Ct Diagnosis of Fahr’s Disease, A Case Report

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Ct Diagnosis of Fahr’s Disease, A Case Report

Journal Title: Imaging Journal of Clinical and Medical Sciences - Year 2018, Vol 5, Issue 1

Abstract

Fahr's disease is a rare inherited or sporadic neurological disorder with a prevalence of <1/1,000,000, with a higher incidence reported among males and a typical age of onset in the 4th–6th 3rd-5th decade of life [1]. Fahr’s disease, or idiopathic striopallido¬dentate calcinosis, or idiopathic basal ganglia calcification is a rare clinical entity characterized by bilateral and symmetrical intracerebral calcifications of the basal ganglia, thalamus, dentate nucleus and centrum semiovale in the absence of any metabolic abnormality including hypoparathyroidism [2,3]. Within the basal ganglia, the globus pallidus is the most frequent site of calcification, but deposits may be present in the putamen, caudate nucleus, and internal capsule, dentate nucleus of the cerebellum, thalamus, and the periventricular white matter [4,5]. Sporadic and family cases have been reported [4,5]. It is usually inherited by an autosomal dominant pattern, although sporadic and autosomal recessive cases had also been reported. In 1930, the calcifications of the basal ganglia named “Fahr’s disease”, after the German neuropathologist Karl Theodor Fahr [6]. The disease was first radiologically defined it by Fritzcher in 1935 [7]. Fahr’s disease should be differen¬tiated from Fahr’s syndrome. The Fahr’s disease is used for primary basal ganglia calcification, and the Fahr syndrome is used for basal ganglia calcifications associated with low serum levels of calcium and phosphorus, which usually occur in hypoparathyroidism [8-10]. Both entities are defined by bilateral intracranial calcification with neuropsychiatric symptoms and extrapyramidal disorder [11].

Authors and Affiliations

A Gligorievski

EP ID EP533914
DOI 10.17352/2455-8702.00003
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