The International Pediatric Multiple Sclerosis Study Group (IPMSSG) put forward the 2007 version of the diagnostic criteria for multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children in 2007 ("2007 version" for short). In 2012, IPMSSG proposed the new diagnostic criteria with reference to the latest research achievements of 150 members ("2012 version" for short). The 2012 version of the consensus statements covers the diagnostic criteria for acute disseminated encephalomyelitis, clinically isolated syndrome, neuromyelitis optica, and multiple sclerosis in children. As the two IPMSSG members in China, the authors give an interpretation of the 2012 version of the consensus statements with reference to related literature and clinical and scientific experience. The authors focus on how the 2012 version comprehensively and thoroughly elaborates on the clinical features, diagnostic criteria, influencing factors, and new ideas of acute demyelinating diseases of the central nervous system in children. These become more operable in clinical diagnosis and treatment of multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children.
Child ; Consensus ; Demyelinating Diseases ; diagnosis ; Encephalomyelitis, Acute Disseminated ; diagnosis ; Humans ; Multiple Sclerosis ; diagnosis ; Neuromyelitis Optica ; diagnosis

Until recently, neuromyelitis optica (NMO) was considered to be a clinical variant of multiple sclerosis (MS). The discovery of disease-specific anti-aquaporin-4 antibody has facilitated the differentiation of NMO from MS and led to the recognition of a broader phenotypic spectrum now referred to as neuromyelitis optica spectrum disorder (NMOSD). However, distinguishing NMOSD from MS remains challenging, as a subgroup of NMOSD patients are found to be seronegative. The ability to differentiate between NMOSD and MS is critical because these conditions have distinct treatments and prognoses, and the disease-modifying treatment for MS can actually aggravate NMOSD. This review focuses on clinically relevant guidance for diagnosing and differentiating between NMOSD and MS.
Diagnosis, Differential* ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis* ; Neuromyelitis Optica* ; Prognosis ; Tomography, Optical Coherence

Neuromyelitis optica spectrum disorder (NMOSD) can present with various symptoms including optic neuritis, transverse myelitis, and area postrema syndrome. However, acute memory loss is an uncommon clinical presentation of NMOSD. We report a patient with NMO-IgG-antibody-positive NMOSD presenting with only acute memory loss, which suggested the presence of bilateral thalamic lesions. This case indicates that NMOSD needs to be considered in the differential diagnosis of acute memory loss.
Area Postrema ; Diagnosis, Differential ; Humans ; Memory Disorders* ; Memory* ; Myelitis, Transverse ; Neuromyelitis Optica* ; Optic Neuritis

To explore the clinical features and influencing factors of first-onset neuromyelitis optica spectrum disease (NMOSD) within 1 year after delivery. A single center, observational cohort study was used to retrospectively analyze 12 patients with first-onset NMOSD within 1 year after delivery hospitalized in the Department of Neurology of Beijing Tong Ren Hospital from June 2015 to June 2018(short as the postpartum onset group). 12 patients with first-onset NMOSD without 1 year after delivery hospitalized in our department during the same period were selected (short as the control group). The results showed the next recurrence interval in the postpartum onset group was longer than the control group [the postpartum onset group: (6.1±3.5) years, the control group: (1.6±1.5) years, t=3.622,P=0.005], the times of relapses were less than the control group [the postpartum onset group: (1.8±1.4) times, the control group:4.0 (3.0, 7.3) times, Z=-3.122,P=0.002], and expanded disability status scale (EDSS) of the last follow-up was lower than the control group [the postpartum onset group: 3.0(2.3, 3.9), the control group: 4.5(4.0, 6.0), Z=-3.358,P=0.001] with statistically significant differences. The recurrence rates of 1 year, 3 years and 5 years in the postpartum onset group (0%, 16.7%, 33.3%) were lower than control group (58.3%, 83.3%, 91.7%) with statistically significant differences (χ²=8.000,P=0.014;χ²=10.667,P=0.003; χ²=8.711,P=0.009). After the second delivery, the recurrence rate in postpartum onset group was 100% (n=3) and in control group was 50%(n=2), but the difference was not statistically significant (χ²=2.100,P=0.429). In the postpartum onset group, combination of autoimmune disease was consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Positive in other autoimmune antibodies were consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Combination of autoimmune disease were consistent with positive in serum other autoimmune antibodies well (Kappa=0.667, P=0.021). In conclusion, the first-onset NMOSD within 1 year after delivery have longer next recurrence interval, less times of relapses, lower relapse rate, better long-term prognosis of central nervous system, and they have trend to suffering from recurrent after the second delivery. For the females, combined with autoimmune disease or autoimmune antibody, who are ready for pregnancy, could detect serum AQP-4; if serum AQP-4 positive, they are recommended to prevent the occurrence of NMOSD after delivery.
Pregnancy ; Female ; Humans ; Neuromyelitis Optica/diagnosis* ; Retrospective Studies ; Cohort Studies ; Postpartum Period ; Recurrence

To explore the clinical features and influencing factors of first-onset neuromyelitis optica spectrum disease (NMOSD) within 1 year after delivery. A single center, observational cohort study was used to retrospectively analyze 12 patients with first-onset NMOSD within 1 year after delivery hospitalized in the Department of Neurology of Beijing Tong Ren Hospital from June 2015 to June 2018(short as the postpartum onset group). 12 patients with first-onset NMOSD without 1 year after delivery hospitalized in our department during the same period were selected (short as the control group). The results showed the next recurrence interval in the postpartum onset group was longer than the control group [the postpartum onset group: (6.1±3.5) years, the control group: (1.6±1.5) years, t=3.622,P=0.005], the times of relapses were less than the control group [the postpartum onset group: (1.8±1.4) times, the control group:4.0 (3.0, 7.3) times, Z=-3.122,P=0.002], and expanded disability status scale (EDSS) of the last follow-up was lower than the control group [the postpartum onset group: 3.0(2.3, 3.9), the control group: 4.5(4.0, 6.0), Z=-3.358,P=0.001] with statistically significant differences. The recurrence rates of 1 year, 3 years and 5 years in the postpartum onset group (0%, 16.7%, 33.3%) were lower than control group (58.3%, 83.3%, 91.7%) with statistically significant differences (χ²=8.000,P=0.014;χ²=10.667,P=0.003; χ²=8.711,P=0.009). After the second delivery, the recurrence rate in postpartum onset group was 100% (n=3) and in control group was 50%(n=2), but the difference was not statistically significant (χ²=2.100,P=0.429). In the postpartum onset group, combination of autoimmune disease was consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Positive in other autoimmune antibodies were consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Combination of autoimmune disease were consistent with positive in serum other autoimmune antibodies well (Kappa=0.667, P=0.021). In conclusion, the first-onset NMOSD within 1 year after delivery have longer next recurrence interval, less times of relapses, lower relapse rate, better long-term prognosis of central nervous system, and they have trend to suffering from recurrent after the second delivery. For the females, combined with autoimmune disease or autoimmune antibody, who are ready for pregnancy, could detect serum AQP-4; if serum AQP-4 positive, they are recommended to prevent the occurrence of NMOSD after delivery.
Pregnancy ; Female ; Humans ; Neuromyelitis Optica/diagnosis* ; Retrospective Studies ; Cohort Studies ; Postpartum Period ; Recurrence

BACKGROUNDAlthough there were criteria for diagnosis of neuromyelitis optica (NMO) and multiple sclerosis (MS), it is still difficult to differentiate NMO from MS, due to the overlapping clinical manifestations. Therefore it is necessary to characterize clinical features of NMO and MS patients in the mainland of China, to simplify the process of disease diagnosis, and to identify criteria for the differential diagnosis of NMO and MS.

METHODSA total of 138 Chinese Han patients from the mainland of China including 73 NMO, 60 MS and 5 MS-like patients with positive NMO-IgG were included in the study. Clinical records were reviewed retrospectively and the results of clinical examination, laboratory experiments, magnetic resonance imaging (MRI) and evoked potentials (EPs) were compared between NMO and MS patients. In addition, the relationship between the NMO-IgG serologic status and clinical characteristics were analyzed.

RESULTSCompared with MS patients (1.3: 1.0), more female prevalence was observed in NMO patients (4.2: 1.0; P = 0.003). There were also statistically significant differences in visual EPs, oligoclonal bands, brainstem lesions in MRI and longitudinally extensive spinal cord lesions (LESCLs) between NMO and MS patients. Brainstem lesions observed in brain MRI were found in 17.9% of MS patients, over 3.7 times higher than in NMO patients (4.8%, P = 0.024). When stratified NMO patients by NMO-IgG, LESCLs were found in 42.1% of NMO-IgG-negative NMO patients, over 3.5 times higher than in NMO-IgG-positive patients (11.9%, P = 0.008). Statistical difference was also observed in CD4+/CD8+ ratios between NMO-IgG-positive and -negative NMO patients.

CONCLUSIONSComprehensive analysis of MRI, laboratory and EPs data can facilitate differential diagnosis of MS and NMO. In addition, the combination of LESCLs and brain MRI findings failing to satisfy MRI criteria for MS is highly sensitive and specific for NMO.

Adult ; China ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multiple Sclerosis ; diagnosis ; Neuromyelitis Optica ; diagnosis ; Retrospective Studies

Neuromyelitis optica (NMO) is considered to be a rarer autoimmune disease than multiple sclerosis. It is very difficult to make a diagnosis of MNO for doctors who are not familiar with its clinical features and diagnostic criteria. We report a case of a young female patient who had been suffering motor weakness and radiating pain in both upper extremities. Cervical MRI showed tumorous lesion in spinal cord and performed surgery to remove lesion. We could not find a tumor mass in operation field and final diagnosis was NMO. NMO must be included in the differential diagnosis of lesions to rescue the patient from invasive surgical interventions. More specific diagnostic tools may be necessary for early diagnosis and proper treatment.
Autoimmune Diseases ; Diagnosis, Differential ; Early Diagnosis ; Female ; Humans ; Multiple Sclerosis ; Neuromyelitis Optica ; Spinal Cord ; Stress, Psychological ; Upper Extremity

Adult ; Female ; Humans ; Magnetic Resonance Imaging ; Methylprednisolone ; therapeutic use ; Neuromyelitis Optica ; diagnosis ; drug therapy ; etiology ; Spondylosis ; complications ; diagnosis ; drug therapy

PURPOSE: The purpose of this study was to demonstrate whether the pattern of optic nerve enhancement in magnetic resonance imaging (MRI) can help to differentiate between idiopathic optic neuritis (ON), neuromyelitis optica (NMO), and multiple sclerosis (MS) in unilateral ON. METHODS: An MRI of the brain and orbits was obtained in patients with acute unilateral ON. Patients with ON were divided into three groups: NMO, MS, and idiopathic ON. The length and location of the abnormal optic nerve enhancement were compared for ON eyes with and without NMO or MS. The correlation between the pattern of optic nerve enhancement and the outcome of visual function was analyzed. RESULTS: Of the 36 patients with ON who underwent an MRI within 2 weeks of the onset, 19 were diagnosed with idiopathic ON, 9 with NMO, and 8 with MS. Enhancement of the optic nerve occurred in 21 patients (58.3%) and was limited to the orbital segment in 12 patients. Neither the length nor the location of the optic nerve enhancement was significantly correlated with visual functions other than contrast sensitivity or the diagnosis of idiopathic ON, MS, or NMO. Patients with greater extent of optic nerve sheath enhancement and more posterior segment involvement showed higher contrast sensitivity. CONCLUSIONS: Our data revealed that the pattern of optic nerve enhancement was not associated with diagnosis of idiopathic ON, NMO, or MS in Korean patients with unilateral ON. We believe further studies that include different ethnic groups will lead to a more definitive answer on this subject.
Brain ; Contrast Sensitivity ; Diagnosis ; Ethnic Groups ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Neuromyelitis Optica ; Optic Nerve* ; Optic Neuritis* ; Orbit

OBJECTIVETo assess the seroprevalence and diagnostic value of aquaporin-4 antibody (AQP4-Ab) in patients with inflammatory central nervous system demyelinating diseases.

METHODSSeventy-two patients with neuromyelitis optica (NMO), 68 with multiple sclerosis (MS), 4 with optic neuritis (ON), and 41 with transverse myelitis (TM) were included in this study. The TM group comprised 19 patients with non-longitudinally extensive transverse myelitis (nLETM), 14 with monophasic longitudinally extensive transverse myelitis (mLETM), and 8 with recurrent longitudinally extensive transverse myelitis (rLETM). The serum levels of AQP4-Ab was detected by indirect immunofluorence assay in these patients.

RESULTSAQP4-Ab was detected in 72.2% (52/72) patients with NMO, 5.9% (4/68) patients with MS, 25.0% (1/4) patients with ON, and 17.1% (7/41) patients with TM, showing a significant difference in the positivity between NMO and MS groups (P<0.01). AQP4-Ab seropositivity rate was 5.3% (1/19) in nLETM patients, 62.5% (5/8) in rLETM patients and 7.1% (1/14) in mLETM patients, significantly higher in rLETM than in nLETM (P<0.01) and mLETM groups (P<0.05), but no statistical difference was found between rLETM and NMO groups.

CONCLUSIONSA high seroprevalence of AQP4-Ab is observed in patients with NMO and rLETM, which support the hypothesis that NMO and rLETM belong to NMO spectrum disorders. AQP4-Ab can serve as a useful index for diagnosing NMO and differential diagnosis from MS. More attention and effective immunosuppressive treatments should be given to patients positive for AQP4-Ab.

Aquaporin 4 ; immunology ; Autoantibodies ; blood ; Demyelinating Autoimmune Diseases, CNS ; diagnosis ; immunology ; Female ; Humans ; Male ; Multiple Sclerosis ; diagnosis ; immunology ; Neuromyelitis Optica ; diagnosis ; immunology ; Seroepidemiologic Studies