Adult metachromatic leukodystrophy with an unusual relapsing-remitting course.
A 46 year old woman had a relapsing-remitting course of hemiparesis, disorientation, paraparesis and seizures, followed by progressive dementia, spasticity and ataxia. Computed tomography at onset showed a parietotemporal hypodense area with diffuse mottled enhancement obliterating the lateral ventricle. Subsequent scans demonstrated symmetric periventricular non-enhancing hypodensities, progressive ventricular enlargement and atrophy. Adult metachromatic leukodystrophy was diagnosed on the basis of low leukocyte arylsulphatase A level and metachromatic material accumulation at neural nerve biopsy.
Sadeh
M
M
Department of Neurology, Chaim Sheba Medical Center, Tel Hashomer, Israel.
Kuritzky
A
A
Ben-David
E
E
Goldhammer
Y
Y
D001921:Brain / Q000000981:diagnostic imaging; D002908:Chronic Disease; D003951:Diagnostic Errors; D004679:Encephalomyelitis / Q000209:etiology; D005260:Female; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008875:Middle Aged; D014057:Tomography, X-Ray Computed