Fahr’s syndrome presenting with pure and progressive presenile dementia |
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Authors: | P J Modrego J Mojonero M Serrano N Fayed |
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Institution: | 1. Av.da de la Ilustración 12, C34, ES-50012, Zaragoza, Spain 2. Neurology Unit, Hospital de Alca?iz, ES-44600, Alca?iz, Spain 3. Magnetic Resonance Unit, Clinica Quirón, Zaragoza, Spain
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Abstract: | 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. |
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Keywords: | Presenile dementia Fahr’ s syndrome |
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