多系统萎缩伴抗利尿激素分泌不当综合征1例  

Multiple system atrophy with syndrome of inappropriate antidiuretic hormone secretion:a case report

作　　者：杨素 郑乾 徐子茜 王启超 雷晓阳 尹建红 贺电 YANG Su;ZHENG Qian;XU Ziqian;WANG Qichao;LEI Xiaoyang;YIN Jianhong;HE Dian(Department of Neurology,the affiliated Hospital of Guizhou Medical University,Guiyang 550004,China)

机构地区：[1]贵州医科大学附属医院神经内科,贵阳550004

出　　处：《中国神经精神疾病杂志》2023年第6期367-370,共4页Chinese Journal of Nervous and Mental Diseases

基　　金：科技创新2030-“脑科学与类脑研究”重大项目(编号:2021ZD0201801);贵州省卫生健康委员会科学技术基金(编号:gzwkj2021-018)。

摘　　要：报告1例多系统萎缩(multiple system atrophy,MSA)伴抗利尿激素分泌不当综合征(syndrome of inap‐propriate antidiuretic hormone secretion,SIADHS)患者。患者为54岁男性,因反复晕厥、发热就诊。查体示平卧位转直立位3 min时血压下降超过20 mmHg/10 mmHg,面部表情少,构音欠清,双侧咽反射消失,四肢肌张力增高,双上肢肌张力齿轮样增高,双上肢轮替试验笨拙,双手握拳试验笨拙,双足拍地运动笨拙,双上肢意向性震颤,双侧掌颌反射(+),双侧Babinski征(+)。胸部CT示肺部感染,血、尿炎性指标升高,血钠及血浆渗透压降低、尿钠升高,经抗感染治疗,肺部及泌尿系感染好转后体温未恢复正常。颅脑MRI示脑干、小脑萎缩。诊断MSA合并SIADHS、中枢性高热,予物理降温、降低环境温度、限水补钠后体温及血钠逐渐恢复正常。MSA合并SIADHS、中枢性高热临床罕见,本文分析该病例的诊治过程及复习相关文献,以提高临床医师对MSA非运动症状的认识,减少误诊、漏诊。To report a case of multiple system atrophy(MSA)with syndrome of inappropriate antidiuretic hormone secretion(SIADHS).A 54-year-old man was admitted to hospital due to recurrent syncope and fever.The physical examination showed a blood pressure drop of more than 20/10 mmHg within 3 minutes of standing,stiff facial expressions,dysarthria,absence of pharyngeal reflexes,limb hypermyotonia,cogwheel rigidity of upper limbs,clumsy rapid alternating movements of hands,clumsy finger taps and hand movements,clumsy leg agility,intention tremor,positive palmomental reflex and Babinski sign.At admission,lung computed tomography revealed pulmonary infection foci.Laboratory tests showed elevated levels of inflammatory indexes in blood and urine,decreased levels of blood sodium and osmolarity,with an elevated level of urine sodium.Brain MRI revealed brainstem and cerebellar atrophy.The man was diagnosed with MSA,SIADHS and central hyperthermia.Following treatments with antibacterial drugs,physical cooling,sodium supplements and reduction of water intake,his body temperature and blood sodium gradually returned to normal levels.MSA accompanying SIADHS and central hyperthermia is rare.This article analyzes the course of diagnosis and treatment and reviews the pertinent literature,with an aim to advance our understanding of non-motor symptoms of MSA and reduce the misdiagnosis and missed diagnosis.

关 键 词：多系统萎缩 抗利尿激素分泌不当综合征 低钠血症 中枢性高热 物理降温 诊断 治疗 

分 类 号：R742.5[医药卫生—神经病学与精神病学]