Marchini C, Vicario L, Zappoli Thyrion E, Marinig L
Riv Patol Nerv Ment. 1979 Mar-Apr;100(2):69-78.
A case of idiopathic unilateral (left) idiopatic basal ganglia calcification with associated extrapiramidal controlateral symptoms is described. The patient, a 20 years old man, showed since childhood a progressive motor impairment of his right limbs more obvious in his arms. Neurological examination showed hypertonia, distonia, motor and other extrapiramidal impairments of his right limbs. Skull X Ray and CT scan revealed left basal ganglia calcification. Laboratory investigations excluded all the common diseases with basal ganglia calcifications: tumors, tiroid and paratiroid disorders, parassites, vascular, inflammatory or degenerative diseases. Psycodiagnostic tests did not reveal relevant abnormalities. We cannot say with certainty that this is a sporadic case as we were unable to examine the whole family. Slight improvement of symptoms was obtained using orfenadrine and diazepam.
本文描述了一例特发性单侧(左侧)基底节钙化伴对侧锥体外系症状的病例。患者为一名20岁男性,自幼右侧肢体出现进行性运动障碍,上肢更为明显。神经系统检查显示右侧肢体肌张力增高、肌张力障碍、运动及其他锥体外系损害。头颅X线和CT扫描显示左侧基底节钙化。实验室检查排除了所有伴有基底节钙化的常见疾病:肿瘤、甲状腺和甲状旁腺疾病、寄生虫、血管性、炎症性或退行性疾病。心理诊断测试未发现相关异常。由于无法对整个家族进行检查,我们不能确定这是一例散发病例。使用奥芬那君和地西泮后症状略有改善。