Progressive dystonia with bilateral putaminal hypodensities.
Three unrelated patients, aged 4, 18, and 47 years, had generalized dystonia associated with bilateral striatal hypodensities on computed tomography. Mitochondrial encephalopathy was considered to be the most likely diagnosis, but this could not be proved. These patients confirm previous reports linking acquired generalized dystonia with bilateral putaminal lesions and they highlight the problem in differential diagnosis of this clinicoradiologic syndrome.
Berkovic
S F
SF
Montreal Neurological Institute, Quebec, Canada.
Karpati
G
G
Carpenter
S
S
Lang
A E
AE
D000293:Adolescent; D001921:Brain / Q000648:ultrastructure; D001927:Brain Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D002675:Child, Preschool; D004421:Dystonia / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D007888:Leigh Disease / Q000150:complications; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000648:ultrastructure; D011699:Putamen / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed