DOI: https://doi.org/10.36347/sjmcr.2025.v13i05.023

Pages: 869-872

Fahr syndrome (FS) is a rare neurological disorder characterized by bilateral and symmetrical intracerebral calcifications, mainly affecting the basal ganglia, thalamus, and cerebellar nuclei. It is frequently associated with metabolic disturbances, particularly parathyroid dysfunctions such as pseudohypoparathyroidism (PHP). PHP is a rare inherited condition marked by resistance to parathyroid hormone, resulting in hypocalcemia and hyperphosphatemia despite normal or elevated PTH levels. This article reports the case of a 15-year-old adolescent presenting with abnormal movements and confusion, found to have severe hypocalcemia and extensive brain calcifications on imaging. The diagnosis of Fahr syndrome secondary to PHP was established. The patient was treated with intravenous then oral calcium and active vitamin D, leading to clinical and biochemical improvement. This case highlights the diagnostic and therapeutic challenges posed by the rare association of FS and PHP, underlining the importance of early recognition, multidisciplinary management, and regular follow-up to prevent irreversible neurological complications. The article also reviews the pathophysiology, clinical features, and management strategies for FS and PHP, emphasizing the need for awareness of metabolic causes in patients with unexplained neurological symptoms and intracranial calcifications.