Beneki Eirini, Papazarkadas Xenofon, Karras Achillefs, Kalantzis Konstantinos, Tsatiris Konstantinos
Drs. Beneki and Tsatiris are with the Department of Cardiology at Karditsa General Hospital in Karditsa, Greece.
Dr. Papazarkadas is with the Department of General Surgery at the Hospital of Valais (CHVR) in Sion, Switzerland.
Innov Clin Neurosci. 2021 Apr-Jun;18(4-6):11-14.
Fahr's syndrome, also known as basal ganglia calcification, is a rare neurodegenerative disorder characterized by radiological findings of symmetrical and bilateral idiopathic abnormal deposits of calcium in areas of the brain that control motor activity, including the basal ganglia and the cerebral cortex. There is neither a specific cure, nor a standard treatment for Fahr's syndrome and treatment is primarily symptomatic. Brain imaging has gained widespread use in order to support clinicians in diagnosing intracranial calcifications.
We present a case of an 83-year-old female patient who presented with symptoms of confusion, fever, nausea, and vomiting. Clinical diagnosis of Fahr's syndrome secondary to hypopathyroidism was based on the neuropsychiatric signs and symptoms, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved following replacement therapy with calcium gluconate, followed by oral supplemental calcitriol.
This case highlights the importance of considering organic causes when patients present with neuropsychiatric disturbances, especially following thyroidectomy.
法尔氏综合征,又称基底节钙化,是一种罕见的神经退行性疾病,其特征是在控制运动活动的脑区,包括基底节和大脑皮层,出现对称性和双侧性特发性钙异常沉积的影像学表现。法尔氏综合征既没有特定的治愈方法,也没有标准的治疗方案,治疗主要是对症治疗。脑成像已广泛用于辅助临床医生诊断颅内钙化。
我们报告一例83岁女性患者,出现意识模糊、发热、恶心和呕吐症状。继发于甲状旁腺功能减退的法尔氏综合征的临床诊断基于神经精神症状、甲状旁腺功能减退的实验室证据以及基底节钙化的影像学表现。患者在接受葡萄糖酸钙替代治疗,随后口服补充骨化三醇后病情好转。
该病例强调了在患者出现神经精神障碍时,尤其是在甲状腺切除术后,考虑器质性病因的重要性。