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Journal: Annals of Neurology; March 3, 2023
Author(s): Sean J. Pittock, Michael Barnett, Jeffrey L. Bennett, Achim Berthele, Jérôme de Sèze, Michael Levy, Ichiro Nakashima, Celia Oreja-Guevara, Jacqueline Palace, Friedemann Paul, Carlo Pozzilli, Marcus Yountz, Kerstin Allen, Yasmin Mashhoon, Ho Jin Kim
Ravulizumab in NMOSD patients testing positive for AQP4
Ravulizumab is a therapy for NMOSD that has recently (in 2024) been approved in the US, following the clinical trial called CHAMPION-NMOSD. This paper, which reviews data from the clinical trial, shows the safety and efficacy of ravulizumab in adult NMOSD patients who tested positive for aquaporin-4 antibodies (AQP4+). Ravulizumab was found to significantly reduce the risk of relapse in patients with AQP4+ NMOSD, with similar levels of safety as eculizumab for NMOSD and as ravulizumab in all other conditions where it has been approved.
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Journal: International Journal of Molecular Sciences; December 24, 2020
Author(s): Wojciech Ambrosius, Sławomir Michalak, Wojciech Kozubski, and Alicja Kalinowska
Overview of MOGAD: Disease behavior, diagnosis, and management
This review presents the current known information regarding MOGAD. is often misdiagnosed as MS or NMOSD but is considered a distinct condition. The most common initial symptom of MOGAD seems to be optic neuritis in adults and inflammation and demyelination of the brain and spinal cord in children. In adults, the disease tends to have relapses, which gradually increase disability. In children, demyelination is usually a one-time incident. Acute treatment directed at the immune system seems to be effective in preventing relapses and limiting disability. The detection of MOG antibodies with a highly specific and sensitive diagnostic test helps in differentiating MOGAD from MS and NMOSD. While there are no clinical trials and approved treatments for MOGAD, high-dose steroids and plasma exchange seem to be helpful for the treatment of acute attacks, whereas immunosuppressive therapies, such as steroids, oral immunosuppressants, and rituximab seem to be effective as maintenance treatment.
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Journal: Neurology; March 6, 2024
Author(s): Gemma Olivé-Cirera, Arlette L. Bruijstens, Elianet G. Fonseca, Li-Wen Chen, Eva Caballero, Eugenia Martinez-Hernandez, Mar Guasp, Maria Sepúlveda, Laura Naranjo, Raquel Ruiz-García, Yolanda Blanco, Albert Saiz, Josep O. Dalmau, and Thaís Armangue for the Spanish Pediatric MOGAD Study Group
What does detection of MOG antibodies only in cerebrospinal fluid mean in children with inflammatory disorders of the central nervous system?
In children with inflammatory disorders of the central nervous system (CNS), MOG antibodies may be detected only in the cerebrospinal fluid (CSF), only in the blood serum, or in both CSF and blood serum. In this study, serum and CSF were tested for MOG antibodies in groups of children with various types of neurological disorders— (a) demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM); (b) non-ADEM encephalitis; and (c) noninflammatory neurologic disorders. The results showed that MOG antibodies may be detected in children with CNS inflammatory disorders. MOG antibody detection only in the CSF and not in the serum is more likely to be indicative of multiple sclerosis.
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Journal: Cureus; March 13, 2024
Author(s): Noah J. Spillers, Patrick M. Luther, Norris C. Talbot, Evan J. Kidder, Connor A. Doyle, Salim C. Lutfallah, Alyssa G. Derouen, Sridhar Tirumala, Shahab Ahmadzadeh, Sahar Shekoohi, Alan D. Kaye, Giustino Varrassi
Optic neuritis — different forms, treatments, diagnosis, and prognosis
Optic neuritis can lead to partial or total permanent vision loss if left untreated. Accurate diagnosis and prompt treatment are essential to prevent long-term eye damage. Optic neuritis is a common symptom of MS, NMOSD, or MOGAD. This review helps physicians evaluate optic neuritis on the basis of MRI and visual tests as a to differentiate between MS, NMOSD, and MOGAD.
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Journal: Nature; February 1, 2024
Author(s): Elie Dolgin
Why are women more likely than men to have autoimmune diseases?
Autoimmune diseases are up to 4 times more common in women than men, but there has been no evidence-based explanation for this until now, although several theories exist. New research on mice shows that this increased female association may be linked to a molecule called Xist found on X chromosomes. This molecule generates specific proteins when two X chromosomes are present, as in women, and some of these proteins seem to be linked to autoimmune diseases. When the Xist molecule was engineered into the DNA of male mice, these mice began developing signs of autoimmune disease almost as severe as in women. If these findings translate well to human studies, it could lead to new and better ways to diagnose and treat autoimmune diseases.
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Journal: Annals of Clinical and Translational Neurology; February 20, 2024
Author(s): Isabella Gomez Hjerthen, Cristina Trápaga Hacker, William Meador, Ahmed Z. Obeidat, Lucas Horta, Farrah J. Mateen
Impact of NMOSD on employment and income in the US
In this study, working-age NMOSD patients participated in a survey designed to understand factors like loss of jobs, income, and work hours associated with this disease in the US.
Of 127 participants (mostly female and Caucasian; average diagnosis age 38.7 years, average disease duration 6.4 years), among whom 94% were under immunotherapy, 56% lost a job due to NMOSD. While 80% were employed before diagnosis, only 68% were after diagnosis. 36% of participants said they no longer worked outside the home, and 68% of those who were employed before diagnosis had to reduce their work hours by about 18 hours per month after. The growth in average annual income was lower for patients than the estimated average in the US. 60% of participants had a regular unpaid caregiver; 34% of caregivers changed their work hours or job to help manage NMOSD.
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Journal: Neurology; December 11, 2020
Author(s): Viktoria Papp, Melinda Magyari, Orhan Aktas, Thomas Berger, Simon A Broadley, Philippe Cabre, Anu Jacob, Jun-Ichi Kira, Maria Isabel Leite, Romain Marignier, Katsuichi Miyamoto, Jacqueline Palace, Albert Saiz, Maria Sepulveda, Olafur Sveinsson, Zsolt Illes
How common is NMOSD around the world?
This study reviewed 33 published papers on NMOSD epidemiology (how a disease occurs in different groups and why) to determine the worldwide prevalence (number of people with a disease at a time), incidence (frequency of occurrence), and basic demographic characteristics of NMOSD and provide a critical overview of the published studies. The results showed that NMOSD is a rare disease worldwide. The incidence and prevalence were highest in the Afro-Caribbean region and the lowest in Australia and New Zealand; and highest in African ethnicity, lowest in White ethnicity. NMOSD occurred most commonly in Middle-aged adults and more in females than males.
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Journal: Multiple Sclerosis Journal; January 27, 2024
Author(s): Farren BS Briggs, Jacqueline Shaia
How common is NMOSD in the United States?
This study aimed to determine the number of people living with NMOSD in the US in 2022. The prevalence (number of people with the disease at a particular timepoint) was 6.88 per 100,000 people. The prevalence was highest in Blacks, followed by Asians and then Whites; more in females than in males. The study estimated that there were near 22,000 Americans living with NMOSD in 2022.
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Journal: Journal of Neurology; September 7, 2023
Author(s): Tania Kümpfel, Katrin Giglhuber, Orhan Aktas, Ilya Ayzenberg, Judith Bellmann-Strobl, Vivien Häußler, Joachim Havla, Kerstin Hellwig, Martin W Hümmert, Sven Jarius, Ingo Kleiter, Luisa Klotz, Markus Krumbholz, Friedemann Paul, Marius Ringelstein, Klemens Ruprecht, Makbule Senel, Jan-Patrick Stellmann, Florian Then Bergh, Corinna Trebst, Hayrettin Tumani, Clemens Warnke, Brigitte Wildemann, Achim Berthele; Neuromyelitis Optica Study Group (NEMOS)
Updated recommendations for immediate attack treatment and long-term management of NMOSD
A group of experts called the neuromyelitis optica study group (NEMOS; see www.nemos-net.de) was founded in 2008 in the US to broaden the understanding of NMOSD and similar but distinct disorders, such as MOGAD. This paper, part 2 of 2, provides recommendations for NMOSD management considering the current available research and therapies.
The recommendations provided in the paper cover both aquaporin-4 (AQP4) seropositive (antibodies detected) and seronegative (antibodies not detected) NMOSD, including
- How to treat attacks
- How to prevent attacks
- Specific therapies based on mechanisms of action
- Classical immunosuppressants, namely, azathioprine, mycophenolate mofetil, low-dose oral glucocorticoids
- B Cell depletion therapies, namely, rituximab, inebilizumab,
- Complement inhibition therapies, namely, eculizumab, ravulizumab
- Interleukin-6 receptor blockade, namely, tocilizumab, satralizumab
- Other interventions such as stem cell therapy
- Ineffective and not recommended treatments
- Recommendations for double sero-negative NMOSD
- Sequencing and switching between therapies
- Decisions regarding therapy duration, pregnancy management, vaccination, and future therapies
Related article: Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis
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Journal: Journal of Neurology; April 6, 2023
Author(s): Sven Jarius, Orhan Aktas, Ilya Ayzenberg, Judith Bellmann-Strobl, Achim Berthele, Katrin Giglhuber, Vivien Häußler, Joachim Havla, Kerstin Hellwig, Martin W Hümmert, Ingo Kleiter, Luisa Klotz, Markus Krumbholz, Tania Kümpfel, Friedemann Paul, Marius Ringelstein, Klemens Ruprecht, Makbule Senel, Jan-Patrick Stellmann, Florian Then Bergh, Hayrettin Tumani, Brigitte Wildemann, Corinna Trebst; Neuromyelitis Optica Study Group (NEMOS)
Updated recommendations for the diagnosis of NMOSD
A group of experts called the neuromyelitis optica study group (NEMOS; see www.nemos-net.de) was founded in 2008 in the US to broaden the understanding of NMOSD and similar but distinct disorders, such as MOGAD. NEMOS has published several US-wide and international multicenter investigations and practical recommendations on the diagnosis and treatment of NMOSD and MOGAD. This paper, part 1 of 2, updates the last NEMOS recommendations for the diagnosis of NMOSD, which were published in 2014.
The recommendations include:
- When to consider NMOSD? Factors based on clinical investigation and its general prevalence in certain populations
- How should NMOSD be diagnosed?
- What alternative diagnoses should be considered?
- Practical recommendations for antibody testing, cerebrospinal fluid analysis, other laboratory tests, MRI, and vision/eye function tests
- A commentary on unmet needs
Patients can refer to this paper for shared decision-making with doctors at the time of diagnosis.
Related article: Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management
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