Lin Y, Itomi K, Takada H, Kuboda T, Okumura A, Aso K, Negoro T, Watanabe K
Department of Pediatrics, Nagoya University School of Medicine, Japan.
Neuropediatrics. 1998 Oct;29(5):268-71. doi: 10.1055/s-2007-973573.
We report video-EEG findings and a long-term follow-up study in 10 patients with benign myoclonic epilepsy in infants (BMEI). A high incidence of a past and family history of febrile convulsions was noted. Six of the 10 patients manifested characteristic vocalization associated with myoclonic seizures. It consisted of a sudden, brief expiratory noise and is considered to be characteristic of BMEI. Afebrile convulsions occurred before the onset of myoclonic seizures or during the clinical course in six patients, but the accurate type of these seizures remains to be clarified. Monotherapy with valproatic acid (VPA) was very effective, but plasma VPA levels over 100 micrograms/ml were initially necessary in most of the cases, although they did not need to be maintained for further seizure control. All patients showed a favorable long-term seizure outcome, although one showed moderate mental retardation.
我们报告了10例婴儿良性肌阵挛癫痫（BMEI）患者的视频脑电图检查结果及长期随访研究。发现热性惊厥的既往史和家族史发生率很高。10例患者中有6例表现出与肌阵挛发作相关的特征性发声。它由突然、短暂的呼气性噪音组成，被认为是BMEI的特征。6例患者在肌阵挛发作之前或临床过程中出现无热惊厥，但其确切发作类型仍有待明确。丙戊酸（VPA）单药治疗非常有效，但在大多数病例中，最初血浆VPA水平需要超过100微克/毫升，不过进一步控制癫痫发作并不需要维持该水平。所有患者的癫痫发作长期预后良好，尽管有1例存在中度智力发育迟缓。
