Objective To observe the clinical features and visual function of recurrent neuromyelitis optica (NMO). Methods Thirty-four patients with NMO were enrolled in this retrospective case series study. The patients included two males and 32 females. The average first onset age was (35.03plusmn;14.56) years old and the average recurrent rate were (4.24plusmn;2.45) times. The recurrent rate of optic neuritis (ON) ranged from two to 12 times. The recurrent rate of ON was two times in 15 eyes of 10 patients, ge;three times in 37 eyes of 24 patients. Vision acuity, direct ophthalmoscope, fundus pre-set lens examination, visual field and visual evoked potential (VEP) were evaluated. Clinical features were observed. The abnormal rate of optic nerve including optic edema and atrophy; abnormal rate of visual field including decreasing retinal sensitivity, central and paracentral scotoma, ring scotoma, half field defects, tunnel visual field, visual field centrality constriction; abnormal rate of VEP including Prolonged latent phase and/or decreasing amplitude of P100 wave from patients of first episode or recurrence was analyzed. Serum NMO-IgG was detected from 28 patients by indirect immunofluorescence technique to observe its positive rate. Results All patients were characterized by repeated episodes of ON and myelitis. The main clinical feature of ON was visual loss, and the main clinical features of myelitis included sensory disability, dyskinesia and vesicorectal disorder. Blindness rate was 41.67% after the first attack of ON, 33.33% after two relapses, and 64.86% after ge; three relapses. The difference of blindness rate between first attack and two episodes was not significant (chi;2=0.270,P=0.603). However, the blindness rate in patients having ge; three episodes was significantly higher than those having two episodes (chi;2=4.300,P=0.038). With recurrence rate increasing, the abnormal rate of the optic nerve (chi;2=6.750,P=0.034）and VEP（chi;2=6.990,P=0.030）increased. But the abnormal rate of visual field did not increase along with recurrent rate (chi;2=0.660,P=0.718). Seropositive rate of NMO-IgG did not differ significantly between patients with first attack ON and that with recurrent ON (chi;2=1.510,P=0.470). But the seropositive patients had significantly higher bilateral blindness rate than seronegative patients (chi;2=5.063,P=0.027). Conclusions NMO are characterized by recurrent ON and myelitis. Visual loss, sensory disability, dyskinesia and vesicorectal disorder are the main clinical features. With recurrence rate increasing, the blindness rate, abnormalities the optic nerve and the abnormity rate of VEP increase. Seropositive recurrent NMO patients have higher bilateral blindness rate than seronegative patients.
Objective To observe the ocular manifestations and therapeutic effect of multiple sclerosis (MS) and neuromyelitis optica (NMO) with ocular symptoms.Methods The clinical data of 107 patients who was diagnosed with MS and NMO with ocular symptoms in our hospital were retrospectively analyzed, including 81 MS patients and 26 NMO patients.The counting of erythocytes and leukocyte，protein content and oligoclonal bands were detected by MRI and cerebrospinal fluid (CSF) in order to ensure the clinical diagnosis with MS and NMO. All the patients had undergone regular ophthalmologic examination of visual acuity，slit lamp microscope and fundus examination.In addition，visual field and visual evoked potential (VEP) examination were performed to analyze the clinical characteristics of ocular manifestations.The patients were received therapy with large dose methylprednisolone or activating blood and dissolving stasis and trophic nerve by chinese medicine. The effects of three methods on ocular manifestations were analyzed. All the patients were followed up for one month to five years. Results Among 81 MS patients，retrobulbar neuritis occurred in 24 patients (29.6%), the other common symptoms included paralytic strabismus and diplopia（30.3%）. Among 26 NMO patients，acute papillitis occurred in 12（46.2%），while retrobulbar neuritis occurred in 14 (53.8%). The most common symptom of both MS and NMO was impaired vision and high abnormal rate. The results of MRI showed that demyelinating lesions beside ventricle was the most performance in MS patients，while abnormalities in spinal cord were found in NMO patients.The results of CSF showed that the positive oligoclonal bands was 75.3% and 19.2% in MS and NMO patients respectively. The potential time delay and (or) amplitude declination were observed by VEP. Large dose methylprednisolone can improve vision and diplopia in a short period.Conclusion The abnormal ocular manifestations of MS and NMO patients are common and complicated. Ocular symptoms has important reference value in the early diagnosis of MS and NMO.
Objective:To observe the effects of testosterone on optic nerve an d retinal ganglion cells (RGC) in experimental autoimmune encephalomyelitis (EAE ). Methods:Fourty one female Wistar rats were randomly divide d into 3 groups: the normal group (10 rats), the untreated control group (15 rats) and the testos terone group (16 rats). The rats in the first two groups were fed with 1% ethano l every day, and the rats in the testosterone group were fed with methyltestoste rone (0.25 mg/kg) every day. On the 20th day, EAE model was induced in the untre ated control group and the testosterone group by injecting guinea pig spinal cor d homogenate in complete Freund's adjuvant and bordetella pertussis vaccine. RGC were labeled with flurogold (FG) by injecting it in superior colliculus and lat eral geniculate body 7 days before establishing EAE model. All rats were fed wit h drugs continuously, and after 1430 days, rats in normal group and rats in un t reated control and testosterone groups who had symptoms within 48～72 hours were observed by light microscopy and flash visual evoked potential (FVEP) to detect the functional and morphological changes of optic nerve. The number of RGC was counted by fluorescence microscopy，and apoptosis of RGC was observed by termina l deoxynucleotidyl transferasemediated biotinylated UTP nick end labeling (TUN E L) Results:EAE rats presented weakness or paralysis of tail a nd hind limbs 10 days after establishing EAE model. Compared with the rats in the untreated contr ol group, the rats in the testosterone group had longer disease delitescence and lower clinical score (P=0.042). Extensive demyelination of optic nerves wi th the circuitous configuration was found in the untreated control group; while mild demyelination of optic nerves with regular figure was found in the testosterone group. In the testosterone group, the latency of N1、P and N2 wave was shorter w hile the amplitude ofN1-P and P-N2was higher than that in the untreated cont rol group (Plt;0.05). The number of RGC was (2284plusmn;132), (934plusmn;78, and (1725 plusmn;95)cells/mm2 in the normal, untreated control and testosterone groups, respectively; w hich was higher in testosterone group than that in untreated control group (P=0.028). The number of TUNEL positive cells was (4.02plusmn;0.16), (24.44plusmn;2.22), and (9.84plusmn;2.36) cells per high power field (times;400) in the 3 grou ps, respectively; wh ich was less in testosterone group than that in untreated control group (P＝0.025). Conclusions:Testosterone may reduce the incidence and clinical score of EAE, inhibit the apoptosis of RGC, alleviate the demyelinatio n of optic nerves, and improved the conduction function of optic nerves.
【摘要】 目的 分析合并免疫指标异常的视神经脊髓炎临床特点。 方法 回顾性分析2009年5月-2010年11月收治的62例视神经脊髓炎患者中24例合并免疫指标异常患者的临床资料。24例均为女性，发病年龄14~53岁。对其临床表现、视觉诱发电位、影像学检查结果、免疫检查结果进行分析。 结果 所有患者均有脊髓和视神经同时或先后受累的表现。24例视觉诱发电位检查23例异常。脊髓MRI显示病变集中于颈段、上胸段脊髓。颈段和胸段脊髓同时受累17例，单纯颈段脊髓受损6例，单纯胸段脊髓受损1例。所有患者抗核抗体滴度均≥1∶100，合并抗SSA抗体阳性14例（55.5％），同时合并抗SSB抗体阳性11例（45.8％），合并抗Rib抗体阳性1例，合并抗SCL-70抗体阳性1例，合并抗dsDNA抗体1例。 结论 视神经脊髓炎合并免疫指标异常的患者以女性较为多见，易复发，青壮年患者发病率最高。脊髓MRI示病变集中于颈段、上胸段脊髓，表现为长节段脊髓损害。视神经脊髓炎患者合并结缔组织病的病例较多。【Abstract】 Objective To analyze the clinical features of neuromyelitis optica (NMO) combined with abnormal immune parameters. Methods We retrospectively reviewed the clinical data of 24 patients with NMO and abnormal immune parameters among the 62 NMO patients who were admitted into our department between May 2009 and November 2010. All patients were female, aged from 14 to 53 years. We analyzed their clinical manifestations, visual evoked potentials, imaging results, and immunological examinations. Results All patients had simultaneous or successive spinal cord and optic nerve involvement. Twenty-three patients had abnormal visual evoked potential. MRI showed that the lesions focused on the cervical and upper thoracic spinal cord. Both cervical and thoracic spinal cord were involved in 17 cases; there were 6 cases of simple cervical spinal cord injury and 1 case of simple thoracic spinal cord damage. Antinuclear antibody titer of all the patients was ≥1∶100. Combined positive anti-SSA antibody occurred in 14 patients (55.5%); Concomitant positive anti-SSB antibodies occurred in 11 patients (45.8%); Combined positive anti-Rib antibodies occurred in 1 patient; Combined positive anti-SCL-70 antibody occurred in 1 patient; and combined positive anti-dsDNA antibodies occurred in 1 patient. Conclusions NMO combined with abnormal immune parameters mainly occurs in female patients, especially in young people. Recurrence rate is high. MRI shows that the lesions focus mainly on the cervical and upper thoracic spinal cord, manifesting the characteristic of long segment damage. And NMO is frequently combined with connective tissue disease.
【摘要】 目的 在实验性自身免疫性脑脊髓炎(experimental autoimmune encephalomyelitis,EAE)模型中,比较常规T2加权成像(T2weighted imaging,T2WI)、钆二乙三胺五醋酸(gadoliniumdiethylenetriamine pentaacetic acid,GdDTPA)和超顺磁性氧化铁(superparamagnetic iron oxide,SPIO)增强图像之间的差异,探讨巨噬细胞在多发性硬化(multiple sclerosis,MS)炎性活动病灶中的细胞学标志。方法 在EAE模型临床症状的亚临床期、初发期、高峰期,13只复发缓解(relapsingremitting,RR)EAE大鼠模型组和13只正常对照组大鼠在注入对比剂之前均行常规T2WI扫描,接着分别在其尾静脉注入GdDTPA后5 min行T1加权成像(T1weighted imaging,T1WI),再注入SPIO,24 h后行T2WI扫描。扫描完毕后立即处死大鼠取脑,行脑组织切片的ED1免疫组织化学染色和Prussian blue染色。结果 EAE模型组大鼠在第11天出现临床症状(初发期),第14天达到高峰期;MRI检查:SPIO增强图像对EAE病灶的显示较常规T2WI和GdDTPA增强图像好。病理学检查:ED1染色,在SPIO显示为低信号的区域内出现了炎症细胞(以巨噬细胞为主)浸润;Prussian blue染色示病灶内巨噬细胞胞质内出现了蓝染颗粒,沉积部位与T2WI上低信号区对应。对照组大鼠均无异常。结论 SPIO较GdDTPA更好地显示EAE模型中炎性活动性病灶内血管周围以巨噬细胞为主的浸润。
【摘要】 目的 分析急性播散性脑脊髓炎的临床特点，提高诊疗。 方法 收集1999年1月-2010年1月住院的急性播散性脑脊髓炎患者42例，对其临床症状体征、实验室检查、影像学改变及治疗进行全面回顾性分析。 结果 42例患者中5～14岁者11例（26.19%）；15～40岁者20例（47.62%），感染后引起的23例（54.76%），无明显诱因占15例（35.71%）；脑脊液23例（23/34，67.65%）异常；脑电图异常者27例（27/32，84.38%）；CT检查阳性率26例（26/40，65.00%），MRI阳性率25例（25/28，89.29%）；糖皮质素、丙种球蛋白治疗有效。 结论 急性播散性脑脊髓炎是一组临床表现多样的免疫介导的炎性疾病，脑脊液、MRI和脑电图有重要诊断价值。急性期大剂量皮质素、静脉丙种球蛋白治疗均有较好疗效。【Abstract】 Objective To analysis the clinical features of acute disseminated encephalomyelitis so as to improve medical treatment. Methods From January, 1999 to January, 2010, 42 inpatients with acute disseminated encephalomyelitis were collected and their clinical data were analyzed retrospectively. Results Out of these 42 patients, 11 (26.19% ) were within 5 to 14 years, 20 (47.62%) ithin 15 to 40 years; 23 (54.76%) had definite infection, and 15 (35. 71%)had no any causes; 23 (23/34, 67.65%) had abnormal cerebrospinal fluid; 27 (27/32, 84.38%) had abnormal electro-encephalograph; 26 (26/40, 65.00%) were CT positive, 25 (25/28, 89.29%) MRI positive; corticosteroids and gamma globulin were effective in the treatment of disseminated encephalomyelitis. Conclusion Acute disseminated encephalomyelitis is a kind of inflammatory disease with various clinical manifestation and mediated by immune. Cerebrospinal fluid, MRI, and electro-encephalograph have important roles in its diagnosis. Large dose of corticosteroids and gamma globulin are effective in the treatment of acute disseminated encephalomyelitis.
目的：探讨复发型视神经脊髓炎(relapsing neuromyelitis optica，R-NMO)与复发型多发性硬化(relapsingremitting multiple sclerosis，RRMS)患者视神经的MRI影像表现。方法：对临床满足R-NMO最新Wingerchuk等制定的诊断标准及满足最新McDonald诊断标准的RRMS患者，进行脑部、脊髓及视神经的扫描，并将两组复发型患者视神经MRI表现特点，与32位健康自愿者进行对照研究。同时回顾性分析患者的VEP检查结果。结果：41例R-NMO患者MRI检查显示患者均有单侧或双侧视神经鞘膜腔信号明显增高，呈“轨道样”改变，22例患者(53.7%)伴有视神经增粗，或(和)扭曲，17例(41.5%)可看视神经内点状高信号表现。随着病程的延长，15例(36.6%)MRI上可见单侧或双侧视神经萎缩、变细，甚至扭曲。最终35例(80.5%)视神经内见到点状高信号表现。121例RRMS患者中，共81名(66.9%)患者在MRI上见到了视神经的异常表现，主要表现为单侧或双侧视神经鞘膜腔部分节段或全段呈“轨道样”高信号改变，其中23例(28.4%)表现为视神经球球后起始段局限性信号增高，视神经15例(12.4%)伴有视神经的肿胀，但视神经萎缩不及R-NMO患者明显。随着时间延长，12例(9.9%)可见视神经内点状高信号脱髓鞘改变。两组患者P100峰值延迟时间差异具有统计学意义(Plt;0.05)。结论：MRI可以成为视神经炎的首选影像学检查方法。两组患者MRI与VEP检查结果存在一定的差异性，可以成为鉴别诊断的依据。