Roulet E, Deonna T, Gaillard F, Peter-Favre C, Despland P A
Unité de Neuropédiatrie, CHUV, Lausanne, Switzerland.
Epilepsia. 1991 Jul-Aug;32(4):495-503. doi: 10.1111/j.1528-1157.1991.tb04683.x.
Severe persistent neuropsychological disorders sometimes develop in the course of a focal epilepsy of unknown origin in previously normal children. Very frequent bilateral focal or generalized discharges are often noted on the sleep EEG records of these patients with no evidence of clinical seizures. The relation between these paroxysms and the observed deterioration remains unclear. We report a child with a partial complex epilepsy and severe disturbances of language, cognition, and behavior acquired in the early years of development who was followed for 15 years. A correlation between the evolution of the striking EEG abnormalities during sleep and the neuropsychological disorders could be established retrospectively. The observed sequence of onset and recovery of the aphasia, the dementia, and the "psychotic" behavior makes a direct causal relation between the deficits quite unlikely. Rather it suggests an association of independent symptoms with a specific language disorder becoming manifest in the course of the evolution. This child shows many of the main characteristics of the syndromes of "acquired aphasia with convulsive disorder" (Landau-Kleffner syndrome) and "epilepsy with continuous spike waves during sleep." Both syndromes describe probably different facets of a similar underlying, still unexplained cerebral dysfunction.
严重的持续性神经心理障碍有时会在原本正常的儿童不明原因局灶性癫痫病程中出现。在这些无临床癫痫发作证据的患者睡眠脑电图记录中，常可见频繁的双侧局灶性或全身性放电。这些发作与所观察到的神经功能恶化之间的关系仍不清楚。我们报告了一名患有部分性复杂性癫痫且在发育早期出现严重语言、认知和行为障碍的儿童，对其进行了15年的随访。回顾性分析发现，睡眠期间显著脑电图异常的演变与神经心理障碍之间存在相关性。所观察到的失语、痴呆和“精神病性”行为的发作和恢复顺序，使得这些缺陷之间不太可能存在直接因果关系。相反，这表明在演变过程中，独立症状与一种特定语言障碍之间存在关联。这名儿童表现出“伴有惊厥性障碍的获得性失语”（Landau-Kleffner综合征）和“睡眠期持续性棘波癫痫”综合征的许多主要特征。这两种综合征可能描述了类似潜在但仍无法解释的脑功能障碍的不同方面。
