Paeds · neurology-neurodisability-and-neuromuscular
Acute disseminated encephalomyelitis and demyelinating disease
Also known as ADEM · Acquired demyelinating syndromes · MOG-antibody associated disease · Paediatric multiple sclerosis · Neuromyelitis optica spectrum disorder · Clinically isolated syndrome
A fellowship approach to the child with a first central nervous system demyelinating event. Recognise acute disseminated encephalomyelitis by the mandatory encephalopathy that separates it from the monofocal clinically isolated syndromes, then drive a unified antibody-led pathway that distinguishes the four entities a general paediatrician must tell apart - ADEM, myelin-oligodendrocyte-glycoprotein antibody-associated disease, paediatric multiple sclerosis, and neuromyelitis optica spectrum disorder - on serum MOG-IgG, aquaporin-4 IgG, MRI patterns, and the cerebrospinal fluid. Treat the acute event early with high-dose corticosteroids, step to intravenous immunoglobulin or plasma exchange when steroid-refractory, and match long-term therapy to the confirmed diagnosis, because the wrong maintenance drug - a multiple-sclerosis disease-modifying therapy given for neuromyelitis optica - can worsen the disease.
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Red flags
- Any child with acute or subacute neurological deficit plus encephalopathy - altered consciousness, irritability, or behavioural change not explained by fever alone - has an acute demyelinating event until proved otherwise, and warrants urgent brain MRI and paediatric neurology referral
- Severe bilateral optic neuritis with a swollen disc, a longitudinally extensive spinal cord lesion spanning three or more vertebral segments, or an area-postrema syndrome of intractable hiccups, nausea, and vomiting points to neuromyelitis optica spectrum disorder - never start a multiple-sclerosis disease-modifying therapy before the aquaporin-4 and MOG antibody status is known
- A child whose acute disseminated encephalomyelitis relapses within days of stopping steroids, or who develops optic neuritis or transverse myelitis after an ADEM onset, has MOG-antibody associated disease or multiphasic ADEM until the serum MOG-IgG returns - the steroid taper must be prolonged and slow
- A monosymptomatic optic neuritis or transverse myelitis in a child older than ten years, with periventricular white-matter lesions on brain MRI, raises paediatric multiple sclerosis - dissemination in space and time on serial imaging or cerebrospinal-fluid oligoclonal bands settles it
- Clinical worsening despite forty-eight to seventy-two hours of high-dose corticosteroids in any demyelinating event is steroid-refractory disease - escalate to intravenous immunoglobulin or plasma exchange rather than repeating steroids
- Never give a multiple-sclerosis disease-modifying therapy to a child with bilateral optic neuritis or a longitudinally extensive myelitis before aquaporin-4 and MOG antibodies are excluded - the wrong drug can precipitate a devastating relapse
Life stages
Care settings
Clinical exam formats
Board mappings
- General and Community Paediatrics
- Acute paediatric medicine
- Neurology and Neurodisability
- Neurology - recognise and investigate the first central nervous system demyelinating event
- Neuroimmunology - distinguish ADEM, MOGAD, multiple sclerosis, and neuromyelitis optica spectrum disorder on antibody status and MRI
- Acute care - lead high-dose corticosteroid therapy and escalation to immunoglobulin or plasma exchange
- Neurology 5-8
- Immunology and Allergy 1-3
- Clinical Applications
- Long Cases
- Short Cases
- 9. Neurology: central nervous system demyelinating disorders
- 5. Immunology: immune-mediated neurological disease
- 1. Clinical evaluation and communication
- Foundation of Practice (FOP)
- Theory and Science (TAS)
- Applied Knowledge in Practice (AKP)
- Clinical
- Neurodisability
- Communication
- History
- General Pediatrics Content Outline - D15 Neurology (6%)
- General Pediatrics Content Outline - D17 Immunology (4%)
- Patient Care 4: Clinical Reasoning
- Patient Care 5: Patient Management
- Medical Knowledge 2: Neurologic Disorders
- Systems-Based Practice 1: Patient Safety
- Medical Expert
- Pediatrics: Neurology - demyelinating disorders of childhood
- Pediatrics: Clinical Immunology
A previously well five-year-old arrives drowsy and irritable four days after a viral upper-respiratory illness, now dragging a leg and slurring his speech. Across the corridor a twelve-year-old describes three days of pain and blurred vision in one eye. In both rooms the unifying question is the same: is this a first central nervous system demyelinating event, which entity is it, and is there a long-term treatment whose window opens only if the antibody answer is right? The fellowship task is to convert that bedside picture into a structured, antibody-led pathway that treats the acute event immediately while the confirmatory tests return, and that matches the maintenance therapy to the confirmed diagnosis rather than to a guess. [1] [4]
Anchor the whole topic with MYELIN - Match the acute event to high-dose methylprednisolone first (20-30 mg/kg/day, max 1 g, three to five days), Yes-or-no on encephalopathy (the single discriminator: present equals ADEM, absent equals a clinically isolated syndrome), Escalate to immunoglobulin or plasma exchange if steroid-refractory, Label by antibody - MOG-IgG, AQP4-IgG, oligoclonal bands - before any long-term drug, Imaging pattern on MRI brain and spine points to the entity, and Never give a multiple-sclerosis disease-modifying therapy until NMOSD is excluded. [1] [3]
Overview & Definition
The clinician's first act is to recognise that a new neurological deficit in a child can be an immune-mediated attack on central nervous system myelin, and to name the family it belongs to. The International Pediatric Multiple Sclerosis Study Group (IPMSSG) defines the acquired demyelinating syndromes of childhood as a group of monophasic or relapsing inflammatory disorders that share a presumed autoimmune attack on myelin but differ in their phenotype, their imaging, and - decisively - their antibody signature and long-term treatment. The family comprises ADEM, the clinically isolated syndromes (optic neuritis, transverse myelitis, brainstem and other focal syndromes), and, after investigation, the three named diseases: MOGAD, paediatric MS, and NMOSD. [1]
Why does this grouping matter at the bedside? Because a child who looks acutely unwell with a polysymomatic encephalopathy and a child who looks well with a single symptomatic optic nerve can both be having a first demyelinating event, yet their prognoses and their maintenance treatments are entirely different. The 2013 IPMSSG definitions deliberately anchor the first fork on a single clinical feature - encephalopathy - because encephalopathy is reproducible, observable, and separates the ADEM phenotype (by definition encephalopathic) from the clinically isolated syndromes (by definition non-encephalopathic, beyond the fever and irritability expected in a sick young child). Getting that one feature right sets the whole pathway in motion. [1] [6]
What makes this group worth knowing in depth is that the modern antibody-led framework has transformed a previously confusing overlap of names into a clean diagnostic logic, and that the long-term treatment now depends on getting the antibody answer right. A child with MOGAD is managed differently from a child with MS, and both differ from a child with NMOSD - and the wrong maintenance drug can actively harm. The general paediatrician's role is to treat the acute event immediately, request the antibody tests and the imaging in parallel, and hand over to the neuroimmunology service with the right provisional label and the confirmatory samples already in flight. [2] [4]
References12ShowHide
- [1]Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC, et al. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler, 2013.PMID 23572237
- [2]Banwell B, Bennett JL, Marignier R, Kim HJ, Brilot F, Flanagan EP, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol, 2023.PMID 36706773
- [3]Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology, 2015.PMID 26092914
- [4]Marignier R, Hacohen Y, Cobo-Calvo A, et al. Myelin-oligodendrocyte glycoprotein antibody-associated disease. Lancet Neurol, 2021.PMID 34418402
- [5]Waldman A, Ness J, Pohl D, Simone IL, Anlar B, Amato MP, et al. Pediatric multiple sclerosis: Clinical features and outcome. Neurology, 2016.PMID 27572865
- [6]Tenembaum S, Chitnis T, Ness J, et al. Acute disseminated encephalomyelitis. Neurology, 2007.PMID 17438235
- [7]Ramanathan S, Mohammad S, Tantsis E, Nguyen TK, Merheb V, Fung VSC, et al. Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry, 2018.PMID 29142145
- [8]Petzold A, Fraser CL, Abegg M, Alroughani R, Alshowaeir D, Alvarenga R, et al. Diagnosis and classification of optic neuritis. Lancet Neurol, 2022.PMID 36179757
- [9]Bruijstens AL, Wendel EM, Lechner C, Bartels F, Finke C, Breu M, et al. E.U. paediatric MOG consortium consensus: Part 5 - Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders. Eur J Paediatr Neurol, 2020.PMID 33176999
- [10]Kornbluh AB, Kahn I. Pediatric Multiple Sclerosis. Semin Pediatr Neurol, 2023.PMID 37451754
- [11]Margoni M, Preziosa P, Rocca MA, Filippi M. Anti-CD20 Therapies in Pediatric Acquired Demyelinating Syndromes: Evidence Across MS, AQP4-IgG-Positive NMOSD and MOGAD. CNS Drugs, 2026.PMID 42334795
- [12]Wendel EM, Thonke HS, Bertolini A, Baumann M, Blaschek A, Merkenschlager A, et al. Temporal Dynamics of MOG Antibodies in Children With Acquired Demyelinating Syndrome. Neurol Neuroimmunol Neuroinflamm, 2022.PMID 36229191