[en] A case of early-onset adult dementia with family history of dementia is reported, characterised by neuropsychological deficits, suggesting frontal involvement, with mild non specific white matter abnormalities on CT scan. Familial Alzheimer's disease was suspected but the neuropathological diagnosis on brain biopsy was metachromatic leukodystrophy. 18FDG-PET revealed a very peculiar pattern of metabolic impairment in thalamic areas, in medial and frontopolar regions, and in occipital lobes. Neuropsychological follow-up showed relatively stable difficulties of long-term memory and signs of frontal lobe dysfunction, similar to those observed in subcortical dementias. MRI subsequently showed periventricular leukoencephalopathy. The brain metabolic pattern observed in that case of metachromatic leukodystrophy was quite different from that reported in other types of dementia.
Disciplines :
Neurology
Author, co-author :
Salmon, Eric ; Université de Liège - ULiège > Centre de Recherches du Cyclotron - Département des sciences cliniques > Neuroimagerie des troubles de la mémoire et révalid. cogn.
Van der Linden, Martial ; Université de Liège - ULiège > Département des sciences cognitives > Psychopathologie cognitive
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