【摘要】 目的 分析急性播散性腦脊髓炎的臨床特點，提高診療。 方法 收集1999年1月-2010年1月住院的急性播散性腦脊髓炎患者42例，對其臨床癥狀體征、實驗室檢查、影像學改變及治療進行全面回顧性分析。 結果 42例患者中5～14歲者11例（26.19%）；15～40歲者20例（47.62%），感染后引起的23例（54.76%），無明顯誘因占15例（35.71%）；腦脊液23例（23/34，67.65%）異常；腦電圖異常者27例（27/32，84.38%）；CT檢查陽性率26例（26/40，65.00%），MRI陽性率25例（25/28，89.29%）；糖皮質素、丙種球蛋白治療有效。 結論 急性播散性腦脊髓炎是一組臨床表現多樣的免疫介導的炎性疾病，腦脊液、MRI和腦電圖有重要診斷價值。急性期大劑量皮質素、靜脈丙種球蛋白治療均有較好療效。【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.
【摘要】 目的 分析合并免疫指標異常的視神經脊髓炎臨床特點。 方法 回顧性分析2009年5月-2010年11月收治的62例視神經脊髓炎患者中24例合并免疫指標異常患者的臨床資料。24例均為女性，發病年齡14~53歲。對其臨床表現、視覺誘發電位、影像學檢查結果、免疫檢查結果進行分析。 結果 所有患者均有脊髓和視神經同時或先后受累的表現。24例視覺誘發電位檢查23例異常。脊髓MRI顯示病變集中于頸段、上胸段脊髓。頸段和胸段脊髓同時受累17例，單純頸段脊髓受損6例，單純胸段脊髓受損1例。所有患者抗核抗體滴度均≥1∶100，合并抗SSA抗體陽性14例（55.5％），同時合并抗SSB抗體陽性11例（45.8％），合并抗Rib抗體陽性1例，合并抗SCL-70抗體陽性1例，合并抗dsDNA抗體1例。 結論 視神經脊髓炎合并免疫指標異常的患者以女性較為多見，易復發，青壯年患者發病率最高。脊髓MRI示病變集中于頸段、上胸段脊髓，表現為長節段脊髓損害。視神經脊髓炎患者合并結締組織病的病例較多。【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.
ObjectiveTo preliminary investigate the impact of the diagnosis-related groups (DRG) payment method reform on the diagnosis and treatment of inpatient medical insurance patients with neuromyelitis optica spectrum disorders (NMOSD), and to propose potential improvement strategies. MethodsA single-center, retrospective study. From October 1, 2020, to September 30, 2022, 44 hospitalized medical insurance patients with acute-phase NMOSD diagnosed and treated at the First Affiliated Hospital of Northwest University (Xi'an First Hospital) were included in the study. Among them, there were 11 males and 33 females, with an average age of (40.8±20.2) years. According to the implementation time of DRG payment, patients were divided into two groups: group A, which consists of cases one year before the implementation of DRG payment from October 1, 2020 to September 30, 2021, and group B, which consists of cases one year after the implementation of DRG payment from October 1, 2021 to September 30, 2022, with 20 and 24 cases, respectively. Detailed information such as hospitalization duration, treatment methods, and hospitalization costs of the two groups of patients was collected. Comparative analysis was conducted on hospitalization costs and treatment methods between the two groups. For intergroup comparison, t-test was used for normally distributed data, and Mann-Whitney U test was used for skewed distributed data. ResultsAmong the 44 patients, 5 cases (5/24, 20.8%) received plasma exchange (PE) treatment, all of whom were in group B. The numbers of patients who received and did not receive intravenous immunoglobulin (IVIG) treatment were 9 and 11 in group A, respectively, and 7 and 12 in group B (except for 5 cases who received PE treatment), respectively. Compared with group A, there was no significant decrease in hospitalization duration (t=0.004) and total hospitalization costs (Z=0.036), as well as costs for western medicine (Z=0.036), examinations (Z=0.011), laboratory tests (Z=0.040), treatments (Z=0.017), and nursing (Z=3.131) in group B, and the differences were not statistically significant (P＞0.05). For patients receiving PE treatment, except for the cost of western medicine (Z=0.062, P=0.804), the other costs (Z=8.288, 5.013, 11.400, 10.925, 9.126) were significantly higher than those of patients not receiving PE treatment, and the hospitalization duration (t=20.474) was significantly prolonged, with statistically significant differences (P＜0.05). The total hospitalization costs of patients receiving IVIG treatment were significantly higher than those not receiving IVIG treatment in both group A and group B, with statistically significant differences (Z=7.690, 10.314; P＜0.05). There was no statistically significant difference in the comparison of total hospitalization costs between patients receiving IVIG treatment in group A and group B (Z=0.137, P＞0.05). ConclusionsThere is no significant decrease in various hospitalization costs of NMOSD medical insurance patients in Xi'an after the implementation of DRG payment, especially for patients receiving PE treatment. It is suggested to optimize the rate stratification of NMOSD patients when implementing DRG payment methods.
Plasma exchange (PE) is a therapeutic blood component replacement method. The blood of patients is first separated into plasma and blood cell components using a blood cell separator in vitro, the plasma containing harmful pathogenic substances is then discarded and replaced with the same volume of exchange solution. Finally the separated blood cells together with the exchange solution are returned back to the blood circulation of patients. By reducing the circulating antibodies, abnormal plasma proteins or cytokines and other pathogenic molecules, PE can block the disease process. PE has a good therapeutic effect on neuromyelitis optica-related optic neuritis (NMO-ON), which shows resistant to glucocorticoid therapy for the first onset. The American Society for Apheresis guideline evaluates PE for acute optic neuritis as a recommended grade 1B, type II indication. In the implementation of PE treatment for NMO-ON and other diseases, indications and contraindications should be strictly adhered to the guideline, treatment procedures and protocols should be optimized, common adverse events and its prevention and management should be known and alerted. It is important to conduct multi-center clinical cooperation and a high standard clinical randomized controlled study, to find out the optimal time window, the best protocol, and the associated factors for the efficacy and prognosis of PE in NMO-ON.
Neuromyelitis optica (NMO) is an autoimmune inflammatory diseases of the central nervous systems (CNS) mainly affecting the optic nerves and spinal cord. It has the characteristics of high recurrence rate and poor prognosis. NMO related optic neuritis is a common neuro-ophthalmic disease which often results in permanent visual loss or even blindness. Aquaporin 4 (AQP4) antibody is a specific and pathogenic autoantibody in NMO patients. Although AQP4 is expressed in multiple tissues, NMO pathology is remarkably limited to the CNS. Corticosteroids and other immunosuppressive drugs are the standard managements for NMO patients, in order to reduce the relapses and the severity of the acute attack. Multiple avenues of investigation in the laboratory have significantly advanced our understanding of NMO pathophysiology, which is helpful for our understanding of immunologic and nonimmunologic mechanisms. Many offer significant means for NMO therapy by selectively targeting pathways. In the future, moving these agents from the bench to the bedside offers the opportunity to identify safe and effective therapies that limit CNS injury and preserve visual function.
ObjectiveTo observe and analyze the subtype-specific prognostic factors for visual recovery in patients with demyelinating optic neuritis (DON) after glucocorticoid pulse therapy. MethodsA retrospective cohort study. A total of 195 patients (249 eyes) with DON diagnosed by ophthalmology examination at Department of Ophthalmology, Xi'an People's Hospital (Xi'an Fourth Hospital) from January 2021 to December 2024 were included in the study. According to the results of serum antibody detection and clinical diagnostic criteria, the patients were divided into the neuromyelitis optica spectrum disorder (NMOSD)-associated optic neuritis (ON) (NMOSD-ON) group, the myelin oligodendrocyte glycoprotein antitide-associated ON (MOG-ON) group, and the double antibody negative ON group. They were 51 cases (58 eyes), 72 cases (103 eyes), and 72 cases (88 eyes) respectively. Baseline clinical data, imaging characteristics, and treatment protocols were collected. The primary endpoints were complete visual recovery [best-corrected visual acuity (BCVA) ≥1.0] and moderate recovery (BCVA ≥0.5) at 3 months post-onset. Multivariate logistic regression was used to identify independent prognostic factors for visual outcomes within each subtype. ResultsAt 3 months post-onset, complete recovery rates were 9 (15.5%, 9/58) in the NMOSD-ON group, 64 (62.1%, 64/103) in the MOG-ON group, and 31 (35.2%, 31/88) in the double-seronegative ON group. The results of multivariate regression analysis showed that age [odds ratio (OR) =0.901, 95% confidence interval (CI) 0.854-0.950, P＜0.001] and peak visual acuity (OR=0.311, 95%CI 0.147-0.660, P=0.002) and the involvement of optic nerve length ≥1/2 (OR=3.849, 95%CI 1.083-13.682, P=0.037) were the influencing factors for the complete recovery of visual acuity in the affected eyes of the double antibody negative ON group. Age (OR=0.958, 95%CI 0.933-0.983, P=0.001) was the only influencing factor for the complete recovery of visual acuity in the affected eyes of the MOG-ON group. Peak visual acuity (OR=0.288, 95%CI 0.090-0.927, P=0.037) and optic nerve involvement length ≥1/2 (OR=19.974, 95%CI 1.905-209.559, P=0.013) were the influencing factors for the complete recovery of visual acuity in the affected eyes of the NMOSD-ON group. Age (OR=0.936, 95%CI 0.890-0.983, P=0.009), time from onset to intravenous infusion of methylprednisolone sodium succinate intervention (OR=0.854, 95%CI 0.759-0.961, P=0.009), optic disc edema (OR=4.405, 95%CI 1.108-17.512, P=0.035) and peak visual acuity (OR=0.13, 95%CI 0.046-0.365, P＜0.001) were the influencing factors for the moderate recovery of visual acuity in the affected eyes of the double antibody negative ON group. Peak visual acuity was the only influencing factor for the moderate recovery of visual acuity in the MOG-ON group (OR=0.060, 95%CI 0.010-0.352, P=0.002) and the NMOSD-ON group (OR=0.163, 95%CI 0.053-0.500, P=0.001). ConclusionsThe prognostic factors for visual recovery in patients with DON after glucocorticoid pulse therapy are subtype-specific. Peak visual acuity is a common predictor for all subtypes. For NMOSD-ON and double antibody-negative ON, attention should be paid to the length of optic nerve lesions. MOG-ON is age-related. Early intravenous infusion of methylprednisolone sodium succinate for double antiantibody negative ON is more likely to achieve moderate vision recovery.