Fahr’s syndrome presenting with pure and progressive presenile dementia.

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Bibliographic Details
Title: Fahr’s syndrome presenting with pure and progressive presenile dementia.
Authors: Modrego, P., Mojonero, J., Serrano, M., Fayed, N.
Source: Neurological Sciences. Dec2005, Vol. 26 Issue 5, p367-369. 3p. 2 Black and White Photographs.
Subjects: Lewy body dementia, Dementia, Progressive supranuclear palsy, Cognition disorders, Eye movement disorders, Paralysis, Parkinson's disease
Abstract: Fahr’s syndrome involves calcification of basal ganglia and dentate nuclei of the cerebellum. Clinically it may present with an array of movement disorders, dementia and other behavioural disturbances. Sporadic and familial cases have been reported with or without calcium/phosphorus metabolism. A rare form of frontotemporal dementia with neurofibrillary tangles and Fahr-type calcifications (DNTC) has been observed mainly in Japan. We report the singular case of a 50-year-old woman with progressive dementia but neither extrapyramidal symptoms nor a metabolic disorder. Brain CT showed Fahr-type calcifications in the basal ganglia, cerebellum and centrum semiovale as well as temporal atrophy; MRI showed diffuse atrophy predominantly in parietotemporal regions. The clinical and radiological features of our patient point to this uncommon form of dementia. [ABSTRACT FROM AUTHOR]
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Database: Psychology and Behavioral Sciences Collection
Description
Abstract:Fahr’s syndrome involves calcification of basal ganglia and dentate nuclei of the cerebellum. Clinically it may present with an array of movement disorders, dementia and other behavioural disturbances. Sporadic and familial cases have been reported with or without calcium/phosphorus metabolism. A rare form of frontotemporal dementia with neurofibrillary tangles and Fahr-type calcifications (DNTC) has been observed mainly in Japan. We report the singular case of a 50-year-old woman with progressive dementia but neither extrapyramidal symptoms nor a metabolic disorder. Brain CT showed Fahr-type calcifications in the basal ganglia, cerebellum and centrum semiovale as well as temporal atrophy; MRI showed diffuse atrophy predominantly in parietotemporal regions. The clinical and radiological features of our patient point to this uncommon form of dementia. [ABSTRACT FROM AUTHOR]
ISSN:15901874
DOI:10.1007/s10072-005-0493-7