One patient, with the typical course, had no seizures but striking positive correlation between epileptic discharge and language disorder; the second, after classic onset, developed intractable temporal lobe epilepsy, a previously unreported outcome of this syndrome. EEG discharges are generalized, bilateral, multifocal, or with shifting predominance but mainly temporal in 85% of reported cases, and unilateral, also predominantly temporal, in 15%. Language areas are preferentially involved. This syndrome has certain biologic features that resemble the benign epilepsies of childhood and may be the result of the unusual localization of the epileptic abnormality.
儿童期获得性言语听觉失认症伴缄默和癫痫放电的综合征已在100多例病例中得到描述。脑炎病因常被推测，但从未得到证实。我们报告了两名接受手术治疗的该综合征患者。尽管进行了仔细检查，但未发现脑炎的病理证据。一名患者病程典型，无癫痫发作，但癫痫放电与语言障碍之间存在显著正相关；第二名患者在典型发病后发展为顽固性颞叶癫痫，这是该综合征以前未报告过的结果。脑电图放电为全身性、双侧性、多灶性或优势部位多变，但在85%的报告病例中主要位于颞叶，15%为单侧性，也主要位于颞叶。语言区域优先受累。该综合征具有某些类似于儿童良性癫痫的生物学特征，可能是癫痫异常异常定位的结果。
