Endocrinopathies in paediatric-onset Aquaporin 4 (AQP4) antibody neuromyelitis optica spectrum disorder

2017 ◽  
Vol 21 ◽  
pp. e7
Author(s):  
Yael Hacohen ◽  
Silvia Messina ◽  
Hoong-Wei Gan ◽  
Sukhvir Wright ◽  
Saleel Chandratre ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Aqp4 Antibody

scholarly journals Endocrinopathies in paediatric-onset neuromyelitis optica spectrum disorder with aquaporin 4 (AQP4) antibody

2017 ◽  
Vol 24 (5) ◽  
pp. 679-684 ◽  
Author(s):  
Yael Hacohen ◽  
Silvia Messina ◽  
Hoong-Wei Gan ◽  
Sukhvir Wright ◽  
Saleel Chandratre ◽  
...  
Keyword(s):  
Morbid Obesity ◽  
Young People ◽  
Weight Management ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Central Hypothyroidism ◽  
Aquaporin 4 Antibody ◽  
Aqp4 Antibody

The involvement of the diencephalic regions in neuromyelitis optica spectrum disorder (NMOSD) may lead to endocrinopathies. In this study, we identified the following endocrinopathies in 60% (15/25) of young people with paediatric-onset aquaporin 4-Antibody (AQP4-Ab) NMOSD: morbid obesity ( n = 8), hyperinsulinaemia ( n = 5), hyperandrogenism ( n = 5), amenorrhoea ( n = 5), hyponatraemia ( n = 4), short stature ( n = 3) and central hypothyroidism ( n = 2) irrespective of hypothalamic lesions. Morbid obesity was seen in 88% (7/8) of children of Caribbean origin. As endocrinopathies were prevalent in the majority of paediatric-onset AQP4-Ab NMOSD, endocrine surveillance and in particular early aggressive weight management is required for patients with AQP4-Ab NMOSD.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

2011 ◽  
Vol 18 (2) ◽  
pp. 244-247 ◽  
Author(s):  
Sung-Min Kim ◽  
Ji-Soo Kim ◽  
Young Eun Heo ◽  
Hye-Ran Yang ◽  
Kyung Seok Park
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Ocular Manifestation ◽  
Initial Manifestation ◽  
Ocular Symptoms ◽  
Head Tremor ◽  
Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

scholarly journals Anti-myelin Oligodendrocyte Glycoprotein in Aquaporin-4 Negative Neuromyelitis Optica Spectrum Disorder

2021 ◽  
Vol 7 (1) ◽  
pp. 10-16
Author(s):  
Vahid Shaygannejad ◽  
◽  
Mahdi Barzegar ◽  
Navid Manouchehri ◽  
Nafiseh Esmaeil ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Cross Sectional Study ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
University Hospital ◽  
Control Group ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Cross Sectional ◽  
Healthy Control

Background: The absence of Aquaporin-4 Antibody (AQP4-Ab) in a fraction of the Neuromyelitis Optica Spectrum Disorder (NMOSD) patients has led to a search for other serologic markers. Myelin Oligodendrocyte Glycoprotein (MOG) is a protein component of the myelin sheets encapsulating the neural fibers. Objectives: We aimed to compare the presence and levels of anti-MOG (Ig-G) in a group of seronegative NMOSD patients with a healthy control group. Materials & Methods: In this cross-sectional study, 30 NMOSD patients with negative AQP-Ab status, who were referred to the Neurology Clinic of Kashani University Hospital in Isfahan City, Iran, from March 2015 to March 2016, and 26 healthy controls were consecutively recruited. Their baseline demographic and clinical data were recorded. Serum anti-MOG levels were measured in both groups. The obtained data were analyzed using the Student t-test, Mann-Whitney U, and Chisquare test in SPSS V. 18. Results: The anti-MOG test results were statistically higher in patients (n=12, 37.5%) compared to controls (n=0, 0%) (P<0.0001). The level of anti-MOG in Healthy Control (HC) was higher compared to patients with negative anti-MOG (P<0.0001) and was lower than patients with positive anti-MOG (P<0.0001). Conclusion: Our study showed that nearly one-third of seronegative NMOSD patients were positive for MOG-Ab. Further studies are needed to assess the characteristics and outcome of these patients.

scholarly journals Neuromyelitis optica spectrum disorder in pediatrics. Case report

Case reports ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. 11-18
Author(s):  
Jhon Camacho ◽  
Sebastian Zuleta ◽  
Maria Paula Alba ◽  
Andrea Hernandez ◽  
Carlos Navas
Keyword(s):  
Neuromyelitis Optica ◽  
Interesting Case ◽  
Spectrum Disorder ◽  
Demyelinating Diseases ◽  
Inflammatory Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System ◽  
High Doses ◽  
Aqp4 Antibody ◽  
Timely Treatment

Introduction: Neuromyelitis optica is an inflammatory disorder of the central nervous system that accounts for 5% of demyelinating diseases in pediatrics. Its clinical presentation is variable and associated to the involved area of the central nervous system.Case presentation: This is the case of a 15-year-old patient who consulted several times for nonspecific neurological symptoms. During his last visit to the Clínica Universitaria Colombia in Bogotá, he presented with bilateral optic neuritis, associated with frontal and parietal headache. Immunophenotyping studies were carried out, reporting positive IgG anti-aquaporin 4 antibodies (anti-AQP4 antibody), thus leading to a diagnosis of seropositive neuromyelitis optica spectrum disorder (NMOSD). Management with methylprednisolone pulses was initiated with subsequent outpatient management with rituximab that allowed stabilizing the disease.Discussion: This is an interesting case due to its insidious and uncertain onset in a pediatric patient. It was possible to evaluate clinical and diagnostic differences in relation to its presentation in adults. NMOSD mediated by anti-AQP4 is rare; brain and bone marrow MRI are essential for diagnosis. The treatment of choice for acute conditions consists of high doses of methylprednisolone.Conclusion: This disorder may result in irreversible neurological damage; for this reason, high suspicion is required for early diagnosis and timely treatment.

Eculizumab in the treatment of neuromyelitis optica spectrum disorder

Immunotherapy ◽  
2020 ◽  
Vol 12 (14) ◽  
pp. 1053-1066
Author(s):  
Katrin Giglhuber ◽  
Achim Berthele
Keyword(s):  
Neuromyelitis Optica ◽  
Controlled Trial ◽  
Water Channel ◽  
Spectrum Disorder ◽  
Phase Iii ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Humanized Monoclonal Antibody ◽  
The Complement System ◽  
Terminal Complement ◽  
Aqp4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the CNS which is distinct from multiple sclerosis and typically presents with a relapsing course of optic neuritis, myelitis and midline brain inflammatory lesions. In at least two-thirds of cases, antibodies against the water channel AQP4 can be found, which lead to an antibody-mediated activation of the complement system with consecutive damage to neuronal structures. Eculizumab, a humanized monoclonal antibody against the terminal complement component 5, was shown to significantly reduce the risk of NMOSD relapse in a Phase III placebo-controlled trial. Based on this, eculizumab (Soliris®) was the first drug to be formally approved for the treatment of anti-AQP4-antibody positive NMOSD in 2019.

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