Pediatric Multiple Sclerosis: an Update

Abstract

The recent 2017 McDonald Criteria for MS provide a simplified means to confirm diagnosis at onset and over time, and have been shown to be equally applicable for POMS. MRI analyses demonstrate that brain volume is reduced at onset, and that both volumetric and tissue integrity measures decline over time, indicating that POMS shares the degenerative aspects that also characterize adult-onset disease. The presence of myelin oligodendrocyte glycoprotein (MOG) antibodies at onset is detected in more than 50% of children with acute disseminated encephalomyelitis. When persistent over time, they are associated with relapsing disease. The first randomized clinical trials of disease supports superiority of fingolimod over subcutaneous interferon beta 1a, and demonstrated a favorable safety profile. Finally, while Expanded Disability Status Scale (EDSS) scores remain low in the first 10 years post-onset, POMS is associated with high rates of patient-reported fatigue and reduced engagement in exercise and carries a risk for cognitive impairment. The past 15 years have borne witness to a marked expansion in recognition and research in POMS. There are now more specific diagnostic criteria, antibodies to CNS proteins appear to define diagnostically distinct disorders, clinical trials have successfully launched and one has completed, and we are gaining increasing appreciation of the impact of MS and related disorders on the lived experience of children and adolescents.

Magnetic resonance imaging diagnosis of demyelinating diseases: An update

“…Patients with ADEM on rare occasions later develop optic neuritis, as "ADEM followed by optic neuritis". 59,92 MOG-IgG is positive in most of these patients. [92][93][94]…”

“…92,98,100,101 A significant percentage of patients previously diagnosed with multiphasic demyelinating encephalomyelitis and ADEM followed by optic neuritis are also MOG-IgG-positive. 92,94 MRI features of the brain, spinal cord and optic nerves in patients with MOG encephalomyelitis are distinct from those of AQP4-IgG-positive NMOSD and typical MS. 97,102…”

“…In a more aggressive form of ADEM (i.e. [92][93][94] MOG encephalomyelitis (MOG-IgG disease) MOG encephalomyelitis (MOG-IgG disease) was recently proposed as a new, distinct disease entity among the demyelinating diseases. 45 Whether acute hemorrhagic leukoencephalitis is a variant of ADEM or separate entity remains controversial.…”

“…59 Spinal cord lesions Spinal cord lesions are seen in 11-28% of patients, and LETM is the typical spinal cord lesion, similar to that in NMOSD. 92 91 The thoracic cord is most often involved.…”

Autologous haematopoietic stem cell transplantation and other cellular therapy in multiple sclerosis and immune-mediated neurological diseases: updated guidelines and recommendations from the EBMT Autoimmune Diseases Working Party (ADWP) and the Joint Accreditation Committee of EBMT and ISCT (JACIE)

“…MS is a rare disease in children, but its consequences are particularly severe as disability may be life-long [56]. In a cohort of 21 patients under 18 years, aHSCT was well tolerated and associated with improvements of EDSS scores in 81% of patients with progression free survival (PFS) of 100% at 3-5 years, hence potentially more efficacious in children than in adults [17].…”