Acute disseminated encephalomyelitis natural history, complications and prognosis: Difference between revisions
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==[[Complications]]== | ==[[Complications]]== | ||
*Residual severe [[disability]] is quite rare in [[pediatric]] [[ADEM]] cases (7%)<ref name="pmid26876769">{{cite journal| author=Shilo S, Michaeli O, Shahar E, Ravid S| title=Long-term motor, cognitive and behavioural outcome of acute disseminated encephalomyelitis. | journal=Eur J Paediatr Neurol | year= 2016 | volume= 20 | issue= 3 | pages= 361-7 | pmid=26876769 | doi=10.1016/j.ejpn.2016.01.008 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26876769 }} </ref>. | |||
*20-30% of [[pediatric]] cases, usually with onset before five years of age, exhibit [[residual]] [[neurologic]] [[deficits]], especially [[cognitive]] [[impairment]] in attention and execution<ref name="pmid26620160">{{cite journal| author=Berzero G, Cortese A, Ravaglia S, Marchioni E| title=Diagnosis and therapy of acute disseminated encephalomyelitis and its variants. | journal=Expert Rev Neurother | year= 2016 | volume= 16 | issue= 1 | pages= 83-101 | pmid=26620160 | doi=10.1586/14737175.2015.1126510 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26620160 }} </ref>. [[Personality]] and [[behavioral]] changes may also be present. | |||
*Adult patients frequently suffer from residual [[ataxia]], [[clumsiness]], [[hemiparesis]] or [[epilepsy]]<ref name="pmid12391351">{{cite journal| author=Tenembaum S, Chamoles N, Fejerman N| title=Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. | journal=Neurology | year= 2002 | volume= 59 | issue= 8 | pages= 1224-31 | pmid=12391351 | doi=10.1212/wnl.59.8.1224 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12391351 }} </ref>. They also have an increased rate of [[hospitalization]], [[ICU]] admissions, and [[mortality]]. | |||
==[[Prognosis]]== | ==[[Prognosis]]== |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sujaya Chattopadhyay, M.D.[2]
Overview
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
- 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[8].
- 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[9].
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.
- ↑ 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.