2019
DOI: 10.7759/cureus.4797
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Bilateral Basal Ganglia Calcification: Fahr's Disease

Abstract: Fahr’s disease/syndrome is a condition defined as bilateral striato-pallido-dentate calcinosis, a neurodegenerative disease with radiological findings of symmetrical and bilateral idiopathic calcifications of the cerebellum, periventricular white matter, and basal ganglia. Clinical correlation with radiological and a calcium metabolism panel is crucial in differentiating between Fahr’s disease and Fahr’s syndrome. We describe a case that presented with the clinical feature of a cerebrovascular accident and had… Show more

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Cited by 11 publications
(10 citation statements)
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“…Those that occur acutely can compromise the nervous and muscular systems, causing symptoms such as seizures, cataracts, and altered mental status, such as those presented in this case. In contrast, those in the chronic form may initiate a metabolic cascade that results in ectopic calcifications and ectopic calcium deposits in the brain, which may cause diverse neurological problems [1][2][3].…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Those that occur acutely can compromise the nervous and muscular systems, causing symptoms such as seizures, cataracts, and altered mental status, such as those presented in this case. In contrast, those in the chronic form may initiate a metabolic cascade that results in ectopic calcifications and ectopic calcium deposits in the brain, which may cause diverse neurological problems [1][2][3].…”
Section: Discussionmentioning
confidence: 99%
“…Those present in an acute form may compromise the nervous and muscular systems. In contrast, those with a chronic condition may initiate a metabolic cascade that results in ectopic calcifications and ectopic calcium deposits in the brain, which may cause diverse neurological problems [1][2][3].…”
Section: Introductionmentioning
confidence: 99%
“…Baseline biochemical investigations may be done to rule out other possible diagnoses. Molecular genetic testing can be done if there is a strong history of autosomal dominant inheritance [11].…”
Section: Discussionmentioning
confidence: 99%
“…A computed tomography (CT) scan is a method of choice to diagnose calcifications in the dentate nuclei, corona radiata, thalami, and lentiform nuclei, while the non-calcified inflammatory processes are detected by magnetic resonance imaging (MRI) in the setting of Fahr’s disease [ 11 ]. In addition, the assessment of other associated conditions, including lipoid proteinosis, mitochondrial myopathy, tuberculous sclerosis, neuroferritinopathy, brucellosis infection, hyperparathyroidism, pseudo-hypothyroidism, secondary hypoparathyroidism, and idiopathic hypoparathyroidism is paramount to ruling out Fahr’s disease [ 12 ]. The treatment algorithms for Fahr’s disease aim to manage neuropsychiatric symptoms and their underlying etiologies [ 13 ].…”
Section: Introductionmentioning
confidence: 99%