Absence of small-vessel abnormalities in alternating hemiplegia of childhood.
OBJECTIVE
To investigate whether Japanese patients with alternating hemiplegia of childhood (AHC) have the similar small-vessel abnormalities in skin reported in European patients with AHC.


METHODS
Electron microscopic observation of biopsied skin specimens were carried out in six Japanese patients with AHC. All patients (aged 5-17, all boys) had been diagnosed with AHC through their typical clinical courses and symptoms.


RESULTS
No abnormal findings in both endothelial cells and smooth muscle cells in skin small-vessels were obtained in the present study, either in the five flunarizine responders or in the one non-responder.


CONCLUSIONS
From our observations, we hypothesized that there may be some subtypes of AHC. The diverse clinical courses in patients with AHC and the differing efficacy of flunarizine treatment could be explained because of the heterogeneity of AHC subtypes.
Sasaki
Masayuki
M
Department of Child Neurology, National Center of Neurology and Psychiatry (NCNP), 4-1-1 Ogawahigashi-cho, Kodaira, Tokyo 187-8551, Japan. masasaki@ncnp.go.jp
Matsufuji
Hironori
H
Inui
Takehiko
T
Arima
Kunimasa
K
D000293:Adolescent; D044466:Asian People; D001706:Biopsy; D001808:Blood Vessels / Q000002:abnormalities*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005444:Flunarizine / Q000627:therapeutic use; D006429:Hemiplegia / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008297:Male; D012867:Skin / Q000098:blood supply / Q000648:ultrastructure
D002121:Calcium Channel Blockers; D005444:Flunarizine