scholarly journals Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies

2021 ◽  
Vol 8 (2) ◽  
pp. e947 ◽  
Author(s):  
Su-Hyun Kim ◽  
Hyunmin Jang ◽  
Na Young Park ◽  
Yeseul Kim ◽  
So-Yeon Kim ◽  
...  
Keyword(s):  
Immunosuppressive Therapy ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Sustained Remission ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Severe Attack ◽  
Remission Period ◽  
Significant Disability ◽  
History Of ◽  
Median Interval

ObjectiveTo evaluate the outcomes of immunosuppressive therapy (IST) discontinuation in patients with neuromyelitis optica spectrum disorder (NMOSD) after a sustained remission period.MethodsWe retrospectively reviewed the medical records of 17 patients with antiaquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period of ≥3 years.ResultsIST was discontinued at a median age of 40 years (interquartile range [IQR], 32–51) after a median relapse-free period of 62 months (IQR, 52–73). Among the 17 enrolled patients, 14 (82%) relapsed at a median interval of 6 months (IQR, 4–34) after IST discontinuation, 3 (18%) of whom experienced severe attacks; notably, all 3 of these patients had a history of severe attack before IST. These 3 patients received steroids, followed by plasma exchange for acute treatment, but 2 exhibited poor recovery and significant disability worsening at 6 months after relapse.ConclusionsIST discontinuation may increase the risk of relapse in seropositive patients with NMOSD even after 5 years of remission. Given the potentially devastating consequence of a single attack of NMOSD, caution is advised with IST discontinuation, particularly in patients with severe attack before IST.

‘Twenty syndrome’ in neuromyelitis optica spectrum disorder

2020 ◽  
Vol 13 (9) ◽  
pp. e234450
Author(s):  
Ritwik Ghosh ◽  
Souvik Dubey ◽  
Subhankar Chatterjee ◽  
Durjoy Lahiri
Keyword(s):  
Optic Neuritis ◽  
Immunosuppressive Therapy ◽  
Neuromyelitis Optica ◽  
Cranial Nerve ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Optic Nerves ◽  
Seventh Cranial Nerve ◽  
Nerve Paresis ◽  
Pons Medulla

A 30-year-old woman presented with recurrent hiccups, vomiting and painful diminution of vision and gait instability for 1 day. She had one-and-a-half syndrome, bilateral seventh cranial nerve paresis with bilateral symptomatic optic neuritis and left-sided ataxic haemiparesis. We described her disorder as the ‘twenty syndrome’ (11/2+7+7+2+2+½=20). MRI of her brain revealed demyelination predominantly in right posterolateral aspect of pons, medulla and bilateral optic nerves. Serum antiaquaporin-4 antibody came out positive. Thus, she was diagnosed as neuromyelitis optica spectrum disorder (NMOSD). She responded brilliantly to immunosuppressive therapy. This is the first ever reported case of the ‘twenty syndrome’ secondary to cerebral NMOSD.

scholarly journals A Rare Case of Neuromyelitis Optica Spectrum Disorder in Patient with Sjogren’s Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Supat Thongpooswan ◽  
Bikash Chapagain ◽  
Sabiha Bandagi
Keyword(s):  
Sjögren’S Syndrome ◽  
Neuromyelitis Optica ◽  
Sjögren's Syndrome ◽  
Oral Prednisone ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
History Of ◽  
Sjögren‘S Syndrome

We report a 48-year-old female with the history of Sjogren’s syndrome who presented with 3-week history of tingling, numbness, and shooting back, waist, and bilateral leg pain and numbness in the pelvic region with urinary and bowel incontinence. Physical examination was remarkable for reduced motor power in both lower extremities with spasticity. Sensory deficit was noted at the T6 level. Laboratory investigation revealed elevated ESR and CRP and positive serum antiaquaporin-4 IgG. Thoracic and lumbar magnetic resonance imaging revealed abnormal patchy areas, leptomeningeal enhancement through the thoracic cord extending from T3 through T6 levels, without evidence of cord compression. Impression of neuromyelitis optica spectrum disorder was made and patient was treated with methylprednisolone intravenously followed by tapering oral prednisone. Neurological symptoms gradually improved with resolution of bowel and urinary incontinence. In a patient with Sjogren’s syndrome who presents with neurological complaints, the possibility of neuromyelitis optica or neuromyelitis optica spectrum disorder should be considered. Awareness of the possibility of CNS disease is important due to the serious nature of CNS complications, some of which are treatable with immunosuppressants. Our patient with Sjogren’s syndrome who presented with myelopathy benefited from early recognition and institution of appropriate therapy.

Severe Relapse After Cessation of Immunosuppressive Therapy in a Patient With Neuromyelitis Optica Spectrum Disorder

2016 ◽  
Vol 21 (6) ◽  
pp. 97-98 ◽  
Author(s):  
Jae-Won Hyun ◽  
Gayoung Kim ◽  
Yeseul Kim ◽  
In Hye Jeong ◽  
Su-Hyun Kim ◽  
...  
Keyword(s):  
Immunosuppressive Therapy ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

scholarly journals Neuromyelitis optica spectrum disorder presenting in an octogenarian

2018 ◽  
pp. bcr-2018-225601 ◽  
Author(s):  
Amy Campbell ◽  
Olayinka Ayodele Ogundipe
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Age Of Onset ◽  
Index Patient ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Spastic Paraplegia ◽  
History Of

This case describes an 81-year-old woman with a history of Sjögren’s syndrome presenting with recurrent falls and poor balance. She subsequently developed new and rapidly evolving neurology including hyperaesthesia, spastic paraplegia and sphincteric dysfunction. Following serial clinical reviews and detailed investigations, MRI (brainstem and cervicothoracic spine) and a seropositive result for aquaporin 4 IgG, a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) was made. This case describes the clinical course of this index patient with an unusual late age of onset. The report also includes a discussion on NMOSD. We review aspects of terminology, brief epidemiology, pathogenesis, notable autoimmune associations, variance in clinical presentation and current diagnostic criteria. We also review the importance of distinguishing NMOSD from multiple sclerosis in view of the significant implications for treatment and prognosis.

scholarly journals Coexistence of myasthenia gravis and neuromyelitis optica spectrum disorder: case report in Colombia and literature review

2020 ◽  
Vol 10 (3) ◽  
pp. 109-112
Author(s):  
Natalia Gomez
Keyword(s):  
Myasthenia Gravis ◽  
Latin American ◽  
Neuromyelitis Optica ◽  
Clinical Presentation ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Immunological Mechanisms ◽  
Latin American Region ◽  
History Of

Myasthenia Gravis (MG) and Neuromyelitis Optica Spectrum Disorder (NMOSD) are antibody-mediated channelopathies caused by complex immunological mechanisms, which are thus associated with multiple autoimmune diseases. This paper describes the case of a Colombian male patient with a history of seropositive MG that required thymectomy. After four years, he had an Optic Neuritis event and 30 years later, due to suspected autoimmune comorbidities, further investigations were performed, confirming the coexistence of NMOSD with presence of Anti-Aquaporin 4 (AQP4) antibodies (Abs). Several reports in the literature show that the coexistence of these two pathologies is more common than expected by chance. Few cases have been reported in Latin America, which makes this report meaningful. In contrast to our case, the literature shows a higher incidence among women, but, on the other hand, it is consistent with an onset with mild or moderate MG symptoms at the clinical presentation, most of which led to thymectomy, followed by manifestations of NMOSD. Timely diagnosis and the consequent therapeutic adjustment may have a significant impact on the disability of these patients. There is a need for more reports in order to extend the statistical information and to improve the characterization of these patients, primarily in our Latin American region.

Sign in / Sign up

Export Citation Format

Share Document