Segawa disease.
A 14-year-old girl with Segawa disease is reported. The dystonic movement began at the age of three and slow progressive deterioration followed. The girl became bed-ridden and required assistance for all activities of daily living. Diurnal fluctuation and sleep benefit was clearly observed. Inheritence appears to be autosomal dominant. A dramatic, immediate and sustained response to L-dopa therapy was noted. This disorder is a form of treatable dystonia in childhood and adolescence and it is recommended that patients in this age group with primary dystonia should be given a trial of low dose L-dopa even though they have a chronic course or severe disability.
Phanthumchinda
K
K
Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Vichichanyakul
M
M
Yodnophaklao
P
P
D000293:Adolescent; D000978:Antiparkinson Agents / Q000627:therapeutic use*; D004421:Dystonia / Q000188:drug therapy* / Q000235:genetics; D005260:Female; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use*
D000978:Antiparkinson Agents; D007980:Levodopa