Niemann-Pick disease type C (NPC) is an autosomal recessive hereditary disease in which sphingomyelin and cholesterol are deposited in various organs of the body. The clinical manifestations of NPC include neurologic symptoms and cataplexy; other symptoms related to sleep have seldom been reported. One previous study described various sleep disorders including chronic insomnia, obstructive sleep apnea, restless legs syndrome, and rapid eye movement sleep behavior disorder, thus suggesting that sleep disorders in patients with NPC are more prevalent than previously thought and warrant close attention. Here, we describe sleep disorders in 2 patients with NPC and discuss the clinical characteristics and, for the first time, discuss potential pathogenic mechanisms underlying sleep disorders in such patients.

Objective: To assess the characteristics of Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorder (MOGAD) with brainstem involvement in the first event (BSIFE) and make comparisons with aquaporin-4IgG seropositive neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS). Methods: From 2017 to 2022, this study identified MOG-IgG-positive patients with brainstem or both brainstem and cerebellum lesions in the first episode. As a comparison group, AQP4-IgG-NMOSD (n = 30) and MS (n = 30) patients with BSIFE were enroled.Results: Thirty-five patients (35/146, 24.0%) were the BSIFE of MOGAD. Isolated brainstem episodes occurred in 9 of the 35 (25.7%) MOGAD patients, which was similar to MS (7/30, 23.3%) but was lower than AQP4-IgGNMOSD (17/30, 56.7%, P = 0.011). Pons (21/35, 60.0%), medulla oblongata (20/35, 57.1%) and middle cerebellar peduncle (MCP, 19/35, 54.3%) were the most frequently affected areas. Intractable nausea (n = 7), vomiting (n = 8) and hiccups (n = 2) happened in MOGAD patients, but EDSS of MOGAD was lower than AQP4IgG-NMOSD (P = 0.001) at the last follow-up. MOGAD patients with or without BSIFE did not significantly differ in terms of the ARR (P = 0.102), mRS (P = 0.823), or EDSS (P = 0.598) at the most recent follow-up. Specific oligoclonal bands appeared in MOGAD (13/33, 39.4%) and AQP4-IgG-NMOSD (7/24, 29.2%) in addition to MS (20/30, 66.7%). Fourteen MOGAD patients (40.0%) experienced relapse in this study. When the brainstem was involved in the first attack, there was an increased likelihood of a second attack occurring at the same location (OR=12.22, 95%CI 2.79 to 53.59, P = 0.001). If the first and second events were both in the brainstem, the third event was likely to occur at the same location (OR=66.00, 95%CI 3.47 to 1254.57, P = 0.005). Four patients experienced relapses after the MOG-IgG turned negative.Conclusion: BSIFE occurred in 24.0% of MOGAD. Pons, medulla oblongata and MCP were the most frequently involved regions. Intractable nausea, vomiting and hiccups occurred in MOGAD and AQP4-IgG-NMOSD, but not MS. The prognosis of MOGAD was better than AQP4-IgG-NMOSD. In contrast to MS, BSIFE may not indicate a worse prognosis for MOGAD. When patients with BSIFE, MOGAD tent to reoccur in the brainstem. Four of the 14 recurring MOGAD patients relapsed after the MOG-IgG test turned negative.

BackgroundImmune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Although cerebellar ataxias (CAs) associated with anti-NMDAR and anti-CASPR2 have been reported in a few cases, they have never been studied systematically. This study aimed to analyze the characteristics of anti-NSAbs-associated CAs.MethodsA retrospective investigation was conducted to identify patients using the keywords IMCAs and NSAbs. We collected the clinical data of 14 patients diagnosed with anti-NSAbs-associated CAs.ResultsThe median age was 33 years (16-66), and the male-to-female ratio was 4:3. Nine were positive for NMDAR-Ab, two for LGI1-Ab, two for CASPR2-Ab, and one for AMPA2R-Ab. CAs were initial symptoms in three patients and presented during the first two months of the disease course (10 days on average) among the rest of the patients. After the immunotherapy, two cases were free from symptoms, and eight cases recovered satisfactorily (10/14, 71.4%). Compared with other causes of IMCAs, anti-NSAbs were more frequently associated with additional extra-cerebellar symptoms (85.7%), mostly seizures (78.6%) and mental abnormalities (64.3%). In the CSF analysis, pleocytosis was detected in ten patients (71.4%) and oligoclonal bands (OB) were observed in nine patients (64.3%). Moreover, compared with PCD and anti-GAD65-Ab-associated CAs, anti-NSAbs-associated CAs showed a better response to immunotherapy.ConclusionIMCAs are rare and atypical in autoimmune encephalitis with neuronal surface antibodies. Compared with other forms of IMCAs, more symptoms of encephalopathy, a higher rate of pleocytosis and positive OB in CSF, and positive therapeutic effect were the key features of anti-NSAbs-associated CAs.

We investigated the safety and explore potential efficacy of batoclimab administered subcutaneously in Chinese patients with generalized myasthenia gravis (gMG). A randomized, double-blinded, placebo-controlled, parallel phase II study was conducted. First, in the double-blinded treatment period, eligible patients received batoclimab (680 mg), batoclimab (340 mg), or placebo on days 1, 8, 15, 22, 29, and 36. In the open-label treatment period, patients received batoclimab (340 mg) on days 50, 64, and 78. In the follow-up period, patients were examined on days 92, 106, and 120. The primary endpoint was Myasthenia Gravis Activities of Daily Living (MG-ADL) score change on day 43 from baseline. In total, 30 eligible patients were enrolled, with 11, 10, and 9 patients in the batoclimab 680 mg, batoclimab 340 mg, and placebo groups, respectively. MG-ADL score changes from baseline to day 43 were −2.2 ± 0.9, −4.7 ± 0.6, and −4.4 ± 1.0 in the placebo, batoclimab 340 mg, and 680 mg groups, respectively. Similar changes were observed in Quantitative Myasthenia Gravis, Myasthenia Gravis Composite, and 15-item Myasthenia Gravis Quality of Life scores in the placebo, batoclimab 340 mg, and 680 mg groups, respectively. The proportion of patients with clinically significant improvement on day 43 was higher in the batoclimab groups. On day 120, all four scales in the placebo group had more significant improvement compared with the batoclimab groups, with total serum IgG levels reaching a plateau. No death or treatment-emergent adverse events (TEAEs) led to study discontinuation. Batoclimab is effective and safe in Chinese patients with gMG. This study was registered at ClinicalTrials.gov (NCT04346888) on 15 April 2020, with the first patient enrolled on 23 July 2020.

Objective: To explore the disease manifestations and radiological characteristics of patients with meningeal carcinomatosis (MC) combined with myelopathy. Patients and Methods: The detailed medical information of patients who suffered from MC with myelopathy in record system were collected and reviewed. Results: In these patients, five cases were male and two cases were female. The age was from fifteen to fifty-seven years. In the course of disease, tumor cells were discovered in cerebrospinal fluid of three patients and in biopsy samples of four patients. Cerebrospinal fluid (CSF) test results showed white blood cell counts increased in seven patients, protein increased in six patients and glucose reduced in five patients. In addition, MRI revealed that the white matter abnormalities showed in all cases and pia mater was enhanced in four patients, meningeal enhanced was observed in three patients. All patients were given appropriate therapy during hospitalization. Follow-up result showed that all patients passed away two to five months after diagnosis. Conclusion: MC causes spinal membrane, spinal nerve root to be involved besides, also can produce the matter of myelopathy. Early detection of intramedullary lesion is conducive to strengthening the awareness of the diagnosis of MC.

IntroductionMinimal manifestation (MM) or better was recommended as the treatment goal for myasthenia gravis (MG). The sustainability of this status has not been described quantitatively in patients who had attained or are close to it.MethodsPatients who were with no or slight impact on daily living were recruited and followed at baseline and 3, 6, and 12 months. The included patients were classified into 3 post-intervention status (PIS) categories: remission (R), MM, and slight impact (SI). The proportion of patients belonging to real-time (not considering the intervals between assessments) and sustained (considering the intervals between assessments) PIS categories was compared at each follow-up. A sensitivity analysis (SA) cohort was established by including patients with PIS categories in all four follow-ups. The QMGS, MG-ADL, and MG-QOL15 scores in patients belonging to each PIS category at each follow-up were compared. The sustainability of the R/MM status was examined and correlated with real-time R/MM status at follow-ups.ResultsAt baseline, 376 patients could be classified, including 55 as R (14.2%), 209 as MM (54.0%), and 112 as SI (28.9%). In the whole cohort, 68.8–89.7%, 71–76.7% and 19.8–77.1% of the patients classified into real-time R, MM, and SI categories remained unchanged in each follow-up compared with the previous follow-up. The proportion of patients belonging to each real-time or sustained R/MM status at the three follow-ups was 89.7–92.1 or 60.8–67. In the SA cohort, at least 86.4% of the baseline R/MM patients remained in R/MM status till 12 months. There were no differences in keeping real-time R/MM status at 6 or 12 months between patients with and without sustained R/MM status at 3 and 6 months. There were differences in the QMGS, MG-ADL, and MG-QOL15 scores among patients belonging to each real-time category at baseline and follow-ups, ranking as R < MM < SI. The same trend was observed in patients belonging to each sustained PIS category with smaller scores than the same items of real-time categories.ConclusionThe sustainability of the R/MM status was confirmed. The R/MM status indicated a stable state of MG. The QMGS, MG-ADL, and MG-QOL15 scores may provide a quantitative reference for these PIS.

Treatment of depression with antidepressants is partly effective. Transcranial alternating current stimulation can provide a non-pharmacological alternative for adult patients with major depressive disorder. However, no study has used the stimulation to treat first-episode and drug-naïve patients with major depressive disorder. We used a randomized, double-blind, sham-controlled design to examine the clinical efficacy and safety of the stimulation in treating first-episode drug-naïve patients in a Chinese Han population. From 4 June 2018 to 30 December 2019, 100 patients were recruited and randomly assigned to receive 20 daily 40-min, 77.5 Hz, 15 mA, one forehead and two mastoid sessions of active or sham stimulation (n = 50 for each group) in four consecutive weeks (Week 4), and were followed for additional 4-week efficacy/safety assessment without stimulation (Week 8). The primary outcome was a remission rate defined as the 17-item Hamilton Depression Rating Scale (HDRS-17) score ≤ 7 at Week 8. Secondary analyses were response rates (defined as a reduction of ≥ 50% in the HDRS-17), changes in depressive symptoms and severity from baseline to Week 4 and Week 8, and rates of adverse events. Data were analysed in an intention-to-treat sample. Forty-nine in the active and 46 in the sham completed the study. Twenty-seven of 50 (54%) in the active treatment group and 9 of 50 (18%) in the sham group achieved remission at the end of Week 8. The remission rate was significantly higher in the active group compared to that in the sham group with a risk ratio of 1.78 (95% confidence interval, 1.29, 2.47). Compared with the sham, the active group had a significantly higher remission rate at Week 4, response rates at Weeks 4 and 8, and a larger reduction in depressive symptoms from baseline to Weeks 4 and 8. Adverse events were similar between the groups. In conclusion, the stimulation on the frontal cortex and two mastoids significantly improved symptoms in first-episode drug-naïve patients with major depressive disorder and may be considered as a non-pharmacological intervention for them in an outpatient setting.

目的 总结硬脊膜动静脉瘘(spinal dural arteriovenous fistula,SDAVF)的临床特点.方法 选取2014年9月至2019年9月首都医科大学宣武医院神经内科收治的接受腰椎穿刺检查的28例SDAVF患者.结果 患者中男27例,女1例.发病年龄41.0～78.0岁,平均55.5岁.临床症状多样,表现为腿痛4例,行走困难21例,肢体麻木22例,尿潴留13例,尿失禁7例.脑脊液白细胞升高2例,蛋白升高21例,免疫球蛋白IgG升高19例.28例患者行脊髓MRI检查,均在MRIT2加权像见髓内高信号,其中12例可见脊髓表面迂曲血管影.为明确诊断,28例患者行全脊髓数字减影血管造影检查,其中瘘口在胸段23例,腰段4例,骶段1例.患者确诊后行瘘切断手术或介入栓塞治疗,术后随访6个月至 1年,22例(84.6％)症状有不同程度好转,病程较长的6例症状无改善,所有病例随访期间无复发.结论 SDAVF是可治性疾病,全脊髓数字减影血管造影是诊断的金标准,早期诊断和治疗往往预后良好.患者脑脊液免疫学指标升高需要警惕SDAVF的可能性.

硬脊膜动静脉瘘(SDAVF)是脊髓血管畸形疾病中最常见的一种,主要表现为横贯性脊髓损害,影像学多表现为长节段脊髓病变伴脊髓表面增多迂曲血管影.该病临床上相对少见,并且可有不典型表现,极易漏诊或误诊为脊髓炎性反应性疾病或椎间盘退行性变等,早期诊断和治疗能明显改善预后.文章对6例不典型SDAVF患者临床特征和实验室资料进行分析,为临床医师提供经验.

Sensorineural hearing loss (SNHL) has been reported rarely in patients with meningeal carcinomatosis (MC). We summarized the clinical data of eight MC patients with SNHL and 35 patients reported from publications. In the eight patients with SNHL, the medium onset age was 48 (range from 37 to 66) years and six (75%) were male. Seven (87.5%) suffered from headaches as the initial symptom, and they experienced SNHL during the first two months after the occurrence of headaches (0.5 to 2 months, average 1.5 months). The audiogram configuration was flat in three patients (37.5%) and showed total deafness in five patients (62.5%). The damage of cranial nerves VI (abducens) was observed in six patients (75%), and four patients (50%) had cranial nerves VII (facial) injury during the disease course. The percentage of damage of cranial nerves was higher than the patients without SNHL (VIth, 75.0% vs. 13.3%, p = 0.002 and VIIth 50.0% vs. 6.7%, p = 0.012). Four (50%) patients suffered from lung adenocarcinoma as primary tumor, two (25%) experienced stomach adenocarcinoma, one had colon cancer, and one patient was unknown. The symptom of SNHL improved after individualized therapy in four patients (focal radiotherapy and chemotherapy for three patients and whole brain radiotherapy for one patient), but all passed away from 2 to 11 months after diagnosis. Total deafness and flat hearing loss in audiogram were the common types of SNHL resulting from MC. MC patients with SNHL were more likely to suffer from the damage of other cranial nerves, especially to cranial nerves VI and VII. Treatment might improve SNHL, but not improve the case fatality rate.

Purpose: The case with staphylococcus aureus meningitis accompanied by intracranial hemorrhage and cerebral infarction is very rare and cerebrovascular complications are often associated with poor outcome. Materials and methods: We describe the clinical characteristics and laboratory data of a patient with meningitis accompanied by cerebrovascular complications. Results: The patient, a young male, was admitted to hospital with 3 weeks of fever, 10 days of slow reaction and 2 days of left limb strength decline. Neurological examination showed cognitive dysfunction, left central hemiplegia and meningeal irritation sign. Brain Imaging examination revealed intracranial hemorrhage and multiple cerebral infarction. The elevated leucocyte and protein, as well as low glucose of cerebrospinal fluid was observed. Cerebrospinal fluid and foot blister culture both suggested staphylococcus aureus infection. With the treatment of meropenem and glucocorticoid, the condition of our patient was improved. Conclusions: Detection of pathogenic bacteria is the gold standard of diagnosis, and timely diagnosis and treatment for pathogens are the keys to a good prognosis for patients.

Background and Objective Essential thrombocythemia (ET) is a rare cause of cerebral venous sinus thrombosis (CVST). Analysis of the risk factors and treatment therapies of CVST in ET has yielded controversial findings. Subjects and Methods We retrospectively investigated the clinical characteristics of CVST events in ET and compared baseline characteristics, causative factors, hematological effects, and treatments between ET patients with and without CVST. Results Overall, 91 of 115 patients who met the ET diagnosis were included in this study. Among them, 23 (25.27%) patients met the diagnostic criteria of ET with CVST for inclusion, 14 (60.87%) of whom were females, with a median age of 34 (range 25–50). CVST diagnosis was made concomitantly to ET in 19 patients (82.61%). The most common symptom and sites of thrombosis of CVST was an acute or subacute headache and sigmoid sinuses, respectively. Compared with ET patients without CVST, ET patients with CVST were significantly younger (37.65±14.45 vs 60.93±13.46, P<0.001) and had lower prevalence of hypertension (4.34 vs 32.35%, P=0.003) and coronary artery disease (0 vs 14.71%, P = 0.045). Patients with CVST presented with significant lower platelet count (510.39±176.71 vs 750.82±249.10, P< 0.001) and higher score of IPSET-thrombosis (P=0.017). Multivariate logistic regression analysis indicated that age (P=0.002, OR 1.096, 95% CI 1.035–1.161), at least one CVRF (P = 0.024, OR 0.037, 95% CI 0.002–0.649), platelet count (P=0.045, OR 0.994, 95% CI 0.989–1.001), and lower percentage of antiplatelet therapy (P=0.035, OR 0.307, 95% CI 0.001-1.280) significantly contributed to the risk of CVST in ET. Conclusion Most patients (95.65%) had a favorable outcome without recurrence after standard anticoagulant and cytoreductive treatment at last follow-up. These findings indicate that CVST may be the initial presentation of ET, with its detection crucial for early diagnosis and appropriate management. Anticoagulant and cytoreductive therapies should be recommended for preventing ET-related CVST with JAK2 V617F mutation.

Objective:To explore the value of metagenomics next generation sequencing of cerebrospinal fluid in the diagnosis test of the pathogen of neurobrucellosis.Methods:Medical records of neurobrucellosis patients who were admitted to Xuanwu Hospital, Capital Medical University from May 2017 to February 2021 were reviewed. Seven patients who underwent cerebrospinal fluid metagenomics next generation sequencing were enrolled. Their clinical characteristics, cerebrospinal fluid results, serological and pathogenic results were analyzed.Results:Among the seven neurobrucellosis patients, including five males and two females, the age was from 21 to 49 [38 (24, 47)] years. Three patients had a history of exposure to cattle and sheep. The duration from onset to diagnosis was 2 to 30 [12 (5, 18)] months. The main neurological manifestations were headache for seven patients, loss of hearing for three patients, paralysis for four patients and urinary and fecal dysfunction for four patients. The blood tests showed that the rose bengal test was positive in three of seven patients, Brucella serum agglutination test was positive in four of six patients, and the blood culture was negative in four patients. The cerebrospinal fluid tests showed that rose bengal test was positive in one of five patients, Brucella serum agglutination test was positive in two of four patients, and the cerebrospinal fluid culture was positive in two of five patients. Cerebrospinal fluid metagenomics next generation sequencing was positive for Brucella in five patients.Conclusions:Comparing with the cerebrospinal fluid Brucella serum agglutination test and cerebrospinal fluid culture, cerebrospinal fluid metagenomics next generation sequencing is sensitive to the diagnosis of neurobrucellosis. It is recommended to perform cerebrospinal fluid metagenomics next generation sequencing in patients with clinically suspected neurobrucellosis or central nervous system infections of which the pathogen cannot be confirmed.

Objective To investigate the clinicopathological features of central nervous system diffuse and multifocal lesions in the cerebral white matter. Methods The clinical and radiological data of 27 cases of diffuse and multifocal lesions in the cerebral white matter were collected from January 2015 to December 2020. The pathological features of these cases were retrospectively analyzed. Results All cases ranged from 10 to 73 years old, female 12 cases, male 15 cases. Clinical manifestations included dyskinesia and paresthesia (17 cases), headache and dizziness (10 cases), cognitive decline and language dysfunction (3 cases each), sleep disorder, visual impairment and epilepsy seizure (2 cases each). They showed diffuse and multifocal lesions in the cerebral white matter involving three or more lobes. Twenty⁃four cases performed stereotactic puncture, and 3 others accepted craniotomy. Pathological findings included 11 tumoral lesions, such as diffuse glioma 8 cases, lymphomatosis cerebri 2 cases, intravascular large B cell lymphoma one case; as well as 16 cases non⁃tumor lesions, such as idiopathic inflammatory demyelinating diseases 10 cases, primary angiitis of central nervous system 3 cases, progressive multifocal leukoencephalopathy one case, leukoencephalopathy with cerebral calcifications and cysts one case, and adult⁃onset leukoencephalopathy with axonal spheroids and pigmented glia one case. Conclusions Diffuse and multifocal lesions of the cerebral white matter includes tumor and non⁃tumoral diseases with no specific clinical manifestations. Stereotactic puncture is needed to reach a correct clinicopathological diagnosis, and ensure the precision treatment. doi:10.3969/j.issn.1672⁃6731.2021.04.007

Objective? Neuropsychiatric systemic lupus erythematosus (NPSLE) encompasses a wide spectrum of neurologic and psychiatric disorders. The neuropsychiatric manifestations can be divided into multiple subtypes. This study aims to describe neuroimaging abnormalities and autobody profile of patients with NPSLE and correlate them with clinical subtypes. Method Patients with NPSLE in Xuanwu Hospital were included between January 1st 2010 to December 31st 2017. Clinical features, neuroimaging, and immunological data were recorded and summarized per NPSLE subtypes. The correlation between clinical subtypes and imaging findings and autobody levels were analyzed.Results? 34 patients with NPSLE presented heterogeneous clinical manifestations and imaging findings. The most common neuropsychiatric symptoms were cognitive impairment (23.5%) and headache (14.7%). The most common brain MRI abnormalities were lesions of cerebral small vessel disease (50.0%) and inflammatory lesions (47.1%). Statistically significant positive correlations between cognitive impairment and acute confusion state and MRI inflammatory lesions were found (P<0.05). Statistically significant positive correlations between acute cerebral vascular disease of NPSLE and serum anti-phospholipid antibody were found (P<0.05). Conclusion The clinical and neroimaging manifestations of NPSLE were heterogeneous. Inflammatory lesions on MRI might be correlated with cognitive impairment and acute confusion state. Serum anti-phospholipid antibody might be correlated with acute cerebral vascular disease of NPSLE.

BACKGROUND: Glucocorticoid inducing paraplegia has been reported and regard as a diagnostic clue for spinal dural arteriovenous fistulas (DAVFs). Intracranial DAVF in the posterior fossa draining into the perimedullary venous system can induce congestion of spinal cord while often be misdiagnosed and treated with steroid. CASE PRESENTATION: A 54-year-old woman presented progressive bilateral extremities weakness, bowel and bladder symptoms. A cervical MRI showed a longitudinally extensive spinal cord lesion from medulla oblongata to level of T4. A diagnose of NMO and cervical spondylopathy was made. On hospital day 2, methylprednisolone was prescribed at dose of 1g intravenous daily. After that, the patient experienced acute paraplegia. Treatment was stopped. And the patient improved on the hospital day 6, almost returned to her initial state. So the diagnosis of SDAVF was suspected. Spinal angiography was performed but the result was normal, and cerebral angiography demonstrated an DAVF fed by the right meningohypophyseal trunk and drain through right petrosal vein and into perimedullary venous system. Operation was performed successfully and the fistula was obliterated. The patient experienced an improvement of weakness and incontinence. CONCLUSIONS: We reported a case of intracranial DAVF with acute paraplegia response to intravenous glucocorticoid therapy.Intracranial DAVF can also lead venous congestion of the spinal cord, which induce similar clinical manifestation with spinal DAVF, the MRI examination of spinal cord can also find cord edema and typical enlarged medullary veins.

目的 探讨多发性硬化(multiple sclerosis,MS)、视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)与急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)的临床特征.方法 收集2014-2015年首都医科大学宣武医院神经内科44例MS,42例NMOSD及55例ADEM患者的临床资料,对3组患者的临床表现及实验室检查、影像学结果进行对比分析.结果 MS组男女比例为1∶1.59,NMOSD组为1∶4.25,ADEM组为1∶0.57.MS组和NMOSD组起病前均无感染,ADEM组起病前出现头痛17例,发热24例,头晕6例,上呼吸道感染4例.痫性发作仅出现在ADEM组(7/55),NMOSD组中肢体疼痛(19/42)及皮肤瘙痒(12/42)较其他两组突出(P=0.000,P=0.000);ADEM组中球部症状(16/55)及认知障碍(32/55)更为常见(P=0.004,P=0.000).所有患者均行腰椎穿刺检查,ADEM组脑脊液白细胞数显著高于MS组和NMOSD组(P=0.000,P=0.000),MS组IgG寡克隆区带阳性率为69.1％,NMOSD组为19.1％,ADEM组为35.4％,MS组显著高于NMOSD组和ADEM组(P=0.000,P=0.002).NMOSD组血清AQP-4抗体检测阳性32例,阳性率为76.2％.头颅MRI显示病灶位于大脑白质、丘脑、小脑或者基底节区的例数比在MS组及ADEM组中更为突出.结论 NMOSD组女性更多见,ADEM组发病前驱感染症状突出,肢体疼痛及皮肤瘙痒在NMOSD组中常见,认知障碍、痫性发作、球部症状及二便障碍的出现多提示ADEM.ADEM组脑脊液白细胞计数较高,MS组IgG寡克隆区带阳性率更为显著,血清AQP-4抗体阳性对NMOSD有重要提示作用.头颅MRI检查,MS和ADEM以大脑半球白质、脑干病灶为主.上述特异性表现对3种疾病有较高的鉴别价值.

Objective To explore immunological diagnostic characteristics in serum and cerebrospinal fluid (CSF) of neurosyphilis patients. Methods Thirty-five patients with neurosyphilis admitted to Xuanwu Hospital of Capital Medical University during June 2013 to July 2016 were selected for retrospective analysis, including 32 patients with serum and CSF immunological test results. Fisher exact test was used to compare the differences of IgA and IgM between serum group and cerebrospinal fluid group meanwhile, the difference of IgG between serum and cerebrospinal fluid group was compared using Chi-square test. The positive detection rate of oligoclonal bands (OB) and the 24-hour synthesis rate of immunoglobulin in cerebrospinal fluid were also analyzed. Results Serum and CSF Immunoglobulin levels were in 32 patients with neurosyphilis. In serum group, IgG level elevated in 53.13%(17/32) patients, IgA level elevated in 6.25% (2/32) patients, and IgM level elevated in 0% (0/32) patient. Nevertheless, CSF-IgG increased in 84.38% (27/32) patients, CSF-IgA increased in 100.00% (32/32) patients and CSF-IgM increased in 90.63% (29/32) patients. There were statistically significant differences in the elevation rate of IgA and IgM between the serum and cerebrospinal fluid group (χ2=56.47, 53.03, P 0.05). The positive rate of oligoclonal band in cerebrospinal fluid was as high as 100.00%, the 24-hour synthesis rate of immunoglobulin in cerebrospinal fluid increased in 96.00% (24/25) patients. Conclusions Neurosyphilis, with various clinical manifastations, is a treatable and easily misdiagnosed disease. Humoral immunity may play an important role in its development. Serum and CSF IgG increase, CSF IgA and IgM elevation and serum IgA and IgM are normal, CSF OB positive, CSF-24 h IgG synthetic ratio elevation are meaningful immunological characteristics for neurosyphilis diagnosis. Key words: Neurosyphilis; Serum; Cerebrospinal fluid; Immune