渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

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.     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

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.     

渗透性脱髓鞘综合征的临床分析和影像学特点（附1例报道）

目的探讨渗透性脱髓鞘综合征的临床及神经影像学特点。方法分析1例渗透性脱髓鞘综合征患者的临床特点,包括起病前诱因、临床表现、头颅MRI特点、治疗及预后情况。结果患者有长期大量饮酒史,以意识改变、四肢瘫痪、肌张力障碍等为临床表现,血钠及CSF正常。头颅MRI表现为右侧额叶、颞叶、皮质、双侧枕叶、双侧基底节、丘脑、脑干及小脑多发病灶,T1WI加权低信号、T2WI加权高信号,头颅MRI增强扫描可见明显强化。预后差,呈持续浅昏迷状态。结论渗透性脱髓鞘综合征与长期大量饮酒有关,可以没有低钠血症,临床具有双相病程。头颅MRI显示病变可累及皮质,增强扫描可见明显强化。     

以脑梗死为首发症状的糖尿病肾病合并无症状性渗透性脱髓鞘综合征一例

目的探讨渗透性脱髓鞘综合征的发病机制、临床表现及MRI特点。方法分析1例以脑梗死为首发症状的糖尿病肾病合并无症状性渗透性脱髓鞘综合征患者的临床资料,并结合文献进行分析。结果该患者存在高血糖、高血钾、高尿素氮、高肌酐,长期处于慢性高渗状态。头颅MRI显示脑桥基底部对称长T1长T2信号的蝶形病灶,但患者无脑桥中央髓鞘溶解症的临床表现。经治疗2w复查头颅MRI,脑桥蝶形病变仍清晰,但头颅DWI示其高信号明显变淡。结论长期高渗状态可导致慢性脑桥渗透性脱髓鞘,此时可不出现脑桥中央髓鞘溶解的临床表现,虽经积极治疗,也仅可改善细胞毒性水肿。     

脑桥中央和脑桥外髓鞘溶解症的临床分析和影像特点

目的:探讨脑桥中央和脑桥外髓鞘溶解症的临床及神经影像特点。方法:分析3例脑桥中央髓鞘溶解症和1例脑桥外髓鞘溶解症患者的临床特点,包括起病前诱因、临床表现、头颅MRI特点、治疗及预后情况。结果:4例患者均有慢性形成低钠血症后被快速纠正的病史,以意识改变、构音和吞咽困难、四肢瘫痪等为临床表现。3例脑桥中央髓鞘溶解症的MRI表现为脑桥部位对称性的T1加权低信号灶、T2加权高信号灶,呈环状分布;1例脑桥外髓鞘溶解症者在基底节区域有对称性的T1加权低信号、T2加权高信号病灶。4例患者总体预后良好。结论:提高髓鞘溶解症的认识对于本病的防治非常重要,缓慢纠正慢性形成的低钠血症是预防的关键。     

渗透性脱髓鞘综合征

渗透性脱髓鞘综合征是一种继发性脱髓鞘性疾病,病因包括慢性酒精中毒、低钠血症的过快纠正、肝移植等,可能与髓鞘中毒有关。此病以对称性脑桥中央及脑桥外脱髓鞘为病理特征,以假性延髓麻痹、闭锁综合征、缄默症或运动障碍、肌张力障碍、帕金森综合征为临床表现,结合病史及颅脑MRI所见对称性脑桥中央或脑桥外长T1、长T2和DWI高信号改变可以诊断。目前尚无有效的治疗方法,通过积极的综合疗法,预后较以往明显改善,但仍有一些不明之处需要研究证实。     

脑桥中央合并脑桥外髓鞘溶解症一例

脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)是原因不明的以脑桥基底部对称性脱髓鞘为病理特征的综合征,常发生在快速纠正低钠血症之后.病灶若出现在脑内的其他部位,如双侧豆状核、尾状核、丘脑和皮层下白质等,则称为脑桥外髓鞘溶解症(extrapontine myelinolysis,EPM),其临床相对少见.现将收集的一例CPM合并EPM报道如下.     

渗透性脱髓鞘综合征

渗透性脱髓鞘综合征是由于慢性低钠血症患者,予以迅速补钠,改变低渗状态,造成有毒损害。渗透性脱髓鞘综合征的临床表现有脑桥中央髓鞘溶解症、脑桥外髓鞘溶解症。本文复习了渗透性脱髓鞘综合征的临床、病因和病理并作介绍。     

脑桥中央髓鞘溶解症的临床和MRI特点(8例报告)

目的 探讨脑桥中央髓鞘溶解症及脑桥外髓鞘溶解的发病原因、临床特点、治疗、疾病预后及急性期、恢复期MRI影像学特点.方法 对2002年～2006年吉林大学中日联谊医院收治的8例CPM患者的病因、临床表现、MRI影像学特点及治疗转归进行回顾性分析.结果 8例患者均有基础疾病.患者表现不同程度的意识障碍、肢体瘫痪、球麻痹;其中1例无相应的临床症状.6例患者在发病早期行头部MRI检查,表现脑桥基底部对称分布的病灶;其中的3例伴有脑桥外的髓鞘溶解,病灶主要分布在丘脑、壳核;其中的2例在发病后的1个月复查头部MRI,发现脑桥病灶有不同程度的缩小,表现T1W1低信号、T2W2高信号;丘脑、壳核的病灶表现T1 W1高信号、T2W2高信号.除1例无临床症状及1例肝移植术后患者外,其余6例一经明确诊断,均立即行激素治疗.6例患者基本恢复正常,生活可以自理.结论 肝功能损伤、慢性肾功能不全的患者容易出现脑桥中央及脑桥外的髓鞘溶解症;患者激素治疗有效;头部MRI是最有效的检查方法,是确诊的重要依据;疾病恢复期丘脑、壳核病灶的短T1、长T2的影像学改变考虑与髓鞘溶解后的脂类堆积有关.     

垂体后叶素致脑桥外髓鞘溶解症的临床特点(附1例报告)

目的 分析垂体后叶素致脑桥外髓鞘溶解症( EPM)的临床特点.方法 回顾性分析1例应用垂体后叶素致EPM患者的临床资料.结果 该患者因支气管扩张咯血予垂体后叶素静脉滴注治疗6d后出现精神行为异常,当时血钠为109.0 mmol/L;2 d内低钠血症纠正后精神症状加重并相继出现意识障碍及发作性抽搐;查体发现咽反射减弱,四肢肌力、肌张力下降,病理反射阳性;血钠137.6 mmol/L;头颅MRI示双侧尾状核、豆状核、丘脑对称性T1低信号,T2、Flair和DWI高信号改变;经糖皮质激素及对症支持治疗后患者症状有所好转,16个月后生活部分自理.结论 垂体后叶素可引起低钠血症继发EPM,过快的纠正低钠血症可加重脑组织的损害.     

Magnetic Resonance Imaging in Complex Partial Seizures

Abstract: Magnetic resonance imaging (MRI) and computed tomography (CT) were performed on 45 patients with intractable complex partial seizures. MRI was performed with a super conducting whole-body scanner operating at 0.5 tesla (T) and 1.5 T. In patients with temporal lobe epilepsy, 8 of 24 patients had abnormal CT, but 16 of 24 patients showed abnormal MRI. 1.5 T MRI detected more abnormality than 0.5 T MRI when CT was normal. In patients with frontal lobe epilepsy, 5 of 7 patients had normal CT and MRI. In 2 other patients, MRI demonstrated an arachnoid cyst and increased signal intensity area on the T2-weighted images, which were not detected by CT. In patients with occipital lobe epilepsy, 5 of 6 patients show abnormal CT and MRI. In patients with tuberous sclerosis, MRI revealed some increased signal intensity areas on the T2-weighted images in the occipital and temporal lobe, which were not detected by CT. Most surface EEG foci corresponded with the side of MRI abnormality. These data indicate that MRI is more informative than CT in complex partial seizures. MRI is the imaging technique of choice in the diagnosis of complex partial seizures.     

Magnetic resonance imaging in complex partial seizures

Magnetic resonance imaging (MRI) and computed tomography (CT) were performed on 45 patients with intractable complex partial seizures. MRI was performed with a superconducting whole-body scanner operating at 0.5 tesla (T) and 1.5 T. In patients with temporal lobe epilepsy, 8 of 24 patients had abnormal CT, but 16 of 24 patients showed abnormal MRI. 1.5 T MRI detected more abnormality than 0.5 T MRI when CT was normal. In patients with frontal lobe epilepsy, 5 of 7 patients had normal CT and MRI. In 2 other patients, MRI demonstrated an arachnoid cyst and increased signal intensity area on the T2-weighted images which were not detected by CT. In patients with occipital lobe epilepsy, 5 of 6 patients show abnormal CT and MRI. In patients with tuberous sclerosis, MRI revealed some increased signal intensity areas on the T2-weighted images in the occipital and temporal lobe, which were not detected by CT. Most surface EEG foci corresponded with the side of MRI abnormality. These data indicate that MRI is more informative than CT in complex partial seizures. MRI is the imaging technique of choice in the diagnosis of complex partial seizures.     

肝豆状核变性合并中央髓鞘溶解症的临床和影像学特点(附2例报告)

目的 探讨肝豆状核变性(WD)合并脑桥中央髓鞘溶解症(CPM)的临床及头颅MRI特点.方法 回顾性分析2例出现脑桥中央髓鞘溶解的WD患者的临床资料,总结其临床特征并进行文献复习.结果 2例WD患者基础状态均较差,在妊娠反复呕吐及严重吞咽困难后,出现急性的延髓麻痹加重及四肢瘫痪;MRI在T2序列上有脑桥中央部大致对称圆形...     

The significance of MRI abnormalities in children with neurofibromatosis

We prospectively evaluated 47 children with neurofibromatosis to determine whether the previously reported high signals on magnetic resonance imaging (MRI) (prolonged T2) correlated with CT, brainstem auditory evoked responses (BAER), EEG, clinical examinations, cognitive abilities, or seizure disorder. Thirty percent of children had a history of seizures and 70% had either learning disabilities or mental retardation. Overall, 74% had an abnormal MRI examination. Sixty-two percent had high signals (prolonged T2) on T2-weighted images. Abnormal signals were located primarily in the basal ganglia, brainstem, and cerebellum. Twenty-five percent of patients had abnormal EEGs, 28% had abnormal CTs, and 27% had abnormal BAER examinations. The abnormal signals on MRI did not consistently relate to findings on CT, BAER, EEG, school placement, or clinical examination. The abnormal signals presumably reflect areas of abnormal brain parenchyma, either hamartomas, heterotopias, or local areas of brain dysplasia.     

Clinical course and evolution of lesion in MRI in central pontine myelinolysis in a patient abusing alcohol

The authors report a 44-year-old patient with at least a 10-year history of alcohol abuse in whom an organic brain syndrome developed with cerebellar ataxia, left-side hemiparesis and bulbar symptoms. MR imaging of the head demonstrated a large hyperintense focus in T2-weighted images involving the central part of the pons. Central pontine myelinolysis was diagnosed. Laboratory investigations showed no hyponatraemia. After withdrawal treatment and motor function rehabilitation the neurological symptoms regressed nearly completely. After 18 months control MR imaging demonstrated considerable reduction in the size of the myelinolysis area in the brainstem, despite the fact that the patient resumed alcohol drinking.     

High Signal Intensity on T2- Weighted MRI Correlates with Hypoperfusion in Temporal Lobe Epilepsy

Single-photon emission computed tomography (SPECT) and [99mTc]HMPAO were used to assess the functional significance of nonspecific magnetic resonance imaging (MRI) abnormalities observed in patients with temporal lobe epilepsy and no focal lesion on CT scan. We studied 18 patients whose MRI was normal or showed high signal intensity on T2-weighted images (T2WIs) at the site of the EEG focus in 11 and 7 cases, respectively. EEG was monitored during regional cerebral blood flow (rCBF) study. Lateralized hypoperfusion was present in 7 of 17 interictal (41%) and in one postictal cases; it was located in the temporal lobe on the side of the EEG focus in all, and was significantly more frequent in patients with high signal intensity on T2WI (86%) than in patients with a normal MRI (18%). The degree of temporal perfusion asymmetry measured in each individual was higher in patients whose MRI was abnormal.     

Clinico-radiological correlation of Wilson's disease by magnetic resonance imaging, computed and positron emission tomography

Follow-up magnetic resonance imaging (MRI) and computed tomography (CT) examinations were performed on five patients with Wilson's disease at intervals from 6 to 29 months. We studied the clinical correlation with MRI and CT, and whether the examination of MRI and CT could be useful for evaluation of the therapeutic effect. Positron emission tomography (PET) was also carried out on 4 cases except for an asymptomatic case (patient 2, sister of patient 1). Close relationship has been observed by MRI between dystonia and the lesion of the lenticular nuclei, abnormality of smooth pursuit eye movements and the brain stem lesion, and severe dysarthria/dysphagia and the lesion of the caudate and lenticular nuclei, respectively. In patient 4, repeated MRI of an interval of 18 months demonstrated decrease of the abnormal high signal in the lateral part of the putamen on T2-weighted image in accordance with marked improvement of clinical manifestations. In patient 3, who had severe dystonia of the extremities and trunk, T2-weighted image showed high signals in the lenticular nuclei. Marked decrease of the high signal in the lenticular nuclei was observed by MRI in this patient after 29 months, when her neurological manifestations were markedly improved. Patient 5 with severe cerebellar signs disclosed abnormal signals in the middle cerebellar peduncles, brain stem and dentate nuclei in addition to low signals in the caudate and lenticular nuclei, and high signals in the lateral part of the putamen on T2-sequence.(ABSTRACT TRUNCATED AT 250 WORDS)