Stafstrom C E, Konkol R J
Department of Pediatrics, New England Medical Center Hospital, Boston, MA 02111.
Dev Med Child Neurol. 1994 Jul;36(7):576-85. doi: 10.1111/j.1469-8749.1994.tb11894.x.
The authors retrospectively identified 17 children with Down syndrome who developed infantile spasms, and analysed their etiology, EEG findings, response to medication, development and prognosis. Compared with symptomatic infantile spasms in the general population, which have a poor prognosis, these patients had a relatively benign outcome with regard to seizure control: only three of 16 survivors currently have seizures and seven of the 16 currently receive anticonvulsants. Developmental outcome was poorest in those with a superimposed hypoxic insult and in those who regressed developmentally at the onset of the spasms and did not regain developmental milestones. The overall neurological prognosis for children with Down syndrome and infantile spasms appears to be better than for children with infantile spasms in the general population.
作者回顾性地确定了17名患婴儿痉挛症的唐氏综合征患儿,并分析了他们的病因、脑电图结果、药物治疗反应、发育情况和预后。与一般人群中预后较差的症状性婴儿痉挛症相比,这些患者在癫痫控制方面预后相对良好:16名幸存者中目前只有3人仍有癫痫发作,16名中有7人目前正在接受抗惊厥药物治疗。在伴有叠加性缺氧损伤的患儿以及在痉挛发作时出现发育倒退且未重新达到发育里程碑的患儿中,发育结局最差。唐氏综合征合并婴儿痉挛症患儿的总体神经学预后似乎比一般人群中患婴儿痉挛症的患儿要好。