title: Subacute dementia and imaging correlates in a case of Fahr's disease.
abstract: We report a case of idiopathic bilateral basal ganglia calcinosis, or Fahr's disease (FD) in a 50 year old patient who developed rapidly progressive behavioural abnormalities and severe neuropsychological impairments, but no movement disorder. Neuropsychological deficits included a severe dysexecutive syndrome, anterograde amnesia, and attentional impairment. Neuropsychiatric features comprised apathy with intermittent disinhibition, anxiety, irritability, frequent mood changes, ritualistic and antisocial behaviour, and psychosis. Fluorodeoxyglucose positron emission tomography showed a massive reduction of glucose metabolism in the basal ganglia and the frontal brain. The observed abnormalities possibly result from a disruption of frontostriatal circuits, presumably at the basal ganglia level. This case indicates that FD may cause exclusively behavioural alterations and that the associated hypometabolism in certain frontal areas is closely related to the clinical picture.
authors.affiliation: University Clinic of Neurology, Innsbruck, Austria. thomas.benke@uibk.ac.at
authors.forename: T
authors.initials: T
authors.lastname: Benke
authors.forename: E
authors.initials: E
authors.lastname: Karner
authors.forename: K
authors.initials: K
authors.lastname: Seppi
authors.forename: M
authors.initials: M
authors.lastname: Delazer
authors.forename: J
authors.initials: J
authors.lastname: Marksteiner
authors.forename: E
authors.initials: E
authors.lastname: Donnemiller
mesh_terms: D000374:Aggression; D001007:Anxiety; D001480:Basal Ganglia Diseases / Q000150:complications* / Q000473:pathology; D002114:Calcinosis / Q000150:complications* / Q000473:pathology; D003072:Cognition Disorders / Q000209:etiology; D003704:Dementia / Q000209:etiology*; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008297:Male; D008875:Middle Aged; D019275:Radiopharmaceuticals; D012919:Social Behavior; D013577:Syndrome; D014055:Tomography, Emission-Computed
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18