12/28/2023 0 Comments Mog ad3,4 The direct pathogenicity of this antibody remains unclear. Unlike ADEM, MOGAD is a specific neuronal antibody-mediated disorder characterized by a significant elevation in MOG immunoglobulin (Ig) G1. Features more characteristic of paediatric MOGAD than MS or NMOSD include bilateral, anterior, longitudinally extensive optic neuritis with optic nerve-sheath involvement and optic nerve–head oedema/haemorrhage spinal cord conus medullaris lesions and ‘H-sign’ indicative of grey-matter involvement pleocytosis of approximately 50 white blood cells/uL with mixed lymphocytes, neutrophils and monocytes encephalopathy or encephalitis (including seizures) fever headache and positive meningeal signs. MOGAD overlaps with MS and NMOSD along a demyelinating disease spectrum. Therefore, we have found it helpful to remove the ADEM diagnosis from a patient’s chart once MOGAD is confirmed to minimize confusion. 3,4 As discussed further below, failure to specify MOGAD as the cause of ADEM can delay targeted and timely treatment. On the other hand, MOGAD confers a significant risk of relapse. 9 When ADEM is not caused by MOGAD, it is more likely to be monophasic. In the aforementioned large ADEM cohort analysis, ADEM was a secondary diagnosis in the vast majority of cases (97.8%). In our experience, a significant proportion of cases continue to be diagnosed as ADEM without specifying MOGAD as the underlying cause. This is particularly important given the multitude of other causes of ADEM and the significant overlap between MOGAD, multiple sclerosis ( MS), and neuromyelitis optica spectrum disorder ( NMOSD). Well over half of the children diagnosed with ADEM have MOG antibodies, 10 although mode of testing, titre level and diagnostic criteria must be carefully weighed before MOGAD can be formally diagnosed. 4 Unlike ADEM, MOGAD does not require encephalopathy, although this can be present. 3–5 Proposed diagnostic criteria for paediatric MOGAD were recently published. 3–5 No preferential racial or ethnic patterns have emerged to date. 3–5 MOGAD can be monophasic, although 30– 50 % of children experience relapsing optic neuritis. 3–5 In paediatric MOGAD, ADEM with or without optic nerve involvement is the most common initial presentation, particularly in children younger than 11. 3–5 Cerebral cortical encephalitis and brainstem and cerebellar presentations are less frequent. MOGAD presents most commonly as ADEM, optic neuritis or transverse myelitis. 3,4 This number is likely to grow with increasing awareness of the disease. Interestingly, MOGAD has a similar incidence as ADEM, with approximately 0.3 cases per 100,000 children recorded annually worldwide. The highest incidence of ADEM is recorded in winter and spring and the lowest in summer, likely reflecting seasonal variation in viral incidences. This discrepancy may be due to higher hospitalization rates from viral infections among these groups. 9 Black, Hispanic and children from other ethnic minority groups showed higher incidences than Caucasian children. 1,2,6,8,9 In a large retrospective USA study of over 3,000 paediatric ADEM hospitalizations between 20, the overall incidence of ADEM cases and costs were found to be increasing across all ages, except in the Northeastern USA. 5–8 The estimated worldwide incidence of ADEM is 0.2 –0.4 per 100,000 children annually, with up to 67 % presenting in those younger than 10 years. The purpose of this review is, therefore, to clarify the differences between MOGAD and ADEM and provide practical updates based on real-world experience for the work-up and management of each.ĪDEM can have numerous causes, with over 70 % of cases preceded by infection. 4 However, here at the Cleveland Clinic Neurological Institute, it is our experience that many clinicians unfamiliar with neuroinflammatory disorders of childhood continue to diagnose, document and manage confirmed MOGAD cases exclusively as ADEM, which can result in suboptimal care. 3,4 Although MOGAD can present with ADEM, MOGAD is a specific disease with its own diagnostic criteria, prognostic implications and therapeutic requirements. 3 With increasing awareness and testing for MOGAD, many children who historically lacked a clear aetiology for ADEM are being diagnosed with MOGAD. 4 Both occur more frequently in children than adults. 2,3 Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease ( MOGAD), a specific neuronal antibody-mediated disease that can often present as ADEM, was discovered only in the last decade. Acute disseminated encephalomyelitis ( ADEM), first characterized in 1931, 1 is a non-specific clinical syndrome of polyfocal central nervous system (CNS) inflammatory demyelination it is characterized by encephalopathy and large, poorly demarcated cerebral white matter lesions.
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