Papazian O, Cañizales E, Alfonso I, Archila R, Duchowny M, Aicardi J
Department of Neurology, Miami Children's Hospital, Miami, FL, USA.
Ann Neurol. 1995 Oct;38(4):687-91. doi: 10.1002/ana.410380423.
Two children developed severe cognitive and behavioral deterioration suggestive of a degenerative disease while being treated with sodium valproate for idiopathic, localization-related epilepsy with centrotemporal spikes. Magnetic resonance imaging revealed marked central and generalized cortical and cerebellar atrophy. In both patients, clinical symptoms and signs cleared in a few weeks following valproate withdrawal. The magnetic resonance imaging appearance improved within 3 months in 1 of the patients and normalized in both after 6 and 12 months. No metabolic changes were associated with the clinical or imaging abnormalities. Although the mechanism of this rare idiosyncratic complication of valproate therapy is unknown, we advocate discontinuing valproate therapy in all epileptic patients with neuromental deterioration or brain atrophy of unknown etiology.
两名患有特发性、与中央颞部棘波相关的局限性癫痫的儿童在接受丙戊酸钠治疗时出现了严重的认知和行为衰退,提示患有退行性疾病。磁共振成像显示明显的中枢性和全身性皮质及小脑萎缩。两名患者在停用丙戊酸钠后的几周内临床症状和体征均消失。其中一名患者的磁共振成像表现在3个月内有所改善,另一名患者在6个月和12个月后均恢复正常。未发现代谢变化与临床或影像学异常相关。尽管丙戊酸钠治疗这种罕见的特异质性并发症的机制尚不清楚,但我们主张在所有病因不明的神经精神衰退或脑萎缩的癫痫患者中停用丙戊酸钠治疗。