Dementia with impaired temporal glucose metabolism in late-onset metachromatic leukodystrophy.
An unusual case of very-late-onset metachromatic leukodystrophy (MLD) with dementia was studied. The patient was a 41-year-old male who presented with mild dementia and a single generalized tonic clonic seizure. Neuropsychological assessment demonstrated mild amnesia, visuospatial dysfunction and attention deficits with a slow psychomotor speed. MR brain imaging displayed confluent hyperintensities of periventricular and subcortical white matter. Low levels of arylsulfatase A confirmed the diagnosis. Impaired cortical glucose metabolism especially of the medial temporal and frontal cortices was observed using positron emission tomography and fluor-18-labeled fluorodesoxyglucose. The neuropsychological deficits are related to the location of deficits in glucose metabolism.
Johannsen
P
P
Department of Neurology, Aarhus University Hospital, Aarhus, Denmark. peter@pet.auh.dk
Ehlers
L
L
Hansen
H J
HJ
D001921:Brain / Q000473:pathology; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D019788:Fluorodeoxyglucose F18; D005625:Frontal Lobe / Q000378:metabolism; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000150:complications* / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D009778:Occipital Lobe / Q000378:metabolism; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000209:etiology; D019275:Radiopharmaceuticals; D012392:Rorschach Test; D012720:Severity of Illness Index; D013702:Temporal Lobe / Q000378:metabolism*; D014055:Tomography, Emission-Computed
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D005947:Glucose