Nasu-Hakola syndrome: polycystic lipomembranous osteodysplasia with sclerosing leucoencephalopathy and presenile dementia.

A Verloes, P Maquet, B Sadzot, M Vivario… - Journal of medical …, 1997 - ncbi.nlm.nih.gov
A Verloes, P Maquet, B Sadzot, M Vivario, A Thiry, G Franck
Journal of medical genetics, 1997ncbi.nlm.nih.gov
Presenile dementia of genetic origin is often attributed to Alzheimer's disease. This disorder
remains by far the commonest cause of early dementia. Pick disease and prion protein
disorders are usually discussed in the differen-tial diagnosis. We review here Nasu-Hakola
syndrome (OMIM 221770), another unusual cause of presenile intellectual deterioration, in
which neurological impairment occurs to-gether with impressive destructive changes of the
skeleton.
Presenile dementia of genetic origin is often attributed to Alzheimer's disease. This disorder remains by far the commonest cause of early dementia. Pick disease and prion protein disorders are usually discussed in the differen-tial diagnosis. We review here Nasu-Hakola syndrome (OMIM 221770), another unusual cause of presenile intellectual deterioration, in which neurological impairment occurs to-gether with impressive destructive changes of the skeleton.
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