Clinical and Radiological Features of Myelin Oligodendrocyte Glycoprotein-Associated Myelitis in Adults

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have recently been established as a biomarker for MOG-antibody-associated disease (MOGAD), which is a distinct demyelinating disease of the central nervous system. Among the diverse clinical phenotypes of MOGAD, myelitis is the second-most-common presentation in adults, followed by optic neuritis. While some features overlap, there are multiple reports of distinctive clinical and radiological features of MOG-IgG-associated myelitis, which are useful for differentiating MOGAD from both multiple sclerosis and neuromyelitis optica spectrum disorder. In this review we summarize the clinical and radiographic characteristics of MOG-IgG-associated myelitis with a particular focus on adult patients.

Associations of Illness Symptoms, Perception of Illness, and Coping with Quality of Life of Thyroid Cancer Patients after Thyroidectomy

Purpose@#To determine associations of illness symptoms, perception of illness, coping with quality of life (QOL) of thyroid cancer patients and identify factors affecting their QOL. @*Methods@#A cross-sectional study was performed using a self-administered questionnaire for 111 thyroid cancer patients after thyroidectomy. They were recruited from the outpatient clinic of one university hospital.Data collection was conducted from August 2018 to November 2018. @*Results@#The QOL was significantly associated with interpersonal coping (β= 0.31, p< .001), monthly household income (β= 0.30, p< .001), illness symptoms (β= -0.22, p= .017), perception of illness (β= -0.20, p= .031), and education (β= 0.18, p= .037) in stepwise multiple regression. These factors explained 33.1% of QOL of thyroid cancer patients. @*Conclusion@#Interpersonal coping is a major contributing factor to QOL. Therefore, thyroid cancer patients need good interpersonal coping for better quality of life.

Trajectories and Prediction Factors of Depression in Elderly Cancer Survivors:Using the Korean Longitudinal Study of Ageing

Purpose@#The purpose of this study was to identify the trajectories of depressive symptoms and the predictive factors that determine the trajectories among elderly cancer survivors in South Korea. @*Methods@#This study was a secondary data analysis using the Korean Longitudinal Study of Ageing (KLoSA). The inclusion criteria were adults who were aged 65 years or older, diagnosed with cancer, and participated in the panel survey at least three times. Data were analyzed using SPSS 25.0 and STATA 16.0 for Group-Based Trajectory Model. @*Results@#This study comprised 197 participants. Depression trajectories were derived into the three groups of ‘nondepression,’ ‘mild depression,’ and ‘depression.’ As a result of multinomial logit analysis using ‘mild depression’ as a reference group, the significant predictors that differentiated the ‘non-depression’ and ‘mild depression’ groups was whether the subject was living alone or not (p=.001). @*Conclusion@#To prevent and manage depression among the elderly cancer survivors living alone, it is necessary to first identify the social support resources from a cancer diagnosis. In addition, a social foundation should be established to enable elderly cancer survivors to utilize the supporting resources. Further studies should be conducted considering disease-specific variables such as types of cancers, stages of cancer, and treatment methods.

Trajectories and Prediction Factors of Depression in Elderly Cancer Survivors:Using the Korean Longitudinal Study of Ageing

Purpose@#The purpose of this study was to identify the trajectories of depressive symptoms and the predictive factors that determine the trajectories among elderly cancer survivors in South Korea. @*Methods@#This study was a secondary data analysis using the Korean Longitudinal Study of Ageing (KLoSA). The inclusion criteria were adults who were aged 65 years or older, diagnosed with cancer, and participated in the panel survey at least three times. Data were analyzed using SPSS 25.0 and STATA 16.0 for Group-Based Trajectory Model. @*Results@#This study comprised 197 participants. Depression trajectories were derived into the three groups of ‘nondepression,’ ‘mild depression,’ and ‘depression.’ As a result of multinomial logit analysis using ‘mild depression’ as a reference group, the significant predictors that differentiated the ‘non-depression’ and ‘mild depression’ groups was whether the subject was living alone or not (p=.001). @*Conclusion@#To prevent and manage depression among the elderly cancer survivors living alone, it is necessary to first identify the social support resources from a cancer diagnosis. In addition, a social foundation should be established to enable elderly cancer survivors to utilize the supporting resources. Further studies should be conducted considering disease-specific variables such as types of cancers, stages of cancer, and treatment methods.

Comparison of Neuropathic Pain in Neuromyelitis Optica Spectrum Disorder and Multiple Sclerosis

3) of the pain in domains of tingling/prickling sensation (p=0.024), mechanical allodynia (p=0.027), sudden pain attacks (p=0.018), and thermal hyperalgesia (p=0.002) were significantly more frequent in NMOSD compared to MS patients. Among the patients experiencing pain with a neuropathic component, total pain-related interference (p=0.045) scores were significantly higher in NMOSD patients than in MS patients. In daily life, pain interfered with normal work (p=0.045) and relationships with other people (p=0.039) more often in NMOSD patients than in MS patients. Although pain medication was prescribed more frequently in NMOSD patients, the percentage of patients experiencing medication-related pain relief was lower in those patients.CONCLUSIONS: The severity of neuropathic pain and the pain-related interference in daily life were greater in NMOSD patients than in MS patients. Individualized analgesic management should be considered based on a comprehensive understanding of neuropathic pain in these patients.

Erratum to: High Seroprevalence and Index of Anti-John-Cunningham Virus Antibodies in Korean Patients with Multiple Sclerosis

Clinical Spectrum of Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G-Associated Disease in Korean Children

Background@#and Purpose: The myelin oligodendrocyte glycoprotein (MOG) antibody is detected at a high rate in childhood acquired demyelinating syndrome (ADS). This study aimed to determine the diagnostic value of the MOG antibody in ADS and the spectrum of MOGantibody-positive demyelinating diseases in children. @*Methods@#This study included 128 patients diagnosed with ADS (n=94) or unexplained encephalitis (n=34). The MOG antibody in serum was tested using an in-house live-cell-based immunofluorescence assay. @*Results@#The MOG antibody was detected in 48 patients (46 ADS patients and 2 encephalitis patients, comprising 23 males and 25 females). Acute disseminated encephalomyelitis (ADEM) (35.4%) was the most-common diagnosis, followed by the unclassified form (17.4%), isolated optic neuritis (ON) (15.2%), neuromyelitis optica spectrum disorder (13.0%), multiple sclerosis (MS) (10.8%), other clinically isolated syndromes [monophasic event except ADEM, isolated ON, or transverse myelitis (TM)] (8.7%), and unexplained encephalitis (4.3%). At the initial presentation, 35 out of the 46 patients with ADS had brain lesions detected in magnetic resonance imaging, and 54% of these 35 patients had encephalopathy. Nine of the 11 patients without brain lesions exhibited only ON. Thirty-nine percent of the patients experienced a multiphasic event during the mean follow-up period of 34.9 months (range 1.4–169.0 months). Encephalopathy at the initial presentation was frequently confirmed in the monophasic group (p= 0.011). @*Conclusions@#MOG antibodies were identified in all pediatric ADS phenotypes except for monophasic TM. Therefore, the MOG antibody test is recommended for all pediatric patients with ADS, especially before a diagnosis of MS and for patients without a clear diagnosis.

Validation of the Korean Version of the 12-Item Multiple Sclerosis Walking Scale and Application to Neuromyelitis Optica Spectrum Disorder

Background@#and PurposeGait problems are a primary complaint in patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). The 12-item Multiple Sclerosis Walking Scale (MSWS-12) is a patient-reported measure assessing the impact of MS on the walking ability. We aimed to adapt and validate the Korean version of the MSWS-12 for the Korean population with MS and NMOSD. @*Methods@#Thirty-four MS and 35 NMOSD patients were recruited. The MSWS-12 questionnaire was translated into the Korean language and evaluated for its validity and reliability in these patients. @*Results@#The MS and NMOSD patients had mean ages of 35.9 and 42.1 years, respectively, median disease durations of 5.6 and 7.2 years, median Expanded Disability Status Scale (EDSS) scores of 2.75 (range, 0–6.5) and 3.5 (range, 0–7.5), and median baseline MSWS-12 total scores of 25 [interquartile range (IQR), 2.60–53.65] and 25 (IQR, 7.29–50.00). The baseline MSWS-12 total score in the patients with MS showed strong correlations with scores for the EDSS, timed 25-foot walk (T25FW), Multiple Sclerosis Impact Scale-29 (MSIS-29) physical dimension, and 36-item Short-Form Health Survey (SF-36) physical component summary (PCS), with Spearman's correlation coefficients (ρ) of 0.922, 0.756, 0.933, and −0.874, respectively. In patients with NMOSD, the baseline MSWS-12 total score showed strong correlations with scores for the EDSS, MSIS-29 physical dimension, and SF-36 PCS (ρ=0.769, 0.910, and −0.852, respectively), and moderate correlations with scores for the T25FW and Fatigue Severity Scale-9 (ρ=0.597 and 0.630, respectively). @*Conclusions@#The Korean version of the MSWS-12 appears to be a valid and reliable scale that can be used for Korean patients with MS. The MSWS-12 can also be applied to patients with NMOSD.

Sleep Related Disorders and Inflammatory Diseases of the Central Nervous System

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are inflammatory diseases of the central nervous system and inflammatory conditions involving sleep-related anatomical structures, associated with sleep-related disorders. After the identification of pathogenic anti-aquaporin-4 antibodies, NMOSD–previously considered a variant of MS–is now regarded as a distinct entity. A higher prevalence of sleep-related disorders is reported in individuals with MS compared to the general population, and recent evidence suggests the same is true in NMOSD. Timely recognition and multidisciplinary therapeutic approaches for sleep-related disorders in individuals with MS or NMOSD improve the patient’s quality of sleep and life. Therefore, this review aims to provide clinicians with a comprehensive understanding of the comorbid conditions and diagnostic and therapeutic strategies in clinical practice.

Sleep Related Disorders and Inflammatory Diseases of the Central Nervous System

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are inflammatory diseases of the central nervous system and inflammatory conditions involving sleep-related anatomical structures, associated with sleep-related disorders. After the identification of pathogenic anti-aquaporin-4 antibodies, NMOSD–previously considered a variant of MS–is now regarded as a distinct entity. A higher prevalence of sleep-related disorders is reported in individuals with MS compared to the general population, and recent evidence suggests the same is true in NMOSD. Timely recognition and multidisciplinary therapeutic approaches for sleep-related disorders in individuals with MS or NMOSD improve the patient’s quality of sleep and life. Therefore, this review aims to provide clinicians with a comprehensive understanding of the comorbid conditions and diagnostic and therapeutic strategies in clinical practice.

Cerebrospinal Fluid CYFRA 21-1 as a Diagnostic Indicator for Leptomeningeal Metastasis in Cancer Patients

BACKGROUND: The early and accurate diagnosis of leptomeningeal metastasis (LM) has become important because of introduction of new therapeutic strategies for LM and increasing incidence of LM along with longer survival of cancer patients. We aimed to evaluate the role of cerebrospinal fluid (CSF) CYFRA 21-1 as a diagnostic indicator for LM in patients with cancer. METHODS: CSF CYFRA 21-1 level was analyzed using electro-chemiluminescent immunoassay. The difference in concentration of CSF CYFRA 21-1 between 91 patients with LM and 339 control groups (patients with other neurological disease or healthy controls) was investigated. The cut-off value of CSF CYFRA 21-1 as a diagnostic indicator for LM and its diagnostic performance were evaluated. RESULTS: The CSF CYFRA 21-1 was significantly higher in LM patients than control groups (p<0.001). A cut-off value of diagnosis for LM in patients with cancer was 1.59 ng/mL. The sensitivity, specificity, accuracy, and positive and negative predictive values of CSF CYFRA 21-1 were 80.2%, 96.2%, 92.8%, 84.9%, 94.8% for diagnosis of LM. CONCLUSIONS: These results suggested that CSF CYFRA 21-1 can be an additional diagnostic indicator for cancer patients with LM.

Real-World Effectiveness of Disease-Modifying Therapies in Korean Patients with Relapsing Multiple Sclerosis

BACKGROUND AND PURPOSE: This study assessed the long-term outcomes of disease-modifying therapies (DMTs) in Korean multiple sclerosis (MS) patients treated in real-world clinical settings in Korea. METHODS: We retrospectively evaluated the medical records of 160 patients with an initial diagnosis of clinically isolated syndrome or relapsing-remitting MS who were treated for at least 2 years. A status of 3 for no evidence of disease activity (NEDA3) was defined as no relapse, disability progression, or active lesions in annual magnetic resonance imaging (MRI) evaluations. RESULTS: Patients who were initially treated with interferon β (n=152), glatiramer acetate (n=6), or teriflunomide (n=2) were included. The mean disease duration was 8.2 years. Compared to pretreatment, annualized relapse rates were significantly reduced after treatment [from 1.0±0.8 to 0.2±0.4 (mean±standard deviation), p < 0.001]. At the follow-up, 79 patients (49%) had changed their treatment regimen due to lack of efficacy (33%), side effects (14%), or other reasons (2%). Disability progression was observed in 18% of the patients over a mean treatment duration of 5.7 years. After 2 years, NEDA3 was observed in 38% of the patients. Loss of NEDA3 at 2 years was associated with long-term disability progression [odds ratio (OR)=17.975, p=0.003]. Poor response to first-line treatment was independently associated with a delay in treatment from disease onset (OR=1.238, p=0.049) and 10 or more brain lesions in the initial MRI (OR=3.648, p=0.047). CONCLUSIONS: This study has provided real-world evidence that DMTs are effective in reducing disease activity and disability progression in Korean MS patients.

High Seroprevalence and Index of Anti-John-Cunningham Virus Antibodies in Korean Patients with Multiple Sclerosis

BACKGROUND AND PURPOSE: The anti-John-Cunningham virus (JCV)-antibody serostatus and index are used in the risk stratification of progressive multifocal leukoencephalopathy (PML) in multiple sclerosis (MS) patients treated with natalizumab. However, little information on these parameters is available for Asian countries. The purpose of this study was to determine the rate of seropositivity, index, and longitudinal index evolution in Korean patients with MS. METHODS: The antibody seroprevalence was analyzed in 355 samples from 187 patients with clinically isolated syndrome or MS using a second-generation, two-step, enzyme-linked immunosorbent assay. A 4-year longitudinal evaluation was applied to 66 patients. RESULTS: The overall antibody seroprevalence was 80% (n=149). Among antibody-positive patients, the index had a median value of 3.27 (interquartile range, 1.52–4.18), with 77% (n=114) and 56% (n=83) of patients having indices >1.5 and >3.0, respectively. The serostatus of 59 (89%) of the 66 patients did not change during the longitudinal analysis, while 3 (6%) of the 53 patients who were initially seropositive reverted to seronegativity, and 2 (15%) of the 13 patients who were initially seronegative converted to seropositivity. All patients with a baseline index >0.9 maintained seropositivity, and 92% of patients with a baseline index >1.5 maintained this index over 4 years. No patients developed PML (median disease duration, 8 years). CONCLUSIONS: The seroprevalence and index of anti-JCV antibodies in Korean patients with MS may be higher than those in Western countries.

Therapeutic Outcome of Alemtuzumab in Korean Patients with Multiple Sclerosis: 2-Year Follow-Up

BACKGROUND AND PURPOSE: Alemtuzumab has shown high efficacy in clinical trials that primarily involved Western multiple sclerosis (MS) patients. To evaluate the therapeutic outcome of alemtuzumab in Korean patients with MS. METHODS: This study enrolled 23 consecutive patients who were treated with alemtuzumab from 2015 to 2018. Efficacy was evaluated using the annualized relapse rate (ARR), Expanded Disability Status Scale (EDSS), and radiological activity. No evidence of disease activity (NEDA) was defined as no clinical relapse, no worsening of the EDSS score, and no radiological activity. The safety profiles were also assessed. RESULTS: The mean age was 36 years and 16 of the patients were female. Seventeen and 12 of 23 patients were followed up for 1 year and 2 years, respectively. The ARR was markedly reduced from 1.52 during the 1-year period preceding alemtuzumab administration to 0.21 after initiating alemtuzumab (p<0.001). During the first and second years after initiating alemtuzumab, EDSS worsening was observed in 3 (18%) and 0 (0%) patients, respectively, and radiological activity was exhibited in 9 (53%) and 4 (33%). NEDA was observed in 6 (35%) patients during the first year and in 8 (67%) patients during the second year. Intriguingly, one patient experienced 2 severe clinical exacerbations, which occurred at 10 months after the first and 10 months after the second infusion of alemtuzumab. Nineteen of the 23 patients exhibited infusion-associated reactions and 3 patients exhibited herpes zoster infection. Thyroid dysfunction occurred in two patients at 18 and 20 months after initiating alemtuzumab. CONCLUSIONS: Consistent with observations in Western populations, alemtuzumab therapy in Korean MS patients led to marked reductions of disease activity without unexpected safety issues.

Neutralizing Antibodies Against Interferon-Beta in Korean Patients with Multiple Sclerosis

BACKGROUND AND PURPOSE: Patients treated with interferon-beta (IFN-β) can develop neutralizing antibodies (NAbs) against IFN-β that can negatively affect the therapeutic response. This study assessed the prevalence of NAbs and the impact of NAb positivity on the therapeutic response to IFN-β in Korean patients with multiple sclerosis (MS). METHODS: This was a multicenter study involving 150 MS patients from 9 Korean medical centers who were treated with IFN-β for at least 6 months. Sera that had not been influenced by acute treatment were assessed for NAbs using a luciferase reporter gene assay. To evaluate the association between persistent positivity for NAbs and disease activity, NAbs were tested at 2 different time points in 75 of the 150 patients. Disease activity was defined as the presence of clinical exacerbations and/or active MRI lesions during a 1-year follow-up after NAb positivity was confirmed. RESULTS: NAbs were found in 39 of the 150 (26%) MS patients: 30 of the 85 (35%) who were treated with subcutaneous IFN-β-1b, 9 of the 60 (15%) who were treated with subcutaneous IFN-β-1a, and 0 of the 5 (0%) who were treated with intramuscular IFN-β-1a. Thirty of the 39 patients exhibiting NAb positivity were tested at different time points, and 20 of them exhibited persistent NAb positivity. Disease activity was observed more frequently in patients with persistent NAb positivity than in those with transient positivity or persistent negativity [16/20 (80%) vs. 4/55 (7%), respectively; p < 0.001]. When disease activity was compared between patients with persistent and transient NAb positivity, the difference was unchanged and remained statistically significant [16/20 (80%) vs. 2/10 (20%), p=0.004]. CONCLUSIONS: These results further support that persistent NAb positivity is associated with disease activity in MS patients treated with IFN-β.

Large-Scale in-House Cell-Based Assay for Evaluating the Serostatus in Patients with Neuromyelitis Optica Spectrum Disorder Based on New Diagnostic Criteria

BACKGROUND AND PURPOSE: The detection of aquaporin 4-IgG (AQP4-IgG) is now a critical diagnostic criterion for neuromyelitis optica spectrum disorder (NMOSD). To evaluate the serostatus of NMOSD patients based on the 2015 new diagnostic criteria using a new in-house cell-based assay (CBA). METHODS: We generated a stable cell line using internal ribosome entry site-containing bicistronic vectors, which allow the simultaneous expression of two proteins (AQP4 and green fluorescent protein) separately from the same RNA transcript. We performed in-house CBA using serum from 386 patients: 178 NMOSD patients diagnosed according to the new diagnostic criteria without AQP4-IgG, 63 high risk NMOSD patients presenting 1 of the 6 core clinical characteristics of NMOSD but not fulfilling dissemination in space, and 145 patients with other neurological diseases, including 66 with multiple sclerosis. The serostatus of 111 definite and high risk NMOSD patients were also tested using a commercial CBA kit with identical serum to evaluate the correlation between the 2 methods. All assays were performed by two independent and blinded investigators. RESULTS: Our in-house assay yielded a specificity of 100% and sensitivities of 80% (142 of 178) and 76% (48 of 63) when detecting definite- and high risk NMOSD patients, respectively. The comparison with the commercial CBA kit revealed a correlation for 102 of the 111 patients: no correlation was present in 7 patients who were seronegative using the commercial method but seropositive using the in-house method, and in 2 patients who were seropositive using the commercial method but seronegative using the in-house method. CONCLUSIONS: These results demonstrate that our in-house CBA is a highly specific and sensitive method for detecting AQP4-IgG in NMOSD patients.

Acute disseminated encephalomyelitis with tremor as the initial symptom

Multiple neurological deficits can occur in acute disseminated encephalomyelitis (ADEM) but rarely movement disorders. A 24-year-old female patient was admitted because of tremor in bothupper limbs. After admission, her mental status progressively declined into coma. Brain magnetic resonance imaging and cerebrospinal fluid analysis were compatible with ADEM. Tests for central nervous system infection and autoimmune diseases were all negative. She was given steroid and IVIG, and the patient fully improved. We report here the first case of adult ADEM, who presented with tremor as the initial neurological manifestation.

Cerebral Cortex Involvement in Neuromyelitis Optica Spectrum Disorder

BACKGROUND AND PURPOSE: Brain lesions involving the cerebral cortex are rarely described in patients with neuromyelitis optica spectrum disorder (NMOSD), in contrast to multiple sclerosis. We investigated cerebral cortex involvement using conventional brain magnetic resonance imaging (MRI) in anti-aquaporin-4 (AQP4)-antibody-positive NMOSD patients. METHODS: The study enrolled 215 NMOSD patients who were seropositive for the anti-AQP4 antibody from 5 referral hospitals, and retrospectively analyzed their demographic, clinical, and MRI findings. Abnormal cerebral cortex lesions on brain MRI were identified by a neuroradiologist and two neurologists using consensus. RESULTS: Most of the 215 enrolled patients (87%) were female. The median age at onset was 22.5 years (range: 15-36 years) and the mean follow-up duration was 123 months. Brain lesions were found in 143 of 194 patients (74%) in whom MRI was performed during follow-up. Brain lesions involving the cerebral cortex were identified in 6 of these 194 patients (3.1%). Five of the patients were female, and the six patients together had a median age of 29 years (range: 15-36 years) at the time of lesion presentation. Three of them showed leptomeningeal enhancement in the lesions. At presentation of the cortex-involving lesions, five of these patients were not being treated at the time of presentation, while the sixth was being treated with interferon-beta. CONCLUSIONS: Although rare, cortical involvement occurs in NMOSD and is commonly combined with leptomeningeal enhancement. We speculate that this occurs only in patients who are not treated appropriately with immunosuppressant drugs.

The Etiological Spectrum of Acute Sensory Myelitis

BACKGROUND AND PURPOSE: Acute myelitis patients exhibiting only sensory deficits upon initial presentation are not commonly encountered in clinical practice, but they definitely exist. Since acute sensory myelitis has not been investigated previously, this study evaluated the etiological spectrum of the condition with the aim of describing the clinical characteristics thereof. METHODS: Patients with acute myelitis who presented at the Ewha Womans University Medical Center (during 1999-2012) and the National Cancer Center (during 2005-2014) with only sensory symptoms as first clinical features were enrolled in this study. Their medical records, electrophysiological and laboratory data, and MRI findings were analyzed retrospectively. RESULTS: Of a total of 341 acute myelitis patients, 52 (15%) were identified as having acute sensory myelitis. The male-to-female ratio of these patients was 35:17, and their age at the onset of the condition was 41.7+/-10.5 years (mean+/-SD; range, 24-72 years). Acute sensory myelitis developed in patients with multiple sclerosis (MS; 14%), neuromyelitis optica spectrum disorder (NMOSD; 17%), and acute myelitis associated with concurrent systemic diseases including Behcet's disease and cancer (6%). Despite detailed evaluation, the etiology of 33 patients with acute myelitis could not be determined. Longitudinally extensive transverse myelitis on spinal MRI and progression of the sensory level were observed most commonly in NMOSD patients (89% and 78%, respectively); however, these patients did not exhibit sensory dissociation. Residual negative sensory symptoms were observed more frequently in NMOSD patients (33%) than in those with acute myelitis of unknown cause (24%) or MS (14%). During the long-term follow-up (4.7+/-2.7 years) of patients who did not undergo maintenance immunotherapy, a monophasic clinical course was common in those with acute myelitis of unknown cause (76%), but not in NMOSD or MS patients. CONCLUSIONS: Accurate identification of the diverse nature of acute sensory myelitis may assist in patient care.

The Systemic Rheumatologic Disease and Neuromyelitis Optica

Neuromyelitis optica (NMO), an autoimmune inflammatory disease of the central nervous system (CNS), is related with autoantibodies for aquaporin-4, which is the most abundant water channel in CNS. The clinical syndromes of NMO, such as longitudinally extensive transverse myelitis and optic neuritis, can occur in the context of systemic rheumatologic diseases, such as systemic lupus erythematosus and Sjogren syndrome. It is debatable as to whether NMO is a feature of genetic tendency toward humoral autoimmunity or a CNS complication of systemic rheumatologic diseases. Current evidence suggests that NMO coexists with systemic rheumatologic diseases rather than a complication from them. Early immunosuppressive therapies should be considered in these patients since just one or two attacks can cause severe neurological disability.