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Á¢¼ö¹øÈ£ : 20081-P0014
Á¦¸ñ : A Case of Atypical Infantile Spasms Preceded by Tonic Seizures of Focal Onset

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ÀúÀÚ : Byung Chan Lim* ,Kyung Nam Koh, Anna Cho, Hee Hwang, Jong Hee Chae, Ki Joong Kim, Yong Seung Hwang
¼Ò¼Ó : Department of Pediatrics, College of Medicine, Seoul National University*
 
Purpose : Determination of seizure types in infancy is often demanding, since seizures in this period are often subtle and difficult to lateralize based on clinical features alone. We report a case of atypical infantile spasm preceded by tonic seizures of focal onset confirmed through long-term video-electroencephalogram monitoring. (LTVEM)

Case : A 4-month-old male infant was referred to our pediatric neurology clinic for delayed developmental milestones. He could hold head in upright position, however, could not have visual fixation since birth. On ophthalmologic evaluation, cortical visual impairment was suspicious, however, brain magnetic resonance imaging revealed no specific abnormality. After the age of 5 months, he showed daily seizure like activity. According to parent¡¯s description, he showed mixed symptoms of motion arrest, tonic eye ball deviation, and irritability with crying sometimes accompanied by brief tonic posture. Serial interictal EEG revealed transition from atypical burst suppression pattern to hypsarrythmia. LTVEM was performed for clarifying the seizure pattern. It started with brief 4-extremities tonic posture with mild eye ball deviation to the right side. Then, sequential right leg and right arm elevation and tonic spasms of both arms repeated in one cluster. Ictal EEG correlated with tonic seizures revealed mild diffuse attenuation of background activity with focal spike or rhythmic theta activity on the left parietal or right temporal area, which suggests focal onset. Atypical complex of infantile spasms was matched with diffuse theta/delta bursts followed by diffuse attenuation. We concluded he had spasms with partial seizures as a single ictal event. After vigabatrin medication, seizure frequency gradually decreased.

Conclusions : Accurate diagnosis of partial seizures with atypical spasms in infancy is often difficult. In infancy, LTVEM can help determining seizure semiology and optimal treatment.