Ohmori I, Ohtsuka Y, Ogino T, Yoshinaga H, Kobayashi K, Oka E
Department of Child Neurology, Okayama University Medical School, 2 - 5 - 1, Shikatacho Okayama, Japan.
Neuropediatrics. 2002 Feb;33(1):15-20. doi: 10.1055/s-2002-23594.
To clarify the relationship between paroxysmal kinesigenic choreoathetosis (PKC) and epilepsy, we investigated the clinical and electroencephalographic (EEG) findings of patients with familial PKC and epilepsy, as well as sporadic cases with both PKC and epilepsy.
Patients consisted of 12 familial cases from seven families and three sporadic cases. The period of follow-up ranged from 17 months to 33 years, 7 months (average: 16 years, 8 months). During the follow-up, a total of 163 EEGs (11 EEGs per subject) were studied, including interictal and ictal EEGs.
Transient epileptic discharges were found in ten of the 15 patients (66.7 %) during the clinical course. As for focus, centro-midtemporal and frontal spikes were most often observed. The ictal EEG of an afebrile convulsion in one patient showed a partial seizure with secondary generalization which originated from the frontal area.
It appears that patients who suffer from both PKC and epilepsy have a functional abnormality of the cerebral cortex, particularly in the perirolandic and frontal regions.
为阐明发作性运动诱发性舞蹈手足徐动症(PKC)与癫痫之间的关系,我们调查了家族性PKC合并癫痫患者以及散发性PKC合并癫痫患者的临床和脑电图(EEG)表现。
患者包括来自7个家族的12例家族性病例和3例散发性病例。随访时间为17个月至33年7个月(平均:16年8个月)。在随访期间,共研究了163次脑电图(每位受试者11次脑电图),包括发作间期和发作期脑电图。
15例患者中有10例(66.7%)在临床过程中发现短暂性癫痫放电。就病灶而言,最常观察到中央-中颞部和额部棘波。1例患者无热惊厥的发作期脑电图显示为部分性发作继发全面性发作,起源于额叶。
似乎同时患有PKC和癫痫的患者存在大脑皮质功能异常,尤其是在中央旁小叶和额叶区域。