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Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report

Fahr’s syndrome, also known as bilateral striatopallidodentate calcinosis is a rare neurological disorder that is characterized by abnormal deposition of calcium in the basal ganglia, cerebellar dentate nuclei, and cerebral cortical white matter. The authors report an extremely uncommon case of Fahr...

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Autores principales: katwal, Shailendra, Bhandari, Sushmita, Ghimire, Aastha, Ghimire, Prasoon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10406094/
https://www.ncbi.nlm.nih.gov/pubmed/37554906
http://dx.doi.org/10.1097/MS9.0000000000001032
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author katwal, Shailendra
Bhandari, Sushmita
Ghimire, Aastha
Ghimire, Prasoon
author_facet katwal, Shailendra
Bhandari, Sushmita
Ghimire, Aastha
Ghimire, Prasoon
author_sort katwal, Shailendra
collection PubMed
description Fahr’s syndrome, also known as bilateral striatopallidodentate calcinosis is a rare neurological disorder that is characterized by abnormal deposition of calcium in the basal ganglia, cerebellar dentate nuclei, and cerebral cortical white matter. The authors report an extremely uncommon case of Fahr’s syndrome with thrombocytopenia, hypoparathyroidism, and seizure. CASE PRESENTATION: A 32-year-old male was brought with a repeated history of twitching of hands, tingling sensation, and uncontrolled seizure despite medications. CLINICAL DISCUSSION: Computed tomography findings showed bilateral basal ganglia, cerebellar dentate nuclei, and subcortical cerebral white matter calcifications. Laboratory studies revealed reduced levels of calcium, parathyroid hormone, and thrombocyte count. Based on these investigations Fahr’s syndrome probably due to hypoparathyroidism with thrombocytopenia was diagnosed. The patient was initially treated with intravenous calcium gluconate and platelet transfusion followed by oral calcium supplementation. CONCLUSION: Fahr’s syndrome due to hypoparathyroidism should be suspected in any patient with neurological symptoms and hypocalcemia. Seizures in the patient of Fahr’s syndrome with thrombocytopenia could be very detrimental due to the risk of intracranial hemorrhage. Hence, treatment should be started as early as possible.
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spelling pubmed-104060942023-08-08 Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report katwal, Shailendra Bhandari, Sushmita Ghimire, Aastha Ghimire, Prasoon Ann Med Surg (Lond) Case Reports Fahr’s syndrome, also known as bilateral striatopallidodentate calcinosis is a rare neurological disorder that is characterized by abnormal deposition of calcium in the basal ganglia, cerebellar dentate nuclei, and cerebral cortical white matter. The authors report an extremely uncommon case of Fahr’s syndrome with thrombocytopenia, hypoparathyroidism, and seizure. CASE PRESENTATION: A 32-year-old male was brought with a repeated history of twitching of hands, tingling sensation, and uncontrolled seizure despite medications. CLINICAL DISCUSSION: Computed tomography findings showed bilateral basal ganglia, cerebellar dentate nuclei, and subcortical cerebral white matter calcifications. Laboratory studies revealed reduced levels of calcium, parathyroid hormone, and thrombocyte count. Based on these investigations Fahr’s syndrome probably due to hypoparathyroidism with thrombocytopenia was diagnosed. The patient was initially treated with intravenous calcium gluconate and platelet transfusion followed by oral calcium supplementation. CONCLUSION: Fahr’s syndrome due to hypoparathyroidism should be suspected in any patient with neurological symptoms and hypocalcemia. Seizures in the patient of Fahr’s syndrome with thrombocytopenia could be very detrimental due to the risk of intracranial hemorrhage. Hence, treatment should be started as early as possible. Lippincott Williams & Wilkins 2023-06-28 /pmc/articles/PMC10406094/ /pubmed/37554906 http://dx.doi.org/10.1097/MS9.0000000000001032 Text en Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by-nd/4.0/This is an open access article distributed under the Creative Commons Attribution-NoDerivatives License 4.0 (https://creativecommons.org/licenses/by-nd/4.0/) , which allows for redistribution, commercial and non-commercial, as long as it is passed along unchanged and in whole, with credit to the author. http://creativecommons.org/licenses/by-nd/4.0/ (https://creativecommons.org/licenses/by-nd/4.0/)
spellingShingle Case Reports
katwal, Shailendra
Bhandari, Sushmita
Ghimire, Aastha
Ghimire, Prasoon
Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
title Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
title_full Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
title_fullStr Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
title_full_unstemmed Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
title_short Fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
title_sort fahr’s syndrome with hypoparathyroidism, thrombocytopenia, and seizure: a rare case report
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10406094/
https://www.ncbi.nlm.nih.gov/pubmed/37554906
http://dx.doi.org/10.1097/MS9.0000000000001032
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