Clinical Characteristics of Patients With Neuromyelitis Optica Spectrum Disorders With Early Onset

2019 ◽  
Vol 34 (9) ◽  
pp. 487-490 ◽  
Author(s):  
Yara Dadalti Fragoso ◽  
Nise Alessandra C. Sousa ◽  
Tania Saad ◽  
Soniza Vieira Alves-Leon ◽  
Maria Lucia V. Pimentel ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Medical Records ◽  
Early Onset ◽  
Disease Onset ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Retrospective Assessment ◽  
Initial Symptoms ◽  
Median Period ◽  
Spectrum Disorders

Neuromyelitis optica spectrum disorder is a severe and disabling disease that manifests with severe relapses of optic neuritis, longitudinally extensive myelitis, and/or brainstem syndromes. The disease is complex and, although onset typically occurs in middle age, children and adolescents may be affected. The present study adds to the literature through detailed clinical data from 36 Brazilian patients with neuromyelitis optica spectrum disorder starting before age 21. This was a retrospective assessment of medical records from 14 specialized units in Brazil. The results showed that the course of neuromyelitis optica spectrum disorder was worse in patients with disease onset before the age of 12 years. Gender and ethnic background did not influence disability accumulation. Over a median period of 8 years, 14% of the patients who presented the initial symptoms of neuromyelitis optica spectrum disorder before the age of 21 years died. In conclusion, the present study adds to the reports from other authors examining the severity of early-onset neuromyelitis optica spectrum disorder.

Wasted tongue in neuromyelitis optica spectrum disorders due to hypoglossal nerve involvement

2014 ◽  
Vol 21 (4) ◽  
pp. 488-491 ◽  
Author(s):  
Shanthi Viswanathan
Keyword(s):  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Hypoglossal Nerve ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Nerve Involvement ◽  
Spectrum Disorders

We report two cases of neuromyelitis optica spectrum disorder with hypoglossal nerve involvement resulting in a wasted tongue associated with other brainstem symptoms of hypogeusia, hypersalivation, hiccough, increased sweating, hyperemesis and myelitis (in the second patient). This occurred due to involvement of the hypoglossal, tractus solitarius and dorsal vagal nuclei. Though the myelitis and other brainstem signs recovered the hypoglossal nerve involvement resulting in a unilateral wasted tongue did not. It is important to consider neuromyelitis optica and its spectrum disorders in the differential diagnosis of a wasted tongue though its occurrence is rare.

Treatment options in Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorders

2021 ◽  
Vol 27 ◽  
Author(s):  
Paulus Rommer ◽  
Uwe K Zettl
Keyword(s):  
Multiple Sclerosis ◽  
Side Effects ◽  
Neuromyelitis Optica ◽  
Treatment Options ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
The Past ◽  
Drug Classes ◽  
Spectrum Disorders

: There are few diseases with as many therapeutic advances in recent years as in multiple sclerosis. Nine different drug classes with more than a dozen approved therapies are now available. Similarly, there have been unimaginable advances in understanding neuromyelitis optica (now neuromyelitis optica spectrum disorder [NMOSD]) over the past 15 years. Building on the knowledge gained, the first therapies have been approved in recent years. In this review, we aim to present all therapies approved for the treatment of MS or NMOSD. The different forms of application, different approval criteria and most important side effects will be presented. This work is intended for physicians who are interested in MS and NMOSD therapies and want to get a first overview and does not replace the respective guidelines of the regulatory authorities.

scholarly journals Gender effect on neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G

2016 ◽  
Vol 23 (8) ◽  
pp. 1104-1111 ◽  
Author(s):  
Sung-Min Kim ◽  
Patrick Waters ◽  
Mark Woodhall ◽  
Yoo-Jin Kim ◽  
Jin-Ah Kim ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Immunoglobulin G ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Spectrum Disorder ◽  
Pattern Reversal ◽  
Neuromyelitis Optica Spectrum Disorder

Background: Neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G (NMOSD-AQP4) is an inflammatory disease characterised by a high female predominance. However, the effect of gender in patients with NMOSD-AQP4 has not been fully evaluated. Objective: The aim of this study was to determine the effect of gender in clinical manifestations and prognosis of patients with NMOSD-AQP4. Methods: The demographics, clinical and radiological characteristics, pattern reversal visual evoked potential (VEP) test results, and prognosis of 102 patients (18 males) with NMOSD-AQP4 were assessed. Results: Male patients had a higher age at onset (48.7 vs 41 years, p = 0.037) and less optic neuritis as the onset attack (17% vs 44%, p = 0.026), higher tendency to manifest as isolated myelitis over the follow-up period (67% vs 28%, p = 0.005), fewer optic neuritis attacks per year (0.08 vs 0.27, p < 0.001), and shorter relative P100 latency on VEP testing (97.1% vs 108.3%, p = 0.001). Moreover, male gender was significantly associated with the absence of optic neuritis attacks over the follow-up period independent of their age of onset. Conclusion: In NMOSD-AQP4 patients, gender impacts on disease onset age and site of attack. This may be an important clue in identifying NMOSD-AQP4 patients with limited manifestations as well as in predicting their clinical courses.

Cognitive impairment differs between neuromyelitis optica spectrum disorder and multiple sclerosis

2016 ◽  
Vol 22 (14) ◽  
pp. 1850-1858 ◽  
Author(s):  
Su-Hyun Kim ◽  
Kichang Kwak ◽  
In Hye Jeong ◽  
Jae-Won Hyun ◽  
Hyo-Jin Jo ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Impairment ◽  
Neuromyelitis Optica ◽  
Visual Memory ◽  
Verbal Learning ◽  
Disease Onset ◽  
Negative Influence ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Memory Tests

Objective: To compare the frequency and pattern of cognitive impairment (CI) between patients with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). Methods: A total of 82 NMOSD patients, 58 MS patients, and 45 healthy controls (HCs) underwent a neuropsychological assessment. Results: CI was observed in 29% of NMOSD and 50% of MS patients ( p < 0.001); CI was considered present if a patient scored lower than the fifth percentile compared with HCs in at least three domains. A lower frequency of CI was consistently found when CI was indicated by at least two failed tests ( p < 0.001). MS patients performed worse than did NMOSD patients on verbal learning and verbal and visual memory tests. Levels of education and depression and the interval from disease onset to treatment were associated with a negative influence on cognition in patients with NMOSD. Conclusion: CI in patients with NMOSD may be not as common as in patients with MS. MS patients exhibited severe impairment, particularly on learning and memory tests, compared with NMOSD patients. Differential prevalence and patterns of CI between NMOSD and MS patients suggest that the two diseases have different mechanisms of brain injury.

Neuromyelitis optica spectrum disorder mimicking extensive leukodystrophy

2018 ◽  
Vol 24 (9) ◽  
pp. 1256-1258 ◽  
Author(s):  
Jonathan Ciron ◽  
Olivier Colin ◽  
Marie-Pierre Rosier ◽  
Soline Lapeyrie ◽  
Damien Biotti ◽  
...  
Keyword(s):  
White Matter ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
White Matter Lesions ◽  
Brain Lesions ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Spectrum Disorders

Brain MRI was originally considered to appear normal in neuromyelitis optica spectrum disorders (NMO-SD). Typical brain lesions are now well described and have been integrated in the latest revision of NMO-SD criteria, but the NMO-SD MRI pattern remains not yet comprehensive. We report here extensive white matter lesions (EWML) mimicking leukodystrophy in a 50-year-old woman with long-lasting anti-AQP4+ NMO-SD. We suggest that EWML could be a possible brain MRI presentation of NMO-SD patients.

scholarly journals Aquaporin-4 serostatus does not predict response to immunotherapy in neuromyelitis optica spectrum disorders

2017 ◽  
Vol 24 (13) ◽  
pp. 1737-1742 ◽  
Author(s):  
Maureen A Mealy ◽  
Su-Hyun Kim ◽  
Felix Schmidt ◽  
Reydmar López ◽  
Jorge A Jimenez Arango ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Neuromyelitis Optica ◽  
Immunosuppressive Treatment ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Immune Mediated ◽  
Spectrum Disorders ◽  
Pre Treatment ◽  
Relapse Rates

Background: Debate exists about whether neuromyelitis optica spectrum disorder seronegative disease represents the same immune-mediated attack on astrocytic aquaporin-4 as in seropositive disease. Objective: We investigated whether response to common treatments for neuromyelitis optica spectrum disorder differed by serostatus, as assessed by change in annualized relapse rate. Methods: We performed a multicenter retrospective analysis of 245 patients with neuromyelitis optica spectrum disorder who were treated with either rituximab or mycophenolate mofetil as their first-line immunosuppressive treatment for disease prevention. Patients were followed for a minimum of 6 months following treatment initiation. Results: In those started on rituximab, the pre-treatment annualized relapse rates for seropositive and seronegative patients were 1.81 and 1.93, respectively. On-treatment annualized relapse rates significantly declined to 0.32 (seropositive; p < 0.0001) and 0.12 (seronegative; p = 0.0001). In those started on mycophenolate mofetil, the pre-treatment annualized relapse rates for seropositive and seronegative patients were 1.79 and 1.45, respectively. On-treatment annualized relapse rates declined to 0.29 (seropositive; p < 0.0001) and 0.30 (seronegative; p < 0.005). Conclusion: In this international collaboration involving a large number of neuromyelitis optica spectrum disorder patients, treatment was effective regardless of serostatus. This suggests that treatment should not differ when considering these treatments.

Meningoencephalitis as an initial manifestation of neuromyelitis optica spectrum disorder

2012 ◽  
Vol 19 (5) ◽  
pp. 639-643 ◽  
Author(s):  
Jin-Ye Wang ◽  
Kai Wang ◽  
Xian-Wen Chen ◽  
Jian-Wen Wang ◽  
Kan Zhang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Brain Lesions ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Mri Imaging ◽  
Resonance Imaging ◽  
Initial Manifestation ◽  
Disturbance Of Consciousness ◽  
Initial Symptoms ◽  
Magnetic Resonance Imaging Mri

Two patients presented with initial symptoms of headache and fever, and two weeks later had disturbance of consciousness. Cerebrospinal fluid (CSF) showed pleocytosis >500×106/L. Magnetic resonance imaging (MRI) showed multiple brain lesions at sites of high aquaporin-4 (AQP-4) expression. Case 1 presented optic neuritis four years after the first attack and case 2 had symptoms of myelitis three weeks after headache. Serum AQP-4 antibody was positive in both cases, and the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) was made. Accordingly, NMOSD can initially present as meningoencephalitis mimicking intracranial infection, and the characteristic MRI imaging is quite critical for differentiation.

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