Title: Clinical Profile of Acute Disseminated Encephalomyelitis in Children- An Experience from a Tertiary Care Centre in Eastern India

Authors: Dr Malay Kumar Sinha, Dr Partha Pratim Paul, Dr Suprit Basu

 DOI: https://dx.doi.org/10.18535/jmscr/v7i7.37

Abstract

Acute Disseminated Encephalomyelitis (ADEM) as one of the etiology of Acute Encephalitis Syndrome (AES) is an acute widespread immune mediated inflammatory demyelinating event of the central nervous system with multifocal neurological deficit; typically accompanied by encephalopathy of varying degree.

This observational prospective hospital based study was conducted in the Pediatric Medicine department of a tertiary care centre in Eastern India over a period of 18 months. All 6 months to 12 years patients fulfilling the inclusion criteria were included in the study and outcome assessed. Out of a total of 30 children; 17 (57%) were boys and 13 (43%) girls. The youngest of them was 10 months old and the eldest being 12 years old. The most common presenting feature was alteration of consciousness seen in 24 (80%) followed by motor weakness seen in 21(70%) children.Glasgow coma score (GCS) less than 8 was seen in 4 patients at presentation. All children were followed up to one and half years. Out of 24 children who had monophasic course of illness 22 children (73.3% of total patients) made uneventful recovery. It requires early diagnosis and institution of specific therapy to decrease neurologic and psychiatric morbidity.

Keywords: ADEM, Methylprednisolone, IVIG.

References

  1. Jayakrishnan MP, Krishnakumar P. Clinical profile of acute disseminated encephalomyelitis in children. J Pediatr Neurosci. 2010 Jul-Dec;5(2):111–114.
  2. Murthy SN, Faden HS, Cohen ME, Bakshi R. Acute disseminated encephalomyelitis in children.Pediatrics. 2002;110:e21. 
  3. 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;21:851–7.
  4. Madan S, Aneja S, Tripathi RP, Batra A, Seth A, Taluja V. Acute disseminated encephalomyelitis: A case series. Indian Pediatr. 2005;42:367–71.
  5. Panicker JN1, Nagaraja D, Kovoor JM, Subbakrishna DK.Descriptive study of acute disseminated encephalomyelitis and evaluation of functional outcome predictors. J Postgrad Med. 2010 Jan-Mar;56(1): 12-6.
  6. Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 2002;59:1224–31. 
  7. Mikaeloff Y, Caridade G, Husson B, Suissa S, Tardieu M. Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse. Eur J Paediatr Neurol. 2007;11:90–5
  8. Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multip-hasic disseminated encephalomyelitis and multiple sclerosis in children . Brain.2000;123:2407–2. 
  9. Gupte G1, Stonehouse M, Wassmer E, Coad NA, Whitehouse WP.Acute disseminated encephalomyelitis: a review of 18 cases in childhood. J Paediatr Child Health 2003 July;39(5):336-42
  10. L Bennetto, N Scolding. Inflammatory/ post-infectious encephalomyelitis.J Neurol Neurosurg Psychiatry 2004;75:i22-i28
  11. Likasitwattanakul S1, Chiewvit P. Acute disseminated encephalomyelitis in Siriraj Hospital: clinical manifestations and short-term outcome. J Med Assoc Thai 2012 Mar;95(3):391-6
  12. Rostásy K1, Nagl A, Lütjen S, Roll K, Zotter S, Blaschek A, Korenke GC, Karenfort M, Gotwald T, Holthausen H. Clinical outcome of children presenting with a severe manifestation of acute disseminated encephalomyelitis. Update Neuro paediatrie neuro 02/2012. 29.06.2012
  13. Sadek AA1, Mohamed MA2, Abou-Taleb A2, Mohammed MI3. Pattern and Outcome of Acute Disseminated Encephalomyelitis (ADEM) in Children: Experience in a Tertiary Center, Upper Egypt.
  14. Shahar E1, Andraus J, Savitzki D, Pilar G, Zelnik N..Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins. J. Child Neurol 2002 Nov; 17(11):810-4
  15. Shilo S1, Michaeli O2, Shahar E3, Ravid S4.Long-term motor, cognitive and behavioral outcome of acute disseminated encephalomyelitis. European Journal of Paediatric Neurology: EJPN. [2016; 20(3):361-367]
  16. Visudtibhan A1, Tuntiyathorn L, Vaewpanich J, Sukjit P, Khongkatithum C, Thampratankul L, Chiemchanya S, Visudhiphan P. Acute disseminated encephalomyelitis: a 10-year cohort study in Thai children. European Journal of Paed Neurol. 2010 Nov;14(6):513-8
  17. Weng WC1, Peng SS, Lee WT, Fan PC, Chien YH, Du JC, Shen YZ.Acute disseminated encephalomyelitis in children: one medical center experience. Acta Paediatr Taiwan, 2006 Mar-Apr; 47(2):67-71
  18. Suppiej A1, Cainelli E2, Casara G3, Cappellari A3, Nosadini M3, Sartori S3.Long-term neurocognitive outcome and quality of life in pediatric acute disseminated encephalomyelitis. Paediatr Neurol 2014 Apr;50(4):363-7.
  19. Parrish JB1, Weinstock-Guttman B, Yeh EA.Cerebellar mutism in pediatric acute disseminated encephalomyelitis. Paediatr Neurol 2014 Apr;50(4):363-7
  20. Hahn CD1, Miles BS, MacGregor DL, Blaser SI, Banwell BL, Hetherington CR.Neurocognitive outcome after acute disseminated encephalomyelitis. Paediatr neurol 2003 Aug29(2):117-23.
  21. Anlar B1, Basaran C, Kose G, Guven A, Haspolat S, Yakut A, Serdaroglu A, Senbil N, Tan H, Karaagaoglu E, Karli Oguz K. Acute disseminated encephalomyelitis in children: outcome and prognosis. Neuropediatrics.2003 Aug;34(4):194-9.
  22. Young PN, Weinshenker GB, Lucchinetti FC. Acute disseminated encephalo-myelitis: current understanding and controversies. Semin Neurol. 2008;28:84–94.
  23. M. Alexander and J. M. K. Murthy , Acute disseminated encephalomyelitis: Treatment guidelines. Ann Indian Acad neurol. 2011 July;14(Suppl 1):S60-4
  24. Miller HG, Stanton JB, Gibbons JL : Parainfectious encephalomyelitis and related syndromes. QJM 1956; 25 : 427-505.  
  25. Schwaz S, Mohar A, Knauth M et al : Acute disseminated encephalomyelitis: a follow-up study of 40 patients. Neurology 2001; 56 : 1383-1318.
  26. Hynson JL, Kornberg AJ, Coleman LT et al : Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001; 56 : 1257-1260.
  27. Murthy JMK, Yangala R, Meena AK et al: Clinical, electrophysiological and magnetic resonance imaging study of acute disseminated encephalomyelitis. J Assoc Physicians India 1999; 47 : 280-283.

Corresponding Author

Dr Suprit Basu

H-1/33 Sarsuna Satellite Township, Kolkata-700061, India