室管膜下结节性异位神经元迁移障碍中的阅读障碍
Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia.
作者信息
Chang B S, Ly J, Appignani B, Bodell A, Apse K A, Ravenscroft R S, Sheen V L, Doherty M J, Hackney D B, O'Connor M, Galaburda A M, Walsh C A
机构信息
Division of Neurogenetics, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA.
出版信息
Neurology. 2005 Mar 8;64(5):799-803. doi: 10.1212/01.WNL.0000152874.57180.AF.
OBJECTIVE
To define the behavioral profile of periventricular nodular heterotopia (PNH), a malformation of cortical development that is associated with seizures but reportedly normal intelligence, and to correlate the results with anatomic and clinical features of this disorder.
METHODS
Ten consecutive subjects with PNH, all with epilepsy and at least two periventricular nodules, were studied with structural MRI and neuropsychological testing. Behavioral results were statistically analyzed for correlation with other features of PNH.
RESULTS
Eight of 10 subjects had deficits in reading skills despite normal intelligence. Processing speed and executive function were also impaired in some subjects. More marked reading difficulties were seen in subjects with more widely distributed heterotopia. There was no correlation between reading skills and epilepsy severity or antiepileptic medication use.
CONCLUSION
The neuronal migration disorder of periventricular nodular heterotopia is associated with an impairment in reading skills despite the presence of normal intelligence.
目的
明确室周结节性异位（PNH）的行为特征，这是一种皮质发育畸形，与癫痫相关，但据报道智力正常，并将结果与该疾病的解剖学和临床特征相关联。
方法
对连续10例患有PNH的受试者进行研究，所有受试者均患有癫痫且至少有两个室周结节，采用结构磁共振成像（MRI）和神经心理学测试。对行为结果进行统计分析，以与PNH的其他特征进行相关性研究。
结果
10名受试者中有8名尽管智力正常，但阅读技能存在缺陷。部分受试者的处理速度和执行功能也受损。在异位分布更广泛的受试者中，阅读困难更为明显。阅读技能与癫痫严重程度或抗癫痫药物使用之间无相关性。
结论
尽管智力正常，但室周结节性异位的神经元迁移障碍与阅读技能受损有关。
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