BACKGROUND Pseudohypoparathyroidism(PHP) is a heterogeneous group of disorders due to impaired activation of c AMP dependant pathways following binding of parathyroid hormone (PTH) to its receptor. In PHP end organ resistance to PTH results in hypocalcaemia, hyperphosphataemia and high PTH levels. CASE PRESENTATION A 59 year old male presented with a history of progressive impairment of speech and unsteadiness of gait for 1 week and acute onset altered behavior for 1 day and one episode of generalized seizure. His muscle power was grade four according to MRC (medical research council) scale in all limbs and Chovstek's and Trousseau's signs were positive. Urgent non contrast computed tomography scan of the brain revealed extensive bilateral cerebral and cerebellar calcifications. A markedly low ionized calcium level of 0.5 mmol/l, an elevated phosphate level of 9.5 mg/dl (reference range: 2.7-4.5 mg/dl) and an elevated intact PTH of 76.3 pg/l were noted. His renal functions were normal. His hypocalcemia was accentuated by the presence of hypomagnesaemia. His 25 hydroxy vitamin D level was only marginally low which could not account for severe hypocalcaemia. A diagnosis of pseudohypoparathyroidism without phenotypic defects, was made due to hypocalcaemia and increased parathyroid hormone levels with cerebral calcifications. The patient was treated initially with parenteral calcium which was later converted to oral calcium supplements. His coexisting Vitamin D deficiency was corrected with 1αcholecalciferol escalating doses. His hypomagnesaemia was corrected with magnesium sulphate parenteral infusions initially and later with oral preparations. With treatment there was a significant clinical and biochemical response. CONCLUSION Pseudohypoparathyroidism can present for the first time in elderly resulting in extensive cerebral calcifications. Identification and early correction of the deficit will result in both symptomatic and biochemical response.

中文翻译：

---

一名因假性甲状旁腺功能减退而广泛脑钙化的患者：一例报告。

背景假性甲状旁腺功能减退症（PHP）是一组异质性疾病，由于甲状旁腺激素（PTH）与其受体结合后c AMP依赖性途径的激活受损。在PHP中，末端器官对PTH的抗性导致低血钙，高磷血症和高PTH水平。病例介绍一名59岁的男性，有进行性语言障碍和步态不稳1周的历史，急性发作行为改变1天，有1次全身性癫痫发作。根据MRC（医学研究理事会）的评分，他的四肢肌肉力量均为四级，Chovstek和Trousseau的体征均为阳性。紧急的脑部非对比计算机断层扫描显示广泛的双侧脑和小脑钙化。离子钙含量极低，为0.5 mmol / l，观察到磷酸盐水平升高到9.5 mg / dl（参考范围：2.7-4.5 mg / dl），完整PTH升高到76.3 pg / l。他的肾功能正常。低镁血症的存在加剧了他的低钙血症。他的25羟基维生素D水平仅略低，不能解释严重的低钙血症。由于血钙过低和伴有脑钙化的甲状旁腺激素水平升高，诊断为无表型缺陷的假性甲状旁腺功能减退症。该患者最初接受肠胃外钙治疗，后来转化为口服钙补充剂。通过增加1α胆钙化固醇的剂量可以纠正他并存的维生素D缺乏症。他的低镁血症最初用硫酸镁肠胃外输注纠正，后来用口服制剂纠正。经过治疗，出现了显着的临床和生化反应。结论假性甲状旁腺功能减退症可在老年人中首次出现，导致广泛的脑钙化。缺陷的识别和早期纠正将导致症状和生化反应。