原發性副甲狀腺機能低下症合併基底神經節鈣化：一病例報告

Primary Hypoparathyroidism with Basal Ganglia Calcification: Report of a Case

周國燊(Kuo-Sun Chow) ； 呂道南(Do-Nan Lu)

《Acta Paediatrica Sinica》 30卷2期 (1989/04) Pp. 129-133

摘要

一3歲大男童被診斷爲原發性副甲狀腺機能低下症。其臨床上每月固定有2-3次的強直性痙攣(tetany)及心智發育遲緩，血清學檢查有低血鈣、高血磷及副甲狀腺荷爾蒙降低，同時電腦斷層掃描顯示病童合併有兩側基底神經節鈣化。故提出來報告並加以討論。

關鍵字

idiopathic hypoparathyroidism ； basal ganglia calcification

並列摘要

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, catarract and permanent retardation.