Acute disseminated encephalomyelitis in a five-year-old girl: a case report

Authors

  • Pradeep Kumar Ranabijuli Department of Pediatrics, JagjivanRam Railway Hospital, Mumbai, Maharashtra, India
  • Komal Bhagwan Kamble Jagjivan Ram Hospital, WesteDepartment of Pediatrics, JagjivanRam Railway Hospital, Mumbai, Maharashtra, Indiarn Railway, Mumbai Central, Mumbai, Maharashtra
  • Nazparveen L. A. Department of Pediatrics, JagjivanRam Railway Hospital, Mumbai, Maharashtra, India
  • Sita Kumari Department of Pediatrics, JagjivanRam Railway Hospital, Mumbai, Maharashtra, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20242753

Keywords:

ADEM, Monophasic, Encephalopathy, Methylprednisolone

Abstract

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with a predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrence has been described and defined as multiphasic disseminated encephalomyelitis (MDEM). ADEM often occurs after infection or immunization and is clinically defined as acute polyfocal neurological deficits, including encephalopathy. Many times, ADEM is a diagnosis of exclusion, and early diagnosis and treatment are the keys to favorable outcomes. Magnetic resonance imaging (MRI) typically demonstrates reversible, large, ill-defined white matter lesions of the brain and often also the spinal cord, along with frequent involvement of thalami and basal ganglia. CSF analysis may reveal mild pleocytosis and elevated protein but is generally negative for intrathecal oligoclonal bands. We report a case of five years six-month- old girl who presented with fever, vomiting, headache, and cough for seven days. She had one episode of involuntary passage of urine. The child was lethargic at presentation and was unable to stand or walk. Clinical features and investigations, including MRI brain and spine, were suggestive of ADEM. She was started on Inj. Methylprednisolone followed by oral prednisolone in tapering dose for four weeks. Clinical improvement was seen in the form of improvement in activity and power in limbs. The child improved with normal neurological function on day three of inj methylprednisolone. On follow-up for six months, the child did not have any relapse and had complete neurological and radiological recovery.

References

Murthy SN, Faden HS, Cohen ME, Bakshi R. Acute disseminated encephalomyelitis in children. Pediatrics. 2002;110(2pt1):e21.

Kumar P, Kumar P, Sabharwal RK. Acute disseminated encephalomyelitis: case report and brief review. J Family Med Prim Care. 2014;3(4):443-5.

Ascherio A, Munger K. Epidemiology of multiple sclerosis: From risk factors to prevention. Semin Neurol. 2008;28(1):17-28.

Panicker JN. Bangalore: National Institute of Mental Health and Neurosciences, NIMHANS (Deemed University). Acute Disseminated Encephalomyelitis: Clinical Profile and Predictors of Outcome (dissertation). 2004.

Menge T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, et al. Acute disseminated encephalomyelitis: An update. Arch Neurol. 2005;62(11):1673-80.

Anlar B, Basaran C, Kose G, Guven A, Haspolat S, Yakut A, et al. Acute disseminated Encephalomyelitis in Children: Outcome and Prognosis. Neuropediatrics. 2003;34(4):194-9.

Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: A long-term follow-up study of 84 pediatric patients. Neurology. 2002;59(8):1224-31.

Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B. Acute disseminated encephalomyelitis: A follow-up study of 40 adult patients. Neurology. 2001;56(10):1313-8.

Singhi PD, Ray M, Singhi S, Kumar Khandelwal N. Acute disseminated encephalomyelitis in North Indian Children: Clinical profile and follow-up. J Child Neurol. 2006;21(10):851-7.

Marchioni E, Tavazzi E, Minoli L, Del Bue S, Ferrante P, Piccolo G, et al. Acute disseminated encephalomyelitis. Neurol Sci. 2008;29:S286-8.

Alexander M, Murthy JM. Acute disseminated encephalomyelitis: Treatment guidelines. Ann Indian Acad Neurol. 2011;14(1):S60-4.

Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology. 2001;56(10):1308-12.

Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BR. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 2000;123:2407-22.

Downloads

Published

2024-09-24

Issue

Section

Case Reports