Department of Pediatric Neurology, Catholic University, Rome, Italy.
Neuromuscul Disord. 2013 Apr;23(4):313-5. doi: 10.1016/j.nmd.2013.01.011. Epub 2013 Mar 7.
Cognitive and behavioral difficulties occur in approximately a third of patients with Duchenne muscular dystrophy. The aim of our study was to assess the prevalence of epilepsy in a cohort of 222 DMD patients. Epileptic seizures were found in 14 of the 222 DMD patients (6.3%). The age of onset ranged from 3 months to 16 years (mean 7.8). Seizures were more often focal epilepsy (n=6), generalized tonic-clonic seizures (n=4) or absences (n=4). They were present in 12 of the 149 boys with normal IQ (8.1%) and in two of the 73 with mental retardation (2.7%). In two cases the parents did not report any past or present history of seizures but only 'staring episodes' interpreted as a sign of 'poor attention'. In both patients EEG showed the typical pattern observed in childhood absence epilepsy. Our results suggest that the prevalence of epilepsy in our study (6.3%) is higher than in the general pediatric population (0.5-1%). The risk of epilepsy does not appear to increase in patients with mental retardation.
约三分之一的杜氏肌营养不良症患者存在认知和行为障碍。我们的研究旨在评估 222 例 DMD 患者中癫痫的患病率。在 222 例 DMD 患者中发现 14 例癫痫发作（6.3%）。发病年龄为 3 个月至 16 岁（平均 7.8 岁）。癫痫发作更常见于局灶性癫痫（n=6）、全面强直阵挛发作（n=4）或失神发作（n=4）。在 149 名智商正常的男孩中（8.1%）有 12 例，在 73 名智力迟钝的男孩中（2.7%）有 2 例。在两种情况下，父母均未报告过去或现在有癫痫发作史，仅报告“凝视发作”，被解释为“注意力不佳”的迹象。在这两例患者中，脑电图显示出与儿童失神性癫痫一致的典型模式。我们的研究结果表明，我们的研究（6.3%）中的癫痫患病率高于一般儿科人群（0.5-1%）。癫痫的风险似乎不会在智力迟钝的患者中增加。
