선천안운동행위상실증의 과거력을 동반한 학령기 현훈성 간질 1예

Abstract

Congenital ocular motor apraxia (COMA) is an infrequently reported eye movement disorder associated with malformation or delayed maturation of the brain. Patients with COMA are unable to initiate voluntary horizontal saccades. This results in characteristic head thrusts into the desired field of gaze to compensate for the lack of saccadic ability. Epileptic vertigo is a rare condition in childhood, and sudden vertigo or sudden falls as an aura of epilepsy is characteristic. Here we report a 13-year-old boy who was revealed to have COMA and epileptic vertigo. The patient presented with a history of frequent dizziness over the previous 2 weeks. He was diagnosed with COMA at 11 months after birth. His eye movement showed direction-changing nystagmus, impaired smooth pursuit, asymmetric optokinetic nystagmus and an inaccurate saccade test, which were findings of COMA. There were no other abnormal neurologic findings. MR imaging of the temporal area and brain revealed a right dysmorphic lateral ventricle and EEG revealed the presence of one episode of less well organized generalized slow spike and wave discharge in both frontal dominancy during the ictal period. These findings suggested a generalized seizure disorder originating from the frontal areas. The patient was treated with valproic acid and the seizures became well-controlled