Boulloche J, Dautreme P, Le Luyer B, Mallet E, Tron P
Département de Pédiatrie, Centre Hospitalier, Le Havre.
Ann Pediatr (Paris). 1991 Feb;38(2):71-4.
The prognosis of infantile spasms is grim when a detectable brain lesion is present. In contrast, cryptogenic infantile spasms, in which there is no identifiable brain lesion, usually run a favorable course under treatment. Few studies have focused on the outcome in children with cryptogenic infantile spasms. Among 111 pediatric patients with a history of infantile spasms hospitalized over 18 consecutive years, 23 (21%) were given a diagnosis of cryptogenic infantile spasms. Follow-up ranged from 4 to 21 years. Outcome was as follows: the IQ was above 80 in 39% of cases and above 100 in 13% of cases; 42% of patients of school age were attending school but half of these had learning disabilities; 30% of patients had severe psychiatric disorders, and 22% had developmental delay and severe epilepsia. Early factors apparently associated with a good prognosis included the mild nature of psychomotor regression, persistence of spindles on EEGs recorded during NREM sleep, and prompt improvement of clinical status and EEG recordings under treatment. Conversely, severe regression, focalized EEG anomalies, failure of development to resume promptly after initiation of therapy, recurrence of spasms and hypsarrhythmia at discontinuation of treatment, and onset before 5 months or after 11 years of age were associated with a poor prognosis.
当存在可检测到的脑损伤时，婴儿痉挛症的预后严峻。相比之下，隐源性婴儿痉挛症（即不存在可识别的脑损伤）在接受治疗时通常预后良好。很少有研究关注隐源性婴儿痉挛症患儿的预后情况。在连续18年住院的111例有婴儿痉挛症病史的儿科患者中，23例（21%）被诊断为隐源性婴儿痉挛症。随访时间为4至21年。结果如下：39%的病例智商高于80，13%的病例智商高于100；42%达到学龄的患者在上学，但其中一半有学习障碍；30%的患者有严重精神障碍，22%的患者有发育迟缓及严重癫痫。明显与良好预后相关的早期因素包括精神运动发育倒退程度较轻、非快速眼动睡眠期脑电图记录中纺锤波持续存在，以及治疗期间临床状况和脑电图记录迅速改善。相反，严重倒退、脑电图局灶性异常、治疗开始后发育未能迅速恢复、治疗中断时痉挛和高峰失律复发，以及在5个月前或11岁后发病与预后不良相关。
