Yamamoto Y, Noto Y, Saito M, Ichizen H, Kida H
Department of Internal Medicine, Kanazawa National Hospital, Japan.
J Int Med Res. 1997 Nov-Dec;25(6):364-8. doi: 10.1177/030006059702500607.
This report describes a 37-year-old man presenting with a gait disturbance due to spastic paraparesis. Physical findings showed typical features of Albright's hereditary osteodystrophy, including short stature, obesity, brachydactyly and dental hypoplasia. He was diagnosed as having pseudohypoparathyroidism type Ia, on the basis of his hypocalcaemia, hyperphosphataemia, increased plasma level of parathyroid hormone (PTH), and the unresponsiveness to exogenous PTH loading of his urinary excretion of both nephrogenous cyclic adenosine monophosphate and phosphate. Magnetic resonance imaging and myelographic computed tomographic scans clearly demonstrated severe compression of the spinal cord at T 9/10 by tumour-like ossifications of the paravertebral ligaments. Neurosurgical decompression therapy was, therefore, performed to alleviate his spastic paraparesis. This was a rare case of pseudohypoparathyroidism complicated with spinal cord compression caused by ectopic ossification of the ligaments.
本报告描述了一名37岁男性，因痉挛性截瘫出现步态障碍。体格检查发现其具有奥尔布赖特遗传性骨营养不良的典型特征，包括身材矮小、肥胖、短指畸形和牙齿发育不全。基于其低钙血症、高磷血症、甲状旁腺激素（PTH）血浆水平升高以及其尿中肾源性环磷酸腺苷和磷酸盐对外源性PTH负荷无反应，他被诊断为Ia型假性甲状旁腺功能减退症。磁共振成像和脊髓造影计算机断层扫描清楚地显示，胸9/10水平的脊髓受到椎旁韧带肿瘤样骨化的严重压迫。因此，进行了神经外科减压治疗以缓解其痉挛性截瘫。这是一例罕见的假性甲状旁腺功能减退症合并韧带异位骨化导致脊髓压迫的病例。
