结节性硬化症中婴儿痉挛、结节与智力的关系。
The relation of infantile spasms, tubers, and intelligence in tuberous sclerosis complex.
作者信息
O'Callaghan F J K, Harris T, Joinson C, Bolton P, Noakes M, Presdee D, Renowden S, Shiell A, Martyn C N, Osborne J P
机构信息
Department of Paediatric Neurology, Bristol Royal Hospital for Children, Institute of Child Health, Upper Maudlin Street, Bristol BS2 8BJ, UK.
出版信息
Arch Dis Child. 2004 Jun;89(6):530-3. doi: 10.1136/adc.2003.026815.
BACKGROUND
The aetiology of the learning difficulty in tuberous sclerosis is debated. It may be related to the amount of tubers in the brain or caused by the infantile spasms that occur in early life.
AIMS
To examine the relative contributions to final intelligence (IQ) made by both cerebral tubers and infantile spasms.
METHODS
As part of an epidemiological study of tuberous sclerosis in the south of England, patients were recruited who were able to undergo magnetic resonance imaging (MRI) without the need for an anaesthetic. Epilepsy history was determined by interview and review of clinical records. IQ was assessed using either Wechsler intelligence scales or Raven's matrices.
RESULTS
A total of 41 patients consented to have an MRI scan. IQ scores were normally distributed about a mean of 91. Twenty six patients had a positive history of epilepsy, and 11 had suffered from infantile spasms. There was a significant relation between the number of tubers and IQ. Infantile spasm status partly confounded the relation between tubers and IQ, but did not render the relation statistically insignificant. The relation between infantile spasms and learning difficulty remained strong even when controlling for the number of tubers.
背景
结节性硬化症学习困难的病因存在争议。它可能与大脑中结节的数量有关，或者由早年发生的婴儿痉挛症引起。
目的
研究脑结节和婴儿痉挛症对最终智力（智商）的相对影响。
方法
作为英格兰南部结节性硬化症流行病学研究的一部分，招募了无需麻醉即可接受磁共振成像（MRI）检查的患者。通过访谈和查阅临床记录确定癫痫病史。使用韦氏智力量表或瑞文推理测验评估智商。
结果
共有41名患者同意进行MRI扫描。智商分数呈正态分布，平均分为91。26名患者有癫痫阳性病史，11名曾患婴儿痉挛症。结节数量与智商之间存在显著关系。婴儿痉挛症状态部分混淆了结节与智商之间的关系，但并未使该关系在统计学上无意义。即使在控制结节数量的情况下，婴儿痉挛症与学习困难之间的关系仍然很强。
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