Putaminal necrosis presenting with hemidystonia.
A 7-year-old female presented with putaminal necrosis associated with hemidystonia. Cranial magnetic resonance imaging revealed bilateral putaminal lesions appearing as hypointense signals on T(1)-weighted images and hyperintense signals on T(2)-weighted images. After a differential diagnosis of basal ganglial degeneration was made, putaminal necrosis was diagnosed. Low doses of levodopa (0.5 mg/kg daily) were administered, but her clinical signs worsened. Positron emission tomography scanning with [(18)F]-6-fluoro-L-dopa revealed asymmetric uptake and right-sided dominant decreases of [(18)F]-6-fluoro-L-dopa uptake of the putamen. On the basis of these findings, standard doses of levodopa (10 mg/kg daily) were administered, and her clinical signs improved. These results suggest that hemidystonia is associated with a disturbance of the dopamine system.
Kawano
H
H
Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Takeuchi
Y
Y
Misawa
A
A
Sawada
T
T
Imahori
Y
Y
D000978:Antiparkinson Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D001927:Brain Diseases / Q000175:diagnosis; D002648:Child; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives; D004305:Dose-Response Relationship, Drug; D004421:Dystonia / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D005260:Female; D005462:Fluorine Radioisotopes; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007980:Levodopa / Q000008:administration & dosage / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D009336:Necrosis; D011699:Putamen / Q000000981:diagnostic imaging / Q000473:pathology*; D014055:Tomography, Emission-Computed; D016896:Treatment Outcome
D000978:Antiparkinson Agents; D005462:Fluorine Radioisotopes; D019788:Fluorodeoxyglucose F18; C043437:fluorodopa F 18; D007980:Levodopa; D004295:Dihydroxyphenylalanine