title: Nasu-Hakola disease as suspected cause for bone disease and dementia.
abstract: Progressive dementia in conjunction with multiple bone fractures in a previously healthy young man led to the investigation of the underlying cause. The differential diagnoses (most importantly hypoparathyroidism) were limited given basal ganglia calcifications on the brain computed tomographic scan. Electronic search of the key words basal ganglia calcification, osteoporosis, and dementia revealed a rare condition termed Nasu-Hakola disease or polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy. This very rare and potentially fatal genetic disease is characterized by pathological fractures, multiple lytic bone lesions, and presenile dementia. We report an Iranian patient with this disease and a review of the literature.
authors.affiliation: From the *Rheumatic Diseases Research Center (RDRC), School of Medicine, †Radiology Department, Ghaem Hospital, School of Medicine, and ‡Avicenna Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Islamic Republic of Iran.
authors.forename: Maryam
authors.initials: M
authors.lastname: Sahebari
authors.forename: Bita
authors.initials: B
authors.lastname: Abbasi
authors.forename: Ali
authors.initials: A
authors.lastname: Akhondpour Manteghi
authors.forename: Nafiseh
authors.initials: N
authors.lastname: Abdollahi
mesh_terms: D015502:Absorptiometry, Photon; D000328:Adult; D015519:Bone Density / Q000502:physiology; D001847:Bone Diseases / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000209:etiology*; D001921:Brain / Q000000981:diagnostic imaging; D003704:Dementia / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007492:Iran; D008060:Lipodystrophy / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D010009:Osteochondrodysplasias / Q000150:complications* / Q000175:diagnosis*; D013344:Subacute Sclerosing Panencephalitis / Q000150:complications* / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed