Background: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease of the central nervous system usually preceded by an infection. While mainly a childhood disease, ADEM carries a worse prognosis in adults. We present a case of severe fulminant ADEM in an 18-year-old patient resulting in rapid neurological injury. CaseReport: An 18-year-old woman presented to our emergency room with fever and an altered level of consciousness followed by a generalized tonic-clonic seizure. Cerebrospinal fluid (CSF) findings were initially suggestive of meningitis; however, subsequent neuroimaging and laboratory evaluation favored a diagnosis of acute disseminated encephalomyelitis. Despite treatment with broad-spectrum antimicrobials and high-dose corticosteroids, the patient experienced rapid neurological deterioration, culminating in extensive and irreversible brain injury. Conclusion: Prompt diagnosis and treatment of ADEM, particularly in the adult population, remains a significant clinical challenge. Our case underscores the need for heightened vigilance and continued research to assist clinicians in the time-sensitive identification of this severe demyelinating disease.
References
[1]
Pohl, D., Alper, G., Van Haren, K., Kornberg, A.J., Lucchinetti, C.F., Tenembaum, S., et al. (2016) Acute Disseminated Encephalomyelitis: Updates on an Inflammatory CNS Syndrome. Neurology, 87, S38-S45. https://doi.org/10.1212/wnl.0000000000002825
[2]
Schwarz, S., Mohr, A., Knauth, M., Wildemann, B. and Storch-Hagenlocher, B. (2001) Acute Disseminated Encephalomyelitis: A Follow-Up Study of 40 Adult Patients. Neurology, 56, 1313-1318. https://doi.org/10.1212/wnl.56.10.1313
[3]
Lucas, J. (1790) An Account of Uncommon Symptoms Succeeding Measles: With Some Additional Remarks on the Infection of Measles and Smallpox. The London Medical Journal, 11, 325-331.
[4]
Otallah, S. (2020) Acute Disseminated Encephalomyelitis in Children and Adults: A Focused Review Emphasizing New Developments. MultipleSclerosisJournal, 27, 1153-1160. https://doi.org/10.1177/1352458520929627
[5]
Krupp, L.B., Tardieu, M., Amato, M.P., Banwell, B., Chitnis, T., Dale, R.C., 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. MultipleSclerosisJournal, 19, 1261-1267. https://doi.org/10.1177/1352458513484547
[6]
Berzero, G., Cortese, A., Ravaglia, S. and Marchioni, E. (2015) Diagnosis and Therapy of Acute Disseminated Encephalomyelitis and Its Variants. ExpertReviewofNeurotherapeutics, 16, 83-101. https://doi.org/10.1586/14737175.2015.1126510
[7]
Hatharasinghe, A., Akhondi, H. and Pepito, D. (2020) Acute Disseminated Encephalomyelitis. HCAHealthcareJournalofMedicine, 1, 77-82. https://doi.org/10.36518/2689-0216.1038
[8]
Li, K., Li, M., Wen, L., Wang, Q., Ding, X. and Wang, J. (2022) Clinical Presentation and Outcomes of Acute Disseminated Encephalomyelitis in Adults Worldwide: Systematic Review and Meta-Analysis. FrontiersinImmunology, 13, Article 870867. https://doi.org/10.3389/fimmu.2022.870867
[9]
Ketelslegers, I., Visser, I., Neuteboom, R., Boon, M., Catsman-Berrevoets, C. and Hintzen, R. (2010) Disease Course and Outcome of Acute Disseminated Encephalomyelitis Is More Severe in Adults than in Children. MultipleSclerosisJournal, 17, 441-448. https://doi.org/10.1177/1352458510390068
[10]
Anilkumar, A.C., Foris, L.A. and Tadi, P. (2024) Acute Disseminated Encephalomyelitis. StatPearls.
[11]
Baumann, M., Sahin, K., Lechner, C., Hennes, E.M., Schanda, K., Mader, S., et al. (2014) Clinical and Neuroradiological Differences of Paediatric Acute Disseminating Encephalomyelitis with and without Antibodies to the Myelin Oligodendrocyte Glycoprotein. JournalofNeurology, Neurosurgery&Psychiatry, 86, 265-272. https://doi.org/10.1136/jnnp-2014-308346
[12]
Corbali, O. and Chitnis, T. (2023) Pathophysiology of Myelin Oligodendrocyte Glycoprotein Antibody Disease. Frontiers in Neurology, 14, Article 1137998. https://doi.org/10.3389/fneur.2023.1137998
[13]
López-Chiriboga, A.S., Majed, M., Fryer, J., Dubey, D., McKeon, A., Flanagan, E.P., et al. (2018) Association of Mog-Igg Serostatus with Relapse after Acute Disseminated Encephalomyelitis and Proposed Diagnostic Criteria for Mog-IgG-Associated Disorders. JAMANeurology, 75, 1355-1363. https://doi.org/10.1001/jamaneurol.2018.1814
[14]
Armangue, T., Olivé-Cirera, G., Martínez-Hernandez, E., Sepulveda, M., Ruiz-Garcia, R., Muñoz-Batista, M., et al. (2020) Associations of Paediatric Demyelinating and Encephalitic Syndromes with Myelin Oligodendrocyte Glycoprotein Antibodies: A Multicentre Observational Study. The Lancet Neurology, 19, 234-246.
[15]
Gklinos, P. and Dobson, R. (2024) Myelin Oligodendrocyte Glycoprotein-Antibody Associated Disease: An Updated Review of the Clinical Spectrum, Pathogenetic Mechanisms and Therapeutic Management. Antibodies, 13, Article 43. https://doi.org/10.3390/antib13020043