Chow K S, Lu D N
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1989 Mar-Apr;30(2):129-33.
A 3 years old boy was admitted due to recurrent attacks of tetany and carpopedal spasm since one and a half years of age. The tetany lasting for 1-2 minutes in each episode was often preceded by an upper respiratory tract infection and occurred 2-3 times a month. Both birth and family history were unremarkable. Physical findings showed mild psychomotor retardation with positive Chvostek sign. Laboratory examination revealed hypocalcemia, hyperphosphatemia, and low serum parathyroid hormone level. EEG showed abnormal tracing with increased slow waves. Head CT Scan demonstrated symmetrical calcification in the basal ganglia region. The clinical features and laboratory findings were consistent with hypoparathyroidism. The mechanism of calcium deposit in the basal ganglia still remains unclear. Tetany, muscle cramping and seizures secondary to hypocalcemia are the most common neurologic signs which respond quickly to calcium replacement. Subsequent supplemental therapy resolved movement disorders and mental retardation. If early treatment prior to the tetanic episodes is instituted in a patient with hypoparathyroidism, it may prevent the development of complications such as intracranial calcifications, cataract and permanent retardation.
一名3岁男孩自1岁半起因反复出现手足搐搦和手足痉挛发作而入院。每次发作持续1 - 2分钟的手足搐搦常在上呼吸道感染后出现,每月发作2 - 3次。出生史和家族史均无异常。体格检查发现轻度精神运动发育迟缓,Chvostek征阳性。实验室检查显示低钙血症、高磷血症和血清甲状旁腺激素水平降低。脑电图显示异常波形,慢波增多。头部CT扫描显示基底节区对称性钙化。临床特征和实验室检查结果与甲状旁腺功能减退相符。基底节区钙沉积的机制仍不清楚。低钙血症继发的手足搐搦、肌肉痉挛和癫痫是最常见的神经症状,补钙后反应迅速。随后的补充治疗解决了运动障碍和智力发育迟缓问题。如果对甲状旁腺功能减退患者在手足搐搦发作前进行早期治疗,可能会预防颅内钙化、白内障和永久性发育迟缓等并发症的发生。