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[甲状旁腺功能减退症的神经学表现:诊断困难。病例报告]

[Neurological manifestations of hypoparathyroidism: diagnostic difficulties. Case report].

作者信息

Nuzhnyi E P, Antonova K V, Tanashyan M M, Illarioshkin S N

机构信息

Research Center of Neurology.

出版信息

Ter Arkh. 2023 Nov 23;95(10):864-869. doi: 10.26442/00403660.2023.10.202429.

Abstract

Hypoparathyroidism is a rare condition characterized by reduced production of parathyroid hormone or tissue resistance which leads to hypocalcemia and hyperphosphatemia. Neurological manifestations often occur as the first symptoms of hypoparathyroidism and are characterized by a wide variety of symptoms of both the central and peripheral nervous systems dysfunction, which requires a differential diagnosis with a wide range of neurological diseases. Two clinical cases illustrating the features of subacute and chronic hypoparathyroidism are presented. In the case of subacute hypoparathyroidism, a young woman presented with severe tetany involving the oculomotor muscles (paroxysmal strabismus), laryngeal muscles (respiratory stridor), body muscles (opisthotonus, «obstetrician's hand») and the development of secondary myopathy. In another case with a long-term chronic course of postoperative hypoparathyroidism, the patient's adaptation to severe hypocalcemia was noted; the clinical features were dominated by cerebral syndromes due to brain structures calcification (Fahr's syndrome). Possible reasons for late diagnosis of hypoparathyroidism, the importance of active detection of symptoms of neuromuscular hyperexcitability and laboratory testing of phosphorus and calcium metabolism are discussed.

摘要

甲状旁腺功能减退是一种罕见疾病,其特征为甲状旁腺激素分泌减少或组织抵抗,进而导致低钙血症和高磷血症。神经学表现常作为甲状旁腺功能减退的首发症状出现,其特征为中枢和外周神经系统功能障碍的多种症状,这需要与多种神经疾病进行鉴别诊断。本文介绍了两例说明亚急性和慢性甲状旁腺功能减退特征的临床病例。在亚急性甲状旁腺功能减退病例中,一名年轻女性出现严重手足搐搦,累及动眼肌(阵发性斜视)、喉肌(呼吸性喘鸣)、身体肌肉(角弓反张、“助产士手”)并继发肌病。在另一例术后甲状旁腺功能减退长期慢性病程的病例中,注意到患者对严重低钙血症的适应情况;临床特征以脑结构钙化导致的脑综合征(法尔综合征)为主。文中讨论了甲状旁腺功能减退诊断延迟的可能原因、积极检测神经肌肉兴奋性增高症状以及磷和钙代谢实验室检测的重要性。

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