Riikonen R
Neuropediatrics. 1982 Feb;13(1):14-23. doi: 10.1055/s-2008-1059590.
The long-term prognosis of 192 surviving children with the syndrome of infantile spasms was evaluated. The children had been admitted to three paediatric hospitals in Helsinki at the time of initial diagnosis. The aetiological factors of the syndrome were carefully studied in each case. ACTH therapy was employed in 162, usually for about six weeks. The follow-up study 3-19 (mean 10.4) years later was made at the Children's Hospital, University of Helsinki. The rate of mortality was 19.6 per cent. Normal development was seen in 12 per cent and slightly subnormal in 10.4 per cent of the surviving children. Psychiatric disorders were seen in 27.6 per cent of the survivors. Sensory defects were also common. Severe cerebral palsy was seen in 4 per cent. Other seizures after cessation of the infantile spasms were seen in 60 per cent. Serial EEG studies showed that the temporal lobe was the most common site of abnormality. Abnormalities in the temporal lobes were seen frequently in children with symptomatic neonatal hypoglycaemia as a probable cause of the spasms. Prognostically favourable factors were "idiopathic" aetiology, normal development and not other fits prior to the spasms, short treatment lag, good response to ACTH and short duration of the spasms. In this study early treatment seemed to be of great importance even with regard to mental development. The factors connected with a bad outcome were: symptomatic aetiology (especially brain malformations, early infections and tuberous sclerosis), slow development before spasms, other seizures before infantile spasms, early onset of the spasms, long treatment lag, long duration of the spasms and other later occurrence of myoclonic-astatic seizures (Lennox-Gastaut). Large doses of ACTH (120-160 units) were not associated with a better prognosis than the smaller doses (20-40 units). The benefit of long versus short treatment schedules could not be evaluated in this study. The relapse rate here was 32 per cent.
对192名存活的婴儿痉挛综合征患儿的长期预后进行了评估。这些患儿在初次诊断时被收治于赫尔辛基的三家儿科医院。对每例患儿的综合征病因进行了仔细研究。162例患儿采用了促肾上腺皮质激素（ACTH）治疗，通常持续约六周。在赫尔辛基大学儿童医院进行了3至19年（平均10.4年）后的随访研究。死亡率为19.6%。12%的存活患儿发育正常，10.4%的患儿发育略低于正常水平。27.6%的幸存者存在精神障碍。感觉缺陷也很常见。4%的患儿患有严重脑瘫。婴儿痉挛停止后，60%的患儿出现其他癫痫发作。系列脑电图研究表明，颞叶是最常见的异常部位。有症状的新生儿低血糖作为痉挛的可能病因，在患儿中颞叶异常较为常见。预后良好的因素包括“特发性”病因、发育正常、痉挛前无其他发作、治疗延迟短、对ACTH反应良好以及痉挛持续时间短。在本研究中，即使对于智力发育而言，早期治疗似乎也非常重要。与不良预后相关的因素包括：症状性病因（尤其是脑畸形、早期感染和结节性硬化症）、痉挛前发育迟缓、婴儿痉挛前有其他发作、痉挛发作早、治疗延迟长、痉挛持续时间长以及后期出现其他肌阵挛-失张力发作（Lennox-Gastaut综合征）。大剂量ACTH（120 - 160单位）与小剂量（20 - 40单位）相比，预后并无更好。本研究无法评估长疗程与短疗程治疗的益处。这里的复发率为32%。
