A kindred of hereditary adult-onset leukodystrophy with sparing of the optic radiations.
We describe a family with hereditary adult-onset leukodystrophy with sparing of the optic radiations, shown clinically, neuroradiologically and electrophysiologically. All five affected members developed their leukodystrophy in their fourth decade and clinical features steadily progressed. The sparing of the optic radiations is not typical of other leukodystrophies. We suggest that this is a new clinical entity.
Department of Neurology, Osaka University Medical School, Japan.
K
K
Abe
M
M
Ikeda
K
K
Watase
H
H
Tanabe
H
H
Fujimura
S
S
Yorifuji
S
S
Ueno
T
T
Mezaki
T
T
Mori
D000328:Adult; D002531:Cerebellum / Q000473:pathology; D002544:Cerebral Infarction / Q000175:diagnosis / Q000235:genetics; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000175:diagnosis / Q000235:genetics*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000235:genetics*; D011712:Pyramidal Tracts / Q000473:pathology