Neuromyelitis optica

Pache, Florence; Wildemann, Brigitte; Paul, Friedemann; Jarius, Sven

doi:10.1055/s-0042-124186

Cited by 7 publications

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“…The Neuropathologic characteristics of NMOSD include deposits of serum antibodies and complement, loss of astrocytes, secondary degeneration of oligodendrocytes and neurons, and necrotic lesions with infiltration of, neutrophilic and eosinophilic granulocytes [13].…”

Section: Clinical Presentations and Evaluationmentioning

confidence: 99%

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2024

MJCCS

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Neuromyelitis optica (sNMO) or Devic's disease, is a chronic, demyelinating, autoimmune and inflammatory disease of the central nervous system (CNS), that affects predominantly the optic nerve and the spinal cord. Recently unified under a broader term, NMOSD (NMO spectrum disorders), 80% of patients with this disease present detectable serum antibodies, very specific and pathogenomic, that target the water channels aquaporin-4, called AQP4-IgG. Classified as a rare genetic disease with multifactorial origin, NMO affects predominantly females and young adults. The case we present in this study represents an exception. Our patient was diagnosed with NMO at 72, having been previously hospitalized three times, two with transverse cervical myelitis and one with binocular diplopia. These episodes did not leave sequealae and where considered of ischemic origin considering a past cerebro vascular accident. However, the patient presented four more episodes of NMO after his definitive diagnose. In the first two of these attacks, AQP4-IgG testing was requested and positive, with values of 5.31 and 1.62 respectively. No testing for antibodies were requested during his other two attacks. In total, this patient suffered eight attacks in 10 years, which included five attacks with transverse spinal cord myelitis, two with binocular diplopia, and one with dorsal transverse myelitis. The purpose of our paper is to describe this case in detail and to discuss the clinical and neurophysiological testing, genetics, immunology and treatment options available for NMOSD, with the intention of give it more visibility and improve early diagnosis, and considering the long-term debilitating effects that it can have in the patients.

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Section: Clinical Presentations and Evaluationmentioning

confidence: 99%

“…AQP4-Ab antibodies are considered pathognomonic and are detectable in approximately 80% of the patients with NMO, helping to distinguish this rare disease from multiple sclerosis (MS) [13]. However, 20-30% of the patients are seronegative for AQP4-IgG.…”

Section: Autoimmune Specificitymentioning

confidence: 99%

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2024

MJCCS

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“…Früher unter dem Namen Devic-Syndrom als seltene Variante der MS betrachtet, ist Neuromyelitis optica spätestens seit der Entdeckung eines krankheitsspezifischen Serumantikörpers gegen den astrozytären Wasserkanal Aquaporin-4 (AQP4) als eigenständige neuroimmunologische Erkrankung anzusehen [77][78][79][80][81][82][83][84][85][86], die anders zu behandeln ist als die MS [87][88][89][90][91][92][93][94]. Die neuesten Diagnosekriterien von 2015 unterscheiden nicht mehr wie zuvor zwischen Neuromyelitis optica und Neuromyelitis optica spectrum disorders (NMOSD), sondern lediglich NMOSD-Patienten mit oder ohne vorhandenen Nachweis von AQP4-Antikörpern [95].…”

Section: Neuromyelitis Optica-spektrum-erkrankungenunclassified

“…In den letzten Jahren sind zahlreiche Publikationen erschienen, die bei einem Teil der für AQP4-Antikörper seronegativen Patienten mit einem NMOSD-Phänotyp, aber auch in einem kleinen Prozentsatz von Patienten, die die MS-Diagnosekriterien erfüllen, Antikörper gegen Myelin-Oligodendrozyten-Glykoprotein (MOG) beschreiben [94,[114][115][116][117][118][119][120][121][122][123]. Die wissenschaftliche Diskussion, ob es sich hierbei um ein eigenständiges neuroimmunologisches Krankheitsbild handelt, ist noch nicht abgeschlossen [124,125].…”

Section: Mog-igg Assoziierte Enzephalomyelitisunclassified

Optische Kohärenztomografie in der Neurologie – Methodik und Anwendung in Forschung und Klinik

2017

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ZusammenfassungDie hochauflösende optische Kohärenztomografie (OCT) als nicht-invasives und rasch anwendbares bildgebendes Verfahren zur Untersuchung und Quantifizierung neuro-axonalen Schadens an der Netzhaut hat in den letzten Jahren zunehmend Eingang in die klinische Forschung inclusive interventioneller Studien in der Neurologie, v. a. auf dem Gebiet entzündlicher Erkrankungen, gefunden. Aktuell ist die OCT in der Lage, die Schädigung bzw. Veränderung verschiedener Netzhautschichten (z. B. der Nervenfaserschicht, der Ganglionzellschicht oder der inneren Körnerschicht) bei zahlreichen neurologischen Erkrankungen wie Optikusneuritis, Multiple Sklerose, Neuromyelitis optica, Susac-Syndrom aber auch primär neurodegenerativen Erkrankungen wie M. Parkinson, M. Alzheimer oder spinozerebellären Ataxien darzustellen. In vielen Fällen konnte eine gute Korrelation zwischen mit der OCT quantifizierter struktureller Netzhautschädigung und funktionellen visuellen Beeinträchtigungen inclusive der visuellen Lebensqualität gezeigt werden, was die klinische Relevanz der OCT-Befunde unterstreicht. Ob diese neue bildgebende Technologie Eingang in die klinische Routine-Diagnostik finden wird und für die Diagnostik und Verlaufsbeurteilung individueller Patienten geeignet ist, wird aktuell intensiv erforscht. Dieser Artikel beschreibt die Grundzüge der OCT-Technologie und die wichtigsten OCT-Befunde in Assoziation mit funktionellen Visusbefunden mit einem Schwerpunkt auf den entzündlichen Erkrankungen, für die bislang die meisten Daten vorliegen. Schließlich wird der mögliche zukünftige Einsatz in klinischen Studien und der klinischen Routine diskutiert.

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“…This can lead to retinal ganglion cell destruction and significant visual loss (4,6). ON can be the initial event in multiple sclerosis (MS), including clinically isolated syndrome (CIS) (3), in aquaporin-4-IgG positive (AQP4-IgG+) and seronegative neuromyelitis optica spectrum disorders (NMOSD) (7), and in myelin oligodendrocyte glycoprotein-IgG (MOG-IgG+)-associated disease (MOGAD) (8). The incidence of ON is stable and similar around the world (4,9), but the proportion of ON patients with AQP4-IgG+ON and MOG-IgG+ON vs. MS-ON differs greatly in different races (4).…”

Section: Introductionmentioning

confidence: 99%

The Acute Optic Neuritis Network (ACON): Study protocol of a non-interventional prospective multicenter study on diagnosis and treatment of acute optic neuritis

et al. 2023

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Optic neuritis (ON) often occurs at the presentation of multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). The recommended treatment of high-dose corticosteroids for ON is based on a North American study population, which did not address treatment timing or antibody serostatus. The Acute Optic Neuritis Network (ACON) presents a global, prospective, observational study protocol primarily designed to investigate the effect of time to high-dose corticosteroid treatment on 6-month visual outcomes in ON. Patients presenting within 30 days of the inaugural ON will be enrolled. For the primary analysis, patients will subsequently be assigned into the MS-ON group, the aquapotin-4-IgG positive ON (AQP4-IgG+ON) group or the MOG-IgG positive ON (MOG-IgG+ON) group and then further sub-stratified according to the number of days from the onset of visual loss to high-dose corticosteroids (days-to-Rx). The primary outcome measure will be high-contrast best-corrected visual acuity (HC-BCVA) at 6 months. In addition, multimodal data will be collected in subjects with any ON (CIS-ON, MS-ON, AQP4-IgG+ON or MOG-IgG+ON, and seronegative non-MS-ON), excluding infectious and granulomatous ON. Secondary outcomes include low-contrast best-corrected visual acuity (LC-BCVA), optical coherence tomography (OCT), magnetic resonance imaging (MRI) measurements, serum and cerebrospinal fluid (CSF) biomarkers (AQP4-IgG and MOG-IgG levels, neurofilament, and glial fibrillary protein), and patient reported outcome measures (headache, visual function in daily routine, depression, and quality of life questionnaires) at presentation at 6-month and 12-month follow-up visits. Data will be collected from 28 academic hospitals from Africa, Asia, the Middle East, Europe, North America, South America, and Australia. Planned recruitment consists of 100 MS-ON, 50 AQP4-IgG+ON, and 50 MOG-IgG+ON. This prospective, multimodal data collection will assess the potential value of early high-dose corticosteroid treatment, investigate the interrelations between functional impairments and structural changes, and evaluate the diagnostic yield of laboratory biomarkers. This analysis has the ability to substantially improve treatment strategies and the accuracy of diagnostic stratification in acute demyelinating ON.Trial registrationClinicalTrials.gov, identifier: NCT05605951.

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Neuromyelitis optica

Cited by 7 publications

References 0 publications

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Optische Kohärenztomografie in der Neurologie – Methodik und Anwendung in Forschung und Klinik

The Acute Optic Neuritis Network (ACON): Study protocol of a non-interventional prospective multicenter study on diagnosis and treatment of acute optic neuritis

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