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目的探讨起病于婴幼儿期的结节性硬化症病人的发病特点。方法采用回顾性调查和追踪观察的方法,分析16例起病于婴幼儿期结节性硬化症患儿的临床资料。结果16例结节性硬化症的儿童发病年龄为~3岁,均以癫痫为首发症状(100%,16/16),主要发作类为婴儿痉挛、复杂部分性发作,并有患儿从部分发作继发全身强直-阵挛发作;智力低下11例(69%,11/16),皮肤损害5例(31%,5/16)。全部患儿均有头部CT或MRI改变。结论结节性硬化症以婴儿痉挛为主要发作形式的患儿脑损害显著;脑损害程度与癫痫发作频率有关;智力低下伴有语言/言语缺陷。
Objective To investigate the incidence of tuberous sclerosis in infants and young children. Methods The clinical data of 16 infants with tuberous sclerosis in infancy were analyzed retrospectively and follow-up. Results The onset age of 16 children with tuberous sclerosis was ~ 3 years old, both of whom had epilepsy as the first symptom (100%, 16/16). The main seizures were infantile spasms and complicated partial seizures. Seizure secondary to tonic-clonic seizures; mental retardation in 11 cases (69%, 11/16), skin lesions in 5 cases (31%, 5/16). All children have head CT or MRI changes. Conclusion Tuberous sclerosis in children with spasticity as the main form of brain damage was significant; the degree of brain damage associated with the frequency of seizures; mental retardation accompanied by language / speech defects.