J Korean Child Neurol Soc.
2009 Nov;17(2):123-130.
Alternating Hemiplegia of Childhood in Korea
- Affiliations
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- 1Department of Pediatrics, School of Medicine, Chosun University, Gwangju, Korea. ryoung@chosun.ac.kr
- 2Department of Pediatrics, School of Medicine, Chonnam National University, Gwangju, Korea.
- 3Department of Pediatrics, College of Medicine, Pusan National University, Busan, Korea.
- 4Department of Pediatrics, College of Medicine, Soonchunhyang University, Cheonan, Korea.
- 5Department of Pediatrics, College of Medicine, Yeungnam University, Taegu, Korea.
- 6Department of Pediatrics, College of Medicine, Yensei University, Seoul, Korea.
- 7Department of Pediatrics, College of Medicine, Chungbuk National University, Chungju, Korea.
Abstract
- 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.
Keyword
MeSH Terms
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Age of Onset
Anticonvulsants
Ataxia
Brain
Cerebral Angiography
Child
Dystonia
Electronic Mail
Epilepsy
Eye Movements
Female
Flunarizine
Fructose
Hemiplegia
Humans
Korea
Magnetic Resonance Imaging
Neurology
Quadriplegia
Surveys and Questionnaires
Seizures
Strabismus
Tomography, Emission-Computed, Single-Photon
Anticonvulsants
Flunarizine
Fructose
Hemiplegia
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