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ISSN 2063-5346
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FAHR SYNDROME SECONDARY TO HYPOPARATHYROIDISM AND PSEUDOHYPO- PARATHYROIDISM

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Violeta Hoxha, Klodiana Poshi, Dorina Ylli, Adela Haxhiraj, Tea Shehu Kolnikaj, Agron Ylli
» doi: 10.31838/ecb/2023.12.1.362

Abstract

Fahr’s Syndrome, also known as striopallidodentate calcinosis is a rare form of neurological disorder characterized by abnormal calcifications in the basal ganglia, cerebellum, and cerebral cortex. Its prevalence goes from 2 to 12.5 %. It can be primary (idiopathic) or secondary (from metabolic disorders especially from hypoparathyroidism and pseudohypoparathyroidism). Cases descriptions: The first patient is a 61 years old woman who came to the Emergency Unit with complaints of body weakness, severe fatigue, left-side numbness, and headache. That's why she got a head injury with an open bleeding wound. A head CT was performed where diffuse cerebral and cerebellar calcifications were noticed. Biochemical analysis demonstrated hypocalcemia due to primary hypoparathyroidism, vitamin D deficiency, and Hashimoto thyroiditis. Immediate treatment with IV calcium (2000mg/day) and Vit D3 (2000UI/day) was started and the patient was advised for further follow-up. The second patient is a 37 years old man who presented in the Emergency Unit with epileptic generalized tonic-clonic seizure. He was known to suffer from epilepsy since 4 months of age but a head CT was performed for the first time where diffuse bilateral brain calcifications were noticed. Biochemical analysis showed hypocalcemia with low/normal Vit D levels and high PTH levels. Diagnose of pseudohypoparathyroidism probably type 1 was made. The patient was treated with IV calcium and Vit D3. On follow-up, levels of serum ionized calcium and 24h calcinuria were improved. Conclusions: Fahr’s Disease is a neurological complication of chronic hypoparathyroidism and pseudohypoparathyroidism. In most cases, the diagnosis is clinical-radiological with calcifications in the basal ganglia. No significant differences are shown between hypoparathyroidism and pseudohypoparathyroidism on CT images. After confirming the diagnosis, the patients should start treatment with cholecalciferol or ergocalciferol (firstly to fill the depots), calcium supplements, and rocatrol.

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