Acute disseminated encephalomyelitis natural history, complications and prognosis: Difference between revisions
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==Overview== | ==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== | ==Natural History== | ||
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*[[MRI]] of patients with co-existent [[M-ADEM]] and [[anti-MOG]] [[antibodies]] typically reveal no new [[lesions]] in the [[asymptomatic period]]<ref name="pmid26869530">{{cite journal| author=Baumann M, Hennes EM, Schanda K, Karenfort M, Kornek B, Seidl R | display-authors=etal| title=Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases. | journal=Mult Scler | year= 2016 | volume= 22 | issue= 14 | pages= 1821-1829 | pmid=26869530 | doi=10.1177/1352458516631038 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26869530 }} </ref>. | *[[MRI]] of patients with co-existent [[M-ADEM]] and [[anti-MOG]] [[antibodies]] typically reveal no new [[lesions]] in the [[asymptomatic period]]<ref name="pmid26869530">{{cite journal| author=Baumann M, Hennes EM, Schanda K, Karenfort M, Kornek B, Seidl R | display-authors=etal| title=Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases. | journal=Mult Scler | year= 2016 | volume= 22 | issue= 14 | pages= 1821-1829 | pmid=26869530 | doi=10.1177/1352458516631038 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26869530 }} </ref>. | ||
== | ==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>. | *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. | *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]]. | *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== | ||
*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]]<ref name="pmid12585719">{{cite journal| author=Shahar E, Andraus J, Savitzki D, Pilar G, Zelnik N| title=Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins. | journal=J Child Neurol | year= 2002 | volume= 17 | issue= 11 | pages= 810-4 | pmid=12585719 | doi=10.1177/08830738020170111001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12585719 }} </ref><ref name="pmid7189253">{{cite journal| author=Pasternak JF, De Vivo DC, Prensky AL| title=Steroid-responsive encephalomyelitis in childhood. | journal=Neurology | year= 1980 | volume= 30 | issue= 5 | pages= 481-6 | pmid=7189253 | doi=10.1212/wnl.30.5.481 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7189253 }} </ref>. | |||
*The average [[recovery]] period varies from 1 to 6 months<ref name="pmid11376179">{{cite journal| author=Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ| title=Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. | journal=Neurology | year= 2001 | volume= 56 | issue= 10 | pages= 1308-12 | pmid=11376179 | doi=10.1212/wnl.56.10.1308 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11376179 }} </ref>, with some cases having a per-acute onset or a prolonged [[disease]] course<ref name="pmid12165620">{{cite journal| author=Murthy SN, Faden HS, Cohen ME, Bakshi R| title=Acute disseminated encephalomyelitis in children. | journal=Pediatrics | year= 2002 | volume= 110 | issue= 2 Pt 1 | pages= e21 | pmid=12165620 | doi=10.1542/peds.110.2.e21 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12165620 }} </ref><ref name="pmid11099444">{{cite journal| author=Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG| title=Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. | journal=Brain | year= 2000 | volume= 123 Pt 12 | issue= | pages= 2407-22 | pmid=11099444 | doi=10.1093/brain/123.12.2407 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11099444 }} </ref>. | |||
*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<ref name="pmid27572859">{{cite journal| author=Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S | display-authors=etal| title=Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. | journal=Neurology | year= 2016 | volume= 87 | issue= 9 Suppl 2 | pages= S38-45 | pmid=27572859 | doi=10.1212/WNL.0000000000002825 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27572859 }} </ref>. | *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<ref name="pmid27572859">{{cite journal| author=Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S | display-authors=etal| title=Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. | journal=Neurology | year= 2016 | volume= 87 | issue= 9 Suppl 2 | pages= S38-45 | pmid=27572859 | doi=10.1212/WNL.0000000000002825 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27572859 }} </ref>. | ||
* [[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<ref name="pmid17188007">{{cite journal| author=Mikaeloff Y, Caridade G, Husson B, Suissa S, Tardieu M, Neuropediatric KIDSEP Study Group of the French Neuropediatric Society| title=Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse. | journal=Eur J Paediatr Neurol | year= 2007 | volume= 11 | issue= 2 | pages= 90-5 | pmid=17188007 | doi=10.1016/j.ejpn.2006.11.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17188007 }} </ref>. | * [[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<ref name="pmid17188007">{{cite journal| author=Mikaeloff Y, Caridade G, Husson B, Suissa S, Tardieu M, Neuropediatric KIDSEP Study Group of the French Neuropediatric Society| title=Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse. | journal=Eur J Paediatr Neurol | year= 2007 | volume= 11 | issue= 2 | pages= 90-5 | pmid=17188007 | doi=10.1016/j.ejpn.2006.11.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17188007 }} </ref>. | ||
==References== | ==References== |
Latest revision as of 10:11, 8 December 2022
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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
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.