Department of Paediatric Neuroscience, IRCCS Foundation Neurological Institute C. Besta, Via Celoria 11, 20133, Milan, Italy.
Neurol Sci. 2011 Dec;32 Suppl 3:S303-6. doi: 10.1007/s10072-011-0697-y.
In patients with Chiari malformation type 1 (CMI), epileptic seizures are occasionally reported both in symptomatic patients candidate to surgery and in patients without symptoms of tonsillar displacement in whom CM1 is often an incidental finding in the diagnostic work up for idiopathic epilepsies. In both groups of patients, the course of epilepsy is almost invariably favorable, with a few seizures easily controlled by treatment. In a subset of CM1 patients, epilepsy occurs in the context of neurodevelopmental disorders that also include mental retardation, autism and somatic dysmorphisms. Epileptic seizures must be accurately differentiated by potentially harmful paroxysmal events due to compression of the medulla, particularly by the cerebellar fits characterized by drop attacks, abnormal extensor posturing and apnea.
在 Chiari 畸形 1 型(CMI)患者中,癫痫发作偶尔在有症状的手术候选患者和无症状的扁桃体移位患者中报告,在这些患者中,CM1 通常是特发性癫痫诊断过程中的偶然发现。在这两组患者中,癫痫的病程几乎总是良好的,少数癫痫发作通过治疗很容易得到控制。在一小部分 CMI 患者中,癫痫发生在包括智力迟钝、自闭症和躯体畸形等神经发育障碍的背景下。癫痫发作必须通过可能因延髓受压引起的有害阵发性事件(特别是由小脑发作引起的猝倒、异常伸展姿势和呼吸暂停)准确地区分。