Ramos-Lopes Joana, Brás Ana, Morgadinho Ana, Moreira Fradique
Serviço de Neurologia. Centro Hospitalar e Universitário de Coimbra. Coimbra. Portugal.
Acta Med Port. 2019 May 31;32(5):402-406. doi: 10.20344/amp.11026.
Pathological basal ganglia calcification, or Fahr's Syndrome, can be secondary to a variety of diseases, namely parathyroid disturbances. Movement disorders are common clinical features, in which chorea is seen in less than 20% of cases and dystonia just in 8%. We report the clinical case of a 49-year-old male with a history of thyroidectomy, who was admitted in Emergency Service with acute generalized chorea and focal painful feet dystonia. Laboratory analysis showed hypocalcemia and rhabdomyolysis, and computed tomography scan revealed parenchymal calcification with basal ganglia involvement. After complementary studies we established a Fahr's Syndrome diagnosis secondary to an iatrogenic hypoparathyroidism. Clinical management has been successful with stabilized calcium levels, with no more neurologic symptoms. Hypocalcemia should be readily investigated and treated after a thyroidectomy, given the irreversibility of intracerebral calcifications and potential neurological or systemic consequences.
病理性基底节钙化,即法尔氏综合征,可能继发于多种疾病,尤其是甲状旁腺功能紊乱。运动障碍是常见的临床特征,其中不到20%的病例出现舞蹈症,仅8%的病例出现肌张力障碍。我们报告了一例49岁男性的临床病例,该患者有甲状腺切除病史,因急性全身性舞蹈症和足部局灶性疼痛性肌张力障碍入住急诊室。实验室分析显示低钙血症和横纹肌溶解,计算机断层扫描显示实质钙化累及基底节。经过补充检查,我们诊断为医源性甲状旁腺功能减退继发的法尔氏综合征。通过稳定钙水平,临床治疗取得成功,未再出现神经症状。鉴于脑内钙化的不可逆性以及潜在的神经或全身后果,甲状腺切除术后应及时对低钙血症进行检查和治疗。