Chae J H, Kim S K, Wang K C, Kim K J, Hwang Y S, Cho B K
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Epilepsia. 2001 Sep;42(9):1204-7. doi: 10.1046/j.1528-1157.2001.43398.x.
The cerebellum is known to have an inhibitory effect on seizures. Nevertheless, cerebellar dysplastic lesions can be epileptogenic. A 4-month-old infant had paroxysmal facial contractions; tachypnea and nystagmoid eyeball and tremulous movements were occasionally combined. These evolved to stereotypic clinical patterns and frequencies, which increased despite administration of antiepileptic drugs (AEDs). Magnetic resonance imaging (MRI) demonstrated a mass arising from the superior cerebellar peduncle, although video-scalp EEG monitoring revealed no abnormal findings. Positron emission tomography with [(18)F]fluorodeoxyglucose revealed focal hypermetabolism in the same area identified by MRI. A depth electrode implanted in the mass revealed focal spike-and-wave discharges. The lesion was partly removed; pathologic diagnosis was ganglioglioma. Because of incomplete seizure control and residual tumor visible on MRI, a second operation was performed. After complete excision of the tumor, the patient became seizure free without AEDs. This case confirms the presence of seizure originating from the cerebellum and emphasizes the need for the complete removal of an epileptogenic lesion.
已知小脑对癫痫发作有抑制作用。然而，小脑发育异常性病变可致痫。一名4个月大的婴儿出现阵发性面部抽搐；偶尔合并呼吸急促、眼球震颤样眼球运动和震颤。这些症状演变为刻板的临床模式和发作频率，尽管使用了抗癫痫药物（AEDs），发作频率仍增加。磁共振成像（MRI）显示小脑上脚有一肿块，尽管视频头皮脑电图监测未发现异常。[(18)F]氟脱氧葡萄糖正电子发射断层扫描显示在MRI确定的同一区域有局灶性代谢亢进。植入肿块的深部电极显示局灶性棘波和慢波放电。部分切除病变；病理诊断为神经节胶质瘤。由于癫痫发作控制不完全且MRI可见残留肿瘤，遂进行了第二次手术。肿瘤完全切除后，患者在未使用AEDs的情况下不再发作。该病例证实存在起源于小脑的癫痫发作，并强调完全切除致痫性病变的必要性。
