Clinical Spectrum of FAHR Syndrome: Report of two cases
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Basal Ganglia Diseases

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Pinzon Tovar, A., Mendez, H., Jimenez Salazar, S., Oviedo Cali, M., & Buitrago-Toro, K. . (2022). Clinical Spectrum of FAHR Syndrome: Report of two cases. Revista Colombiana De Endocrinología, Diabetes &Amp; Metabolismo, 9(3).


Background: Calcifications of the basal ganglia have a wide spectrum of clinical presentation. Neuro-psychiatric manifestations associated with calcifications of primary etiology it is known as Fahr's disease, while if they are secondary to different metabolic disorders, infections, autoimmune diseases or intoxications, it is called Fahr syndrome.

Purpose: To present the diagnostic approach and different clinical presentations of Fahr's syndrome and disease.

Presentation of the cases: We present two clinical cases as part of the illustration of the diagnostic process. A 38-year-old patient with epilepsy and cognitive deficit in whom Fahr's syndrome secondary to hypoparathyroidism is diagnosed and the case of a 28-year-old patient with no relevant history, in whom, after ruling out multiple causes, a diagnosis of Idiopathic Fahr's Disease is considered. Fahr's syndrome is much more frequent than Fahr's disease.

Discussion and conclusion: In the diagnostic approach to calcifications of the basal ganglia, secondary etiologies should be initially studied; if they are ruled out, a primary etiology should be investigated.
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