Sehatpour Faezeh, Mohseni Shahrzad, Pejman Sani Mahnaz
Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.
Case Rep Endocrinol. 2025 Jul 15;2025:6355371. doi: 10.1155/crie/6355371. eCollection 2025.
Fahr's syndrome is a rare neurodegenerative condition characterized by bilateral progressive calcification of the basal ganglia and other brain structures. Due to overlapping symptoms, it can be misdiagnosed as other neurological disorders. A 68-year-old man was presented to the emergency department with an exacerbating decline in the level of consciousness and dysarthria over a 20-day period. On admission, the laboratory examinations revealed a low level of calcium and parathyroid hormone. Brain imaging findings showed bilateral calcifications in the basal ganglia, pulvinar region of the thalami, and dentate nuclei. In addition, a prolonged QTc interval on his electrocardiogram (ECG) indicated hypocalcemia. After receiving calcium gluconate 10%, the calcium level and QTc interval stabilized, and the patient's level of consciousness gradually improved. Fahr's syndrome due to hypoparathyroidism should be suspected in any patient with neurological symptoms and hypocalcemia. Hence, early identification and management of hypoparathyroidism can prevent progression of calcification and improve patients' quality of life and prognosis.
法尔氏综合征是一种罕见的神经退行性疾病,其特征是基底神经节和其他脑结构出现双侧进行性钙化。由于症状重叠,它可能被误诊为其他神经系统疾病。一名68岁男性在20天内意识水平恶化并出现构音障碍,被送往急诊科。入院时,实验室检查显示钙和甲状旁腺激素水平较低。脑部影像学检查结果显示基底神经节、丘脑枕叶区域和齿状核有双侧钙化。此外,他的心电图(ECG)显示QTc间期延长,提示低钙血症。静脉注射10%葡萄糖酸钙后,血钙水平和QTc间期稳定,患者的意识水平逐渐改善。任何有神经症状和低钙血症的患者都应怀疑为甲状旁腺功能减退所致的法尔氏综合征。因此,早期识别和治疗甲状旁腺功能减退可以预防钙化进展,提高患者的生活质量和预后。