Global InformationAcute disseminated encephalomyelitis information
| Acute disseminated encephalomyelitis | |
|---|---|
| Other names | Acute demyelinating encephalomyelitis |
 | |
| Fulminating ADEM showing many lesions. The patient survived, but remained in a persistent vegetative state | |
| Specialty | Neurology  |
Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. The cause is often a trigger such as from viral infection or vaccinations.[1][2][3][4][5][6]
ADEM's symptoms resemble the symptoms of multiple sclerosis (MS), so the disease itself is sorted into the classification of the multiple sclerosis borderline diseases. However, ADEM has several features that distinguish it from MS.[7] Unlike MS, ADEM occurs usually in children and is marked with rapid fever, although adolescents and adults can get the disease too. ADEM consists of a single flare-up whereas MS is marked with several flare-ups (or relapses), over a long period of time. Relapses following ADEM are reported in up to a quarter of patients, but the majority of these 'multiphasic' presentations following ADEM likely represent MS.[8] ADEM is also distinguished by a loss of consciousness, coma and death, which is very rare in MS, except in severe cases.
It affects about 8 per 1,000,000 people per year.[9] Although it occurs in all ages, most reported cases are in children and adolescents, with the average age around 5 to 8 years old.[10][11][12][13] The disease affects males and females almost equally.[14] ADEM shows seasonal variation with higher incidence in winter and spring months which may coincide with higher viral infections during these months.[13] The mortality rate may be as high as 5%; however, full recovery is seen in 50 to 75% of cases with increase in survival rates up to 70 to 90% with figures including minor residual disability as well.[15] The average time to recover from ADEM flare-ups is one to six months.
ADEM produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter. Usually these are found in the subcortical and central white matter and cortical gray-white junction of both cerebral hemispheres, cerebellum, brainstem, and spinal cord,[16] but periventricular white matter and gray matter of the cortex, thalami and basal ganglia may also be involved.
When a person has more than one demyelinating episode of ADEM, the disease is then called recurrent disseminated encephalomyelitis[17] or multiphasic disseminated encephalomyelitis[18] (MDEM). Also, a fulminant course in adults has been described.[19]
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- ^ Garg RK (January 2003). "Acute disseminated encephalomyelitis". Postgraduate Medical Journal. 79 (927): 11–17. doi:10.1136/pmj.79.927.11. PMC 1742586. PMID 12566545.
- ^ Jones CT (November 2003). "Childhood autoimmune neurologic diseases of the central nervous system". Neurologic Clinics. 21 (4): 745–64. doi:10.1016/S0733-8619(03)00007-0. PMID 14743647.
- ^ Huynh W, Cordato DJ, Kehdi E, Masters LT, Dedousis C (December 2008). "Post-vaccination encephalomyelitis: literature review and illustrative case". Journal of Clinical Neuroscience. 15 (12): 1315–22. doi:10.1016/j.jocn.2008.05.002. PMC 7125578. PMID 18976924.
- ^ Rust RS (June 2000). "Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions". Seminars in Pediatric Neurology. 7 (2): 66–90. doi:10.1053/pb.2000.6693. PMID 10914409.
- ^ Poser CM, Brinar VV (October 2007). "Disseminated encephalomyelitis and multiple sclerosis: two different diseases – a critical review". Acta Neurologica Scandinavica. 116 (4): 201–06. doi:10.1111/j.1600-0404.2007.00902.x. PMID 17824894. S2CID 44411472.
- ^ Krupp LB, Banwell B, Tenembaum S (April 2007). "Consensus definitions proposed for pediatric multiple sclerosis and related disorders" (PDF). Neurology. 68 (16 Suppl 2): S7–12. doi:10.1212/01.wnl.0000259422.44235.a8. PMID 17438241. S2CID 26001350. Archived from the original (PDF) on 9 February 2015.
- ^ Koelman DL, Chahin S, Mar SS, Venkatesan A, Hoganson GM, Yeshokumar AK, et al. (May 2016). "Acute disseminated encephalomyelitis in 228 patients: A retrospective, multicenter US study". Neurology. 86 (22): 2085–93. doi:10.1212/WNL.0000000000002723. PMID 27164698. S2CID 20818721.
- ^ Leake JA, Albani S, Kao AS, Senac MO, Billman GF, Nespeca MP, et al. (August 2004). "Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features". The Pediatric Infectious Disease Journal. 23 (8): 756–64. doi:10.1097/01.inf.0000133048.75452.dd. PMID 15295226. S2CID 31668039.
- ^ Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ (May 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. S2CID 23898511.
- ^ Anlar B, Basaran C, Kose G, Guven A, Haspolat S, Yakut A, et al. (August 2003). "Acute disseminated encephalomyelitis in children: outcome and prognosis". Neuropediatrics. 34 (4): 194–99. doi:10.1055/s-2003-42208. PMID 12973660. S2CID 260242587.
- ^ Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B (May 2001). "Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients". Neurology. 56 (10): 1313–18. doi:10.1212/WNL.56.10.1313. PMID 11376180. S2CID 25391094.
- ^ a b Bhatt P, Bray L, Raju S, Dapaah-Siakwan F, Patel A, Chaudhari R, et al. (March 2019). "Temporal Trends of Pediatric Hospitalizations with Acute Disseminated Encephalomyelitis in the United States: An Analysis from 2006 to 2014 using National Inpatient Sample". The Journal of Pediatrics. 206: 26–32.e1. doi:10.1016/j.jpeds.2018.10.044. PMID 30528761. S2CID 54478510.
- ^ Koelman DL, Mateen FJ (September 2015). "Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome". Journal of Neurology. 262 (9): 2013–24. doi:10.1007/s00415-015-7694-7. PMID 25761377. S2CID 26642207.
- ^ Menge T, Kieseier BC, Nessler S, Hemmer B, Hartung HP, Stüve O (June 2007). "Acute disseminated encephalomyelitis: an acute hit against the brain". Current Opinion in Neurology. 20 (3): 247–54. doi:10.1097/WCO.0b013e3280f31b45. PMID 17495616. S2CID 14049476.
- ^ Wingerchuk DM (May 2003). "Postinfectious encephalomyelitis". Current Neurology and Neuroscience Reports. 3 (3): 256–64. doi:10.1007/s11910-003-0086-x. PMID 12691631. S2CID 453016.
- ^ Poser CM (May 2008). "Multiple sclerosis and recurrent disseminated encephalomyelitis are different diseases". Archives of Neurology. 65 (5): 674, author reply 674–75. doi:10.1001/archneur.65.5.674-a. PMID 18474749.
- ^ Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG (December 2000). "Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children". Brain. 123 (12): 2407–22. doi:10.1093/brain/123.12.2407. PMID 11099444.
- ^ Di Pauli F, Höftberger R, Reindl M, Beer R, Rhomberg P, Schanda K, et al. (December 2015). "Fulminant demyelinating encephalomyelitis: Insights from antibody studies and neuropathology". Neurology. 2 (6): e175. doi:10.1212/NXI.0000000000000175. PMC 4635550. PMID 26587556.