Clinical features and tibial nerve somatosensory evoked potential findings in patients with neuromyelitis optica spectrum disorder

2017 ◽  
Vol 381 ◽  
pp. 248-249
Author(s):  
H. Fujii ◽  
K. Kurokawa ◽  
M. Hayashi ◽  
F. Eto ◽  
K. Tachiyama ◽  
...  
2019 ◽  
Vol 27 ◽  
pp. 289-293 ◽  
Author(s):  
Thomas Mathew ◽  
Amrutha Avati ◽  
Delon D’Souza ◽  
Manjusha Therambil ◽  
Anita Angela Baptist ◽  
...  

2019 ◽  
Author(s):  
Arman Cakar ◽  
Canan Ulusoy ◽  
Tuncay Gunduz ◽  
Cem Ismail Kucukali ◽  
Murat Kurtuncu

2017 ◽  
Vol 24 (13) ◽  
pp. 1753-1759 ◽  
Author(s):  
Maria Sepúlveda ◽  
Nuria Sola-Valls ◽  
Domingo Escudero ◽  
Bojan Rojc ◽  
Manuel Barón ◽  
...  

Background: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. Objective: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. Methods: Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG. Paraneoplastic patients were defined as those with cancer identified within 2 years of the diagnosis of NMOSD. Results: Five (3.2%) of 156 patients had paraneoplastic NMOSD, and 12 previously reported patients were identified. The most common tumors were adenocarcinoma of the lung (five patients) and breast (five). Compared with the 151 non-paraneoplastic NMOSD patients, the 17 (5 current cases and 12 previously reported) were older at symptom onset (median age = 55 (range: 17–87) vs 40 (range: 10–77) years; p = 0.006), more frequently male (29.4% vs 6.6%; p = 0.009), and presented with severe nausea and vomiting (41.2% vs 6.6%; p < 0.001). The frequency of longitudinal extensive transverse myelitis (LETM) as heralding symptom was similar in both groups, but patients with paraneoplastic NMOSD were older than those with non-paraneoplastic NMOSD (median age: 63 (range: 48–73) vs 43 (range: 14–74) years; p = 0.001). Conclusion: Patients, predominantly male, with NMOSD and AQP4-IgG should be investigated for an underlying cancer if they present with nausea and vomiting, or LETM after 45 years of age.


2019 ◽  
Vol 7 (20) ◽  
pp. 3433-3436
Author(s):  
Laura Tambunan ◽  
K. Ritarwan ◽  
K. P. Surbakti

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. The symptoms can occur simultaneously or separated by a variable period. NMOSD is associated with serum aquaporin antibodies 4 immunoglobulin G (AQP4-IgG). CASE PRESENTATION: We report a case of a 22-year-old male with complaints of weakness of all four limbs, impaired vision, urinary incontinence, and dyspnea. The Expanded Disability Status Scale (EDSS) was nine. Spinal magnetic resonance imaging (MRI) showed longitudinal extensive transversal myelitis. The brain MRI showed a normal impression, whereas the brain magnetic resonance spectroscopy (MRS) examination showed a description of the mild demyelination process. The serum antibody AQP4 (AQP4-IgG) results were seronegative, the cerebrospinal fluid examination was normal, and the oligoclonal band was negative. The ophthalmoscopic examination found bilateral papillary atrophy but optical coherence tomography (OCT) was still normal. Somatosensory evoked potential and visual evoked potential examinations were abnormal. The patient was diagnosed with NMOSD and was given combination immunosuppressant therapy, corticosteroids, and therapeutic plasma exchange. The patient experienced significant improvement with EDSS decreased to six. CONCLUSION: In the case of relapsing NMOSD patient, combination therapy of immunosuppressants, corticosteroids, and TPE was used. There were significant improvements from EDSS nine to six.


2021 ◽  
Vol 11 ◽  
Author(s):  
Ling Wang ◽  
Kaiqun Liu ◽  
Xiao Tan ◽  
Lin Zhou ◽  
Yuxin Zhang ◽  
...  

Background: To investigate the remedial efficacy and safety of intravenous cyclophosphamide (CP) in the acute phase in patients with neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) who are refractory to intravenous methylprednisolone (MP) treatment.Design: This study was a single-center, retrospective, observational case-control cohort study.Methods: Thirty-six patients who had acute NMOSD-ON attacks and were refractory to MP treatment were included. Patents were divided into two groups: the remedial CP group, and the MP group. The best-corrected visual acuity (BCVA), mean deviation (MD) of the visual field (VF), visual evoked potential amplitude (VEP-A), visual evoked potential latency (VEP-T), and average thickness of the retinal nerve fiber layer (RNFL) at onset, 1 month (m), 3 m, and 6 m after the attack were analyzed. Routine blood test results, liver and kidney function, routine urinalysis results and general condition were analyzed for safety issues at each follow-up. Fisher's exact test, the Mann-Whitney U test, the Kruskal-Wallis test and the Wilcoxon rank-sum test were used for statistical analysis.Results: The remedial CP group showed significant improvement over 6 m with regard to BCVA and MD (P &lt; 0.05),whereas MP group only showed significant improvement in MD (P &lt; 0.05). Regarding remedial CP intervention time window, the CP ≤ 30 days group showed significant improvement over 6 m with regard to BCVA (P = 0.002), MD (P = 0.003), and VEP-A (P = 0.036), while those CP &gt; 30 days group did not. Both two subgroups showed significantly RNFL thickness reduction, however, BCVA, MD, VEP-A, VEP-T, and RNFL thickness showed no significant differences between the two subgroups at any follow-up point (P &gt; 0.05).Conclusion: CP within 30 days of attack onset is safe and might have a beneficial degree of therapeutic efficacy for acute-phase treatment of NMOSD-ON that is refractory to MP treatment alone.


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