Myelin-Oligodendrocyte Glycoprotein Antibodies Spectrum Disorders

Etemadifar, Masoud; Fazli, Ali

doi:10.29252/nirp.cjns.3.11.231

Cited by 1 publication

(4 citation statements)

References 43 publications

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“…The sequence of the events characterizing the syndrome (ADEM followed by ON) seems to depend on the typical distribution of ADS during childhood, with encephalopathic syndromes concerning younger children, and monofocal or multifocal nonencephalopathic events more typically affecting the older ones. 7,18 It has been demonstrated that MOG-abs are common in younger patients with encephalopa-thy and that MOG-abs syndromes frequently affect the optic nerves. 14 The data in the literature indicates that ADEM-ON represents a common evolution of MOG-abs seropositive patients who initially presented ADEM.…”

Section: Adem-onmentioning

confidence: 99%

“…Its precise function is not known, but it is thought to play a role in myelin sheath structural integrity and maintenance. 18,19 Its location on the outer surface of myelin sheaths makes it available for antibody binding and a potential target for autoantibody mediated diseases. 20,21 MOG-abs, widely studied in recent years, are now considered a marker of demyelination, by transiently disrupting oligodendrocytic microtubule organization.…”

Section: Clinical Features and Coursementioning

confidence: 99%

“…Since the first descriptions of ADEM-ON, efficacy of highdose steroids (20-30 mg/kg/die, for 3-5 days) or plasmapheresis during the acute phase was reported. 5,6,9,13,15,18 The main effect of high-dose steroid treatment seems to be hastening the recovery phase. 5 Maintenance oral prednisone has been recommended to reduce early relapses in ADEM-ON and, generally, in MOG-SD.…”

Section: Treatment Optionsmentioning

confidence: 99%

“…5 Maintenance oral prednisone has been recommended to reduce early relapses in ADEM-ON and, generally, in MOG-SD. 9,15,17,18 However, patients responsive to steroids showed relapse rate within the first 2 months of steroid cessation with a rapid taper. 15,16 Two studies on ADEM-ON children and MOG-SD pediatric/adult patients report oral prednisone efficacy, in terms of avoiding relapses, if given at a dosage higher than 10 mg/die.…”

Section: Treatment Optionsmentioning

confidence: 99%

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Acute Disseminated Encephalomyelitis followed by Optic Neuritis: A Rare Syndrome of Uncertain Treatment and Prognosis

et al. 2020

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Aim Acute Disseminated Encephalomyelitis followed by optic neuritis (ADEM-ON), first described in 2013, is a rare demyelinating syndrome, typical of the pediatric age. We conducted a mini review of the existing literature, focusing on clinical, laboratory, radiological, therapeutic, and prognostic aspects in order to improve the identification of new cases. Methods We searched PubMed and Cochrane Library for studies on ADEM-ON between 2013 and 2018. Results Examination of the reported cases (three case reports and eight observational studies) established the following features. Time between ADEM and ON is highly variable. Almost all patients show antimyelin oligodendrocyte glycoprotein antibody (MOG-abs) seropositivity. High-dose intravenous steroid and plasmapheresis efficacy is reported for the acute phase; oral prednisone and other maintenance drugs may be useful in avoiding relapses. The clinical history may lead to a complete recovery but also to residual deficits. Conclusion MOG-abs detection strongly supports ADEM-ON diagnosis, confirming this entity as part of MOG-abs spectrum disorder. Owing to the very small number of cases so far reported, predicting clinical evolution is very difficult.

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Section: Adem-onmentioning

confidence: 99%