CAJ | 학술논문

目的探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.
목적 탐토삼투성탈수초종합정적림상화신경영상특점.방법 대4례삼투성탈수초종합정환자적림상연변과정、CSF、두로CT화MRI、EEG동태변화특점、치료급예후진행분석.결과 4례환자균존재저납혈증,규정후출현정신의식개변、구음화탄인곤난、사지탄탄、기장력장애등증상,림상과정유쌍상성.EEG출현일과성적중도이상.두로CT급CSF균미견이상.MRI특정성영상만우림상표현10 d이후출현,4례환자수차MRI균위음성,7～13 d후복사재현시병조.MRI시4례환자균존재뇌교외수초용해증병조,T1WI가권저신호,T2WI가권고신호,대칭성지루급쌍측미상핵、두상핵、구뇌、뇌도협피질、해마두부등부위,기중3례동시존재뇌교중앙수초용해증개변,정뇌교기저부위대칭성T1저、T2고신호적접형병조;Flair가권이상신호경청초.3례유호전혹전유,기중1례유류명현기장력장애.결론 삼투성탈수초종합정여만성저납혈증유관,합병저혈갑、저혈록시가능경역발생.치료시응진량피면과쾌규정,림상병정구유쌍상성.MRI적특정성개변출현교지,복사MRI시비상필요적.
Objective To investigate the clinical features and neuroimaging features of patients with osmotic demyelination syndrome (ODS).Methods The clinical features and examination results ,including the clinical manifestations,the data of cranial MRI/CT,changes of EEG,treatment and prognosis,were analyzed in 4 patients with ODS.Results All the 4 patients had the history of hyponatraemia.The common clinical manifestations included psychiatric disorder,altered consciousness,dysphasia,dysphagia,quadriplegia and dystonia.Severe transient abnormal EEG was found in these patients,and all the brain CT scanning and CSF were negative.MRI features could only be noted 10 d after the appearing of clinical manifestations and all the first time MRI was negative in these 4 patients.Four patients were diagnosed as having extrapontine myelinolysis,showing symmetrical low T1-weighted signal and high T2-weighted signal within the pons,the basal ganglia,the thalami,the insular cortex and the hippocampal head.Three patients were also diagnosed as having central pontine myelinolysis,showing symmetrical T1 low signal and T2 high signal in the basilar part of pons; much clear imaging could be noted with the help of weighing the abnormal signals.Three patients got improvement with 1 having dystonia sequel.Conclusion ODS is correlated with chronic hyponatraemia,and both hypokalaemia and hypochloremia may be the 2 possible triggers; when they appear,quick correction is not needed.MRI features may be significantly delayed,thus,repeated imaging study is necessary.