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

Abstract

Introduction and Importance: 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.