Clinical Study Of Neuromyelitis Optica Spectrum Disorders

Objective To investigate the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSDs) and compare with multiple sclerosis (MS).Methods Retrospectively review the patients with central nervous system (CNS) demyelinating disease followed at outpatient and inpatient department偶of neurology of General Hospital of PLA from November 2008 to March 2011. The clinical characteristics were compared between NMOSDs and MS.Results A total of 356 patients were enrolled, including 72 with CIS,132 with MS,108 with NMO,8 with RON and 36 with rLETM. The ratios of female to male of the patients with NMO, NMOSDs and MS were 12.5:1,6.6:1 and 1.64:1, respectively, and the average ages of onset were 36.9,37.2 and 32.7 years old, respectively. About 20.5% of MS patients and 14.5% of NMOSDs patients existed potential precursor factors before the first attack. The initial symptoms of NMO patients were common to optic nerve lesions, however, those of brain parenchymal lesions were more common in MS patients. The seroprevalences of AQP4-Ab and ANAs in the group of NMO, rLETM and RON were significantly higher than those of MS group (P<0.05). There is no significant differences of ratios of CSF abnormality and pleocytosis among NMO, rLETM and MS patients (P>0.05), and 61.9% of NMO patients have brain abnormalities.Conclusions Compare to MS, NMO and NMOSDs predominantly affect females. The seroprevalences of AQP4-Ab and ANAs in the group of NMO, rLETM and RON were significantly higher than those of MS group, supporting the hypothesis that NMO, rLETM and RON are NMOSDs. Most NMOSDs and MS patients had no obvious precursor factors. CSF detection was of limited value in the diagnosis and differential diagnosis between NMOSDs and MS. A considerable proportion of NMO patients had brain abnormalities. Objective To analyze the MRI characteristics of spinal lesions and brain abnormalities in patients with NMO.Methods We retrospectively reviewed the MRI films of 108 patients with NMO. All patients underwent routine MRI scans of the spinal cord,91 of which underwent enhanced scans. Eight-four patients underwent routine MRI scans of the brain, and 33 of which underwent enhanced scans.Results On MRI,34 (31.5%) cervical cords alone,45 (41.7%) combined cervical and thoracic cords,29 (26.9%) thoracic cords alone were involved. Diffuse lesions, combined linear and diffuse, and linear lesions alone were observed sagittally in 68 (63.0%),31 (28.7%) and 9 (8.3%) patients, respectively. Majority in centered distribution and minority in decentered distribution were observed axially. Enhancement was found in 41 (41/91,40.8%) patients. Fifty-two of 84 patients (61.9%) had brain abnormalities,40 (40/52,76.9%) patients presented with supratentorial lesions (mostly juxtacortical/subcortical/deep white matter, n=21),21(21/52,40.4%) presented with infratentorial lesions (mostly preiaqueduct-fourth ventricular-central canal, n=14), and 9 (9/52,17.3%) had supra-and infratentorial lesion simultaneously.Conclusions:MRI presentation of NMO is complicated. LETM is the most prominent feature of spinal cord lesions of NMO, and a considerable proportion of NMO patients had brain abnormalities. Preiaqueduct-third ventricular-fourth ventricular-central canal lesions, which localized at sites of high aquaporin 4 expression may be unique. Objective To determine seroprevalence and diagnostic value of aquaporin-4 antibody (AQP4-Ab) in Chinese patients with central nervous system inflammatory demyelinating diseases.Methods Total 258 patients with neuromyelitis optica (NMO, n=88), multiple sclerosis (MS, n=94), recurrent optic neuritis (ON, n=8), transverse myelitis (TM, n=68), including monophasic longitudinally extensive transverse myelitis (mLETM, n=15), recurrent longitudinally extensive transverse myelitis (rLETM, n=29), non-longitudinally extensive transverse myelitis (nLETM, n=24) were included in our study. AQP4-Ab was detected by indirect immunofluorence.Results AQP4-Ab were detectable in 61.4%(54/88) patients with NMO, in 5.3%(5/94) patients with MS, in 50.0%(4/8) patients with ON, and in 20.6% (14/68) patients with TM. There was significant difference between NMO and MS (P<0.01). The seroprevalence of AQP4-Ab in rLETM, mLETM, and nLETM patients was 41.4%(12/29),6.7%(1/15), and 4.2%(1/24), respectively, and the seroprevalence of AQP4-Ab in rLETM patients was significantly higher than in mLETM and nLETM patients. There were no significant differences of AQP4-Ab seroprevalence among the rLETM, RON and NMO group patients.Conclusions Seroprevalence of AQP4-Ab in NMO patients was significantly higher than in MS patients, AQP4-Ab is valuable for diagnosing NMO and differentiating it from MS. A high seroprevalence of AQP4-Ab was also observed in patients with RON and rLETM, supporting the hypothesis that both RON, rLETM and NMO are distinct from MS and are NMOSDs. Objective The goal of this study was to investigate the distribution and diagnostic value of ANAs in patients with NMOSDs and MS.Methods Eighty-one patients with NMOSDs and 49 with MS were screened for serum ANAs and enrolled in the study. The NMOSD group included 53 patients with NMO,20 with rLETM, and 8 with RON. Seroprevalence of ANAs was analyzed. An indirect immunofluorescence assay was used for detection of ANA and ACA; a dot-immunogold filtration assay was used to detect anti-dsDNA antibody; and enzyme-linked immunoassay dot technique was used for the detection of anti-ENA polypeptide antibody spectrum.Results Seroprevalence of ANAs in NMOSD patients was 44.4%(36/81), of which the seroprevalence of ANA, anti-dsDNA, anti-centromere antibody (ACA), anti-SSA and anti-SSB were 35.8%,6.2%,1.2%,24.7% and 8.6%, respectively. In the MS group, only 1 patient showed seropositive ANAs at a rate of 2.0%. Significant difference was noted between the 2 groups (P<0.01). The sensitivity of serum ANAs for diagnosing NMOSDs was 44.4%, and the specificity was up to 98.0%. The seroprevalence of ANAs in NMO, rLETM and RON patients was 47.2%,40.0% and 37.5%, respectively, and the difference was not statistically significant (P>0.05).Conclusion Seroprevalence of ANAs in NMO, rLETM and RON patients was significantly higher than in MS patients, thereby supporting the hypothesis that both NMO, rLETM and RON are distinct from MS and are NMOSDs. ANAs detection helps to diagnose NMOSDs and distinguish them from MS. Objective To investigate the diagnosis value of combined testing of AQP4-Ab and ANAs in patients with neuromyelitis optica spectrum disorders NMOSDs and MS.Methods Sixty-five patients with NMOSDs and 45 with MS were screened for serum AQP4-Ab and ANAs simultaneously and enrolled in the study. The NMOSD group included 42patients with NMO,15 with rLETM, and 8 with RON. Serum samples were collected and sent to the laboratory of Rheumatology Department of the PLA General Hospital for ANAs screening and to the Medical Diagnosis Institute, Berlin, Germany for AQP4-Ab screening in a blinded clinical diagnosis. Sensitivity and specificity of AQP4-Ab, ANAs, combined both AQP4-Ab and ANAs in diagnosis of NMOSDs was calculated and analyzed.Results Seroprevalences of AQP4-Ab in NMOSDs and MS patients were 55.4% and 4.4%, respectively. Those of ANAs were 49.2% and 2.2%, and of combined both AQP4-Ab and ANAs were 72.3% and 6.7%. The sensitivity and specificity of AQP4-Ab in diagnosis of NMOSDs were 55.4% and 95.6%, respectively. Those of ANAs were 49.2% and 97.8%, and of combined both AQP4-Ab and ANAs were 72.3% and 93.3%. The sensitivity of parallel test of AQP4-Ab and ANAs in diagnosis of NMOSDs was significantly higher than that of detection of AQP4-Ab (P＜0.05) or ANAs (P＜0.01) separately.Conclusion Combined testing of AQP4-Ab and ANAs can improve the sensitivity in diagnosis of NMOSDs and is valuable for early diagnosis and treatment of NMOSDs.