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KMID : 0391520090170020123
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Journal of the Korean Child Neurology Society
2009 Volume.17 No. 2 p.123 ~ p.130
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Alternating Hemiplegia of Childhood in Korea
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Rho Young-Il
Woo Young-Jong
Nam Sang-Ook
Kim Young-Chang
Moon Han-Ku
Lee Joon-Soo
Kim Won-Seop
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Abstract
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Purpose: Alternating hemiplegia of childhood(AHC) is a rare neurologic syndrome of unknown pathophysiology. The typical clinical features are characterized by recurrent hemiplegic attacks shifting from one side to the other, beginning before 18 months, dystonia, strabismus, nystagmus, epilepsy, and immediate resolution of all symptoms during sleep. This study is to access clinical features and outcome of treatment of AHC in Korea.
Methods: We sent standardized questionnaires by e-mail to 118 council members of the Korean Society of Child Neurology. We received it through e-mail from 7 centers and analyzed clinical characteristics, treatment, and outcomes.
Results:We collected 11 AHC patients(male 6, female 5). The mean age was 6.13±5.23 years. The mean age of onset of hemiplegia was 9.80±6.67 months. The mean frequency of episode was 10.73±11.44 per month and mean duration was 3.10±2.50 days. The manifestations were hemiplegia in 100%, dystonia in 81.8%, abnormal eye movements in 81.8%, developmental delay in 81.8%, seizure in 72.7%, autonomic phenomena in 54.5%, ataxia 45.5%, and episodes of quadriparesis in 45.5%. The onset age of seizure was 3-84 months. Psychomotor retardation was more severe in patients with seizures than in those without seizures. Magnetic resonance imaging of brain and cerebral angiography were normal in all patients. Interictal SPECT shows hypoperfusion in the frontal in 2 of 5 patients. Nine patients received flunarizine and antiepileptic drugs but two patients received antiepileptic drugs only. A favorable response was reported in 45.5%, which included excellent response to the combination therapy of flunarizine and high dose topiramate.
Conclusions: Patients with seizures faired the poorer psychomotor retardation. The combination of flunarizine and high dose topiramate was very effective in AHC.
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KEYWORD
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Alternating Hemiplegia, Children
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