title: [Presenile dementia in xeroderma pigmentosum].
abstract: Neurological manifestations of xeroderma pigmentosum, a rare autosomal recessive neurocutaneous syndrome, are variable. The association with progressive mental retardation, usually with onset in childhood, is well known. We present a case of x.p. with progressive presenile dementia. This combination has, to our knowledge, not yet been reported in the literature. Although no hints on another aetiology have been found, the coincidental combination of x.p. with M. Alzheimer has to be taken into consideration. CT scan and MRI showed a marked cerebral atrophy.
authors.affiliation: Neurologische Klinik, Universität zu Lübeck.
authors.forename: D
authors.initials: D
authors.lastname: Dorndorf
authors.forename: K
authors.initials: K
authors.lastname: Wessel
authors.forename: P
authors.initials: P
authors.lastname: Vieregge
authors.forename: R
authors.initials: R
authors.lastname: Verleger
authors.forename: D
authors.initials: D
authors.lastname: Kömpf
mesh_terms: D001284:Atrophy; D001921:Brain / Q000473:pathology; D003704:Dementia / Q000175:diagnosis / Q000235:genetics*; D003952:Diagnostic Imaging; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D014983:Xeroderma Pigmentosum / Q000175:diagnosis / Q000235:genetics*
vernacular_title: Präsenile Demenz bei Xeroderma pigmentosum.