Acute disseminated encephalomyelitis natural history, complications and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sujaya Chattopadhyay, M.D.[2]
Overview
The classic form, accounting for 70-90% of cases, typically follows a monophasic pattern. Residual severe disability is quite rare in pediatric ADEM cases (7%). Adult patients frequently suffer from residual ataxia, clumsiness, hemiparesis or epilepsy. The poor prognosis and long-term outcomes of ADEM have changed dramatically owing to efficient vaccination coverage and widespread, early use of high-dose steroids.
Natural History
- The classic form, accounting for 70-90% of cases, typically follows a monophasic pattern[1][2]. However, multiphasic disease progression (M-ADEM) has also been previously reported[3].
- The lag period between onset to first relapse can vary from 2 months to 8 years[2], with one case showing recurrence after three decades[4].
- Most of M-ADEM patients demonstrate resolution of lesions with no neurological sequelae on long-term clinical and imaging follow-up[5].
- MRI of patients with co-existent M-ADEM and anti-MOG antibodies typically reveal no new lesions in the asymptomatic period[6].
Complications
- Residual severe disability is quite rare in pediatric ADEM cases (7%)[7].
- 20-30% of pediatric cases, usually with onset before five years of age, exhibit residual neurologic deficits, especially cognitive impairment in attention and execution[3]. Personality and behavioral changes may also be present.
- Adult patients frequently suffer from residual ataxia, clumsiness, hemiparesis or epilepsy[2]. They also have an increased rate of hospitalization, ICU admissions, and mortality.
Prognosis
- The poor prognosis and long-term outcomes of ADEM have changed dramatically owing to efficient vaccination coverage and widespread, early use of high-dose steroids[8][9].
- The average recovery period varies from 1 to 6 months[10], with some cases having a per-acute onset or a prolonged disease course[11][12].
- About 65-85% of pediatric ADEM cases exhibit a favourable prognosis with a good functional recovery within a few weeks. Clinical improvement is visible within days of starting treatment[13].
- Prognostic factors for a relapse of ADEM include coexistent optic neuritis, MRI findings similar to multiple sclerosis, and a history of CNS disorders in the family[14].
References
- ↑ Leake JA, Albani S, Kao AS, Senac MO, Billman GF, Nespeca MP; et al. (2004). “Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features”. Pediatr Infect Dis J. 23 (8): 756–64. doi:10.1097/01.inf.0000133048.75452.dd. PMID 15295226.
- ↑ 2.0 2.1 2.2 Tenembaum S, Chamoles N, Fejerman N (2002). “Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients”. Neurology. 59 (8): 1224–31. doi:10.1212/wnl.59.8.1224. PMID 12391351.
- ↑ 3.0 3.1 Berzero G, Cortese A, Ravaglia S, Marchioni E (2016). “Diagnosis and therapy of acute disseminated encephalomyelitis and its variants”. Expert Rev Neurother. 16 (1): 83–101. doi:10.1586/14737175.2015.1126510. PMID 26620160.
- ↑ Numa S, Kasai T, Kondo T, Kushimura Y, Kimura A, Takahashi H; et al. (2016). “An Adult Case of Anti-Myelin Oligodendrocyte Glycoprotein (MOG) Antibody-associated Multiphasic Acute Disseminated Encephalomyelitis at 33-year Intervals”. Intern Med. 55 (6): 699–702. doi:10.2169/internalmedicine.55.5727. PMID 26984094.
- ↑ Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC; et al. (2013). “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. 19 (10): 1261–7. doi:10.1177/1352458513484547. PMID 23572237.
- ↑ Baumann M, Hennes EM, Schanda K, Karenfort M, Kornek B, Seidl R; et al. (2016). “Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases”. Mult Scler. 22 (14): 1821–1829. doi:10.1177/1352458516631038. PMID 26869530.
- ↑ Shilo S, Michaeli O, Shahar E, Ravid S (2016). “Long-term motor, cognitive and behavioural outcome of acute disseminated encephalomyelitis”. Eur J Paediatr Neurol. 20 (3): 361–7. doi:10.1016/j.ejpn.2016.01.008. PMID 26876769.
- ↑ Shahar E, Andraus J, Savitzki D, Pilar G, Zelnik N (2002). “Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins”. J Child Neurol. 17 (11): 810–4. doi:10.1177/08830738020170111001. PMID 12585719.
- ↑ Pasternak JF, De Vivo DC, Prensky AL (1980). “Steroid-responsive encephalomyelitis in childhood”. Neurology. 30 (5): 481–6. doi:10.1212/wnl.30.5.481. PMID 7189253.
- ↑ Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ (2001). “Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children”. Neurology. 56 (10): 1308–12. doi:10.1212/wnl.56.10.1308. PMID 11376179.
- ↑ Murthy SN, Faden HS, Cohen ME, Bakshi R (2002). “Acute disseminated encephalomyelitis in children”. Pediatrics. 110 (2 Pt 1): e21. doi:10.1542/peds.110.2.e21. PMID 12165620.
- ↑ Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG (2000). “Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children”. Brain. 123 Pt 12: 2407–22. doi:10.1093/brain/123.12.2407. PMID 11099444.
- ↑ Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S; et al. (2016). “Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome”. Neurology. 87 (9 Suppl 2): S38–45. doi:10.1212/WNL.0000000000002825. PMID 27572859.
- ↑ Mikaeloff Y, Caridade G, Husson B, Suissa S, Tardieu M, Neuropediatric KIDSEP Study Group of the French Neuropediatric Society (2007). “Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse”. Eur J Paediatr Neurol. 11 (2): 90–5. doi:10.1016/j.ejpn.2006.11.007. PMID 17188007.
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