Kartin P, Zupevc M, Pogacnik T, Cerk M
J Neurol. 1982;227(3):171-6. doi: 10.1007/BF00313572.
A patient is described who 32 years after thyroidectomy developed a chronic progressive syndrome due to hypoparathyroidism, with multiple extrapyramidal signs (faciobuccolingual dyskinesias, choreiform and athetotic movements of upper extremities, tremor of hands, cogwheel phenomenon), cerebellar manifestations (dysarthria, dysgraphia, mild gait ataxia), and pyramidal signs as well as an organic psychosis and epilepsy. A CT scan showed calcification of the basal ganglia. Therapy for hypoparathyroidism produced partial regression of the disorder. To explain the physiopathologic mechanism of the syndrome, Selye et al. advanced an attractive hypothesis of calciphylaxis, which assumes that a sensitizer and a challenger together induce development of calcification. In the case reported calcification involved the periventricular region and was not limited to the basal ganglia. It is suggested that either a sensitizer or a challenger may enter the periventricular tissue via the ependyma.
本文描述了一名患者，在甲状腺切除术后32年因甲状旁腺功能减退出现慢性进行性综合征，伴有多种锥体外系体征（面颊舌运动障碍、上肢舞蹈样和手足徐动样动作、手部震颤、齿轮现象）、小脑表现（构音障碍、书写障碍、轻度步态共济失调）、锥体束征以及器质性精神病和癫痫。CT扫描显示基底节钙化。甲状旁腺功能减退的治疗使病情部分缓解。为了解释该综合征的病理生理机制，塞利等人提出了一个有吸引力的钙质沉着假说，该假说认为致敏剂和激发剂共同诱导钙化的发展。在报道的病例中，钙化累及脑室周围区域，并不局限于基底节。提示致敏剂或激发剂可能通过室管膜进入脑室周围组织。
