Pattern and Outcome of Acute Disseminated Encephalomyelitis (ADEM) in Children

Experience in a Tertiary Center, Upper Egypt

Authors

  • Abdelrahim Abdrabou Sadek Assistant Professor and head of Pediatric Neurology Unit, Pediatric Department, Faculty of Medicine, Sohag University, Sohag, Egypt

Keywords:

Acute disseminated encephalomyelitis, ADEM, Egypt

Abstract

Introduction: Acute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the brain. Although it occurs in all ages, most reported cases are in children and adolescents. The aims of this study were to study the clinical pattern and outcome of ADEM in children in a tertiary center in Upper Egypt and to determine the effect of combined use of steroids and IVIg on outcome.  

Methods: This observational study was carried out from January 2014 through December 2014 in the Pediatric Department of Sohag University Hospital (Egypt). All children diagnosed as ADEM during a one year period were included in this study. The treatments used were IV methylprednisolone followed by oral prednisone taper and intravenous immunoglobulin for severe cases. All studied cases were followed up and reevaluated at three months and six months. We used SPSS version 10 and Chi Square, Spearman’s test and t-test for data analysis.

Results: Eighteen children were included in this study (10 males and 8 females), the average age was 5.5 ± 0.9 years. Prodroma was found in 72.22% of the cases while the main complaint was encephalopathy (83.33%) followed by seizures (11.11%). The neurological findings were convulsions in 83.33%, quadriparesis (33.33%), hemiparesis (33.33), bladder involvement (both retention and incontinence) in 61.11%, and cranial nerve affection (11.11%). Demyelination patches were multifocal in 50%, mainly subcortical in 27.78%.  Intelligence quotient (IQ) assessment after 6 months follow up showed that 50% were below average, 25% had mild MR while neurological evaluation showed that 75% of our patients were completely cured. The predictors of better outcome were; children related to the age group (1-4 years) (p = 0.01), children with higher GCS (6-14) (p = 0.01), and children who received steroids on the first day of symptoms and intravenous immunoglobulin in the first week (p = 0.03). 

Conclusion: The clinical pattern of acute disseminated encephalomyelitis is variable, and a disturbed level of consciousness was the most common presentation. The outcome is generally favorable although motor deficit and cognitive impairment were reported. The combined use of steroids and IVIg has substantial effect on the outcome in children with ADEM.

 

References

Dale RC. Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 2003; 14(2): 90-5. doi:

1053/spid.2003.127225. PMID: 12881796.

Banwell B, Kennedy J, Sadovnick D, Arnold DL, Magalhaes S, Wambera K, et al. Incidence of acquired

demyelination of the CNS in Canadian children. Neurology. 2009; 72(3): 232-9. doi:

1212/01.wnl.0000339482.84392.bd. PMID: 19153370.

Stonehouse M, Gupte G, Wassmer E, Whitehouse WP. Acute disseminated encephalomyelitis: recognition

in the hands of general paediatricians. Arch Dis Child. 2003; 88(2): 122-4. doi: 10.1136/adc.88.2.122.

PMID: 12538312, PMCID: PMC1719460.

Kuni BJ, Banwell BL, Till C. Cognitive and behavioral outcomes in individuals with a history of acute

disseminated encephalomyelitis (ADEM). Dev Neuropsychol. 2012; 37(8): 682-96. doi:

1080/87565641.2012.690799. PMID: 23145566.

Brass SD, Caramanos Z, Santos C, Dilenge ME, Lapierre Y, Rosenblatt B. Multiple sclerosis vs acute

disseminated encephalomyelitis in childhood. Pediatr Neurol. 2003; 29(3): 227-31. doi: 10.1016/S0887- 8994(03)00235-2. PMID: 14629906.

Davis LE, Booss J. Acute disseminated encephalomyelitis in children: a changing picture. Pediatr Infect

Dis J. 2003; 22(9): 829-31. doi: 10.1097/01.inf.0000087847.37363.78. PMID: 14506377.

Tenembaum S, Chitnis T, Ness J, Hahn JS. International Pediatric MS Study Group. Acute disseminated

encephalomyelitis. Neurology. 2007; 68(16Suppl2): 23-36. doi: 10.1212/01.wnl.0000259404.51352.7f.

PMID: 17438235.

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. doi: 10.1212/WNL.59.8.1224. PMID:

Shahar E, 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; 17(11): 810-4. doi:

1177/08830738020170111001. PMID: 12585719.

Feasby T, Banwell B, Benstead T, Bril V, Brouwers M, Freedman M, et al. Guidelines on the use of

intravenous immune globulin for neurologic conditions. Transfus Med Rev. 2007; 21(2 Suppl 1): 57-107.

doi: 10.1016/j.tmrv.2007.01.002. PMID: 17397768.

Hahn CD, Miles BS, MacGregor DL, Blaser SI, Banwell BL, Hetherington CR. Neurocognitive outcome

after acute disseminated encephalomyelitis. Pediatr Neurol. 2003; 29(2): 117-23. doi: 10.1016/S0887- 8994(03)00143-7. PMID: 14580654.

Krupp LB, Banwell B, Tenembaum S; International Pediatric MS Study Group. Consensus definitions

proposed for pediatric multiple sclerosis and related disorders. Neurology. 2007; 68(16 Suppl 2): 7-12. doi:

1212/01.wnl.0000259422.44235.a8. PMID: 17438241.

Thorndike RL, Hagen EP, Sattler JM. The Stanford Binet. Intelligence Scale. Fourth ed Chicago:

Riverside. 1987.

Kotby MN, Khairy A, Barakah M, Refaie N, El Shobary A. Language testing of Arabic speaking children.

Process XVIII World Congress Int Assoc Logopedics Phoniatrics, Cairo. 1995: 263-6.

Tenembaum SN. Acute disseminated encephalomyelitis. Handb Clin Neurol. 2013; 112: 1253-62. doi:

1016/B978-0-444-52910-7.00048-9. PMID: 23622336.

Weng WC, Peng SS, Lee WT, Fan PC, Chien YH, Du JC, et al. Acute disseminated encephalomyelitis in

children: one medical center experience. Acta Paediatr Taiwan. 2006; 47(2): 67-71. PMID: 16927630.

Pavone P, Pettoello-Mantovano M, Le Pira A, Giardino I, Pulvirenti A, Giugno R, et al. Acute

disseminated encephalomyelitis: a long-term prospective study and meta-analysis. Neuropediatrics. 2010;

(6): 246-55. doi: 10.1055/s-0031-1271656. PMID: 21445814.

Atzori M, Battistella PA, Perini P, Calabrese M, Fontanin M, Laverda AM, et al. Clinical and diagnostic

aspects of multiple sclerosis and acute monophasic encephalomyelitis in pediatric patients: a single centre

prospective study. Mult Scler. 2009; 15(3): 363-70. doi: 10.1177/1352458508098562. PMID: 18987105.

Alper G, Heyman R, Wang L. Multiple sclerosis and acute disseminated encephalomyelitis diagnosed in

children after long-term follow-up: comparison of presenting features. Dev Med Child Neurol. 2009; 51(6):

-6. doi: 10.1111/j.1469-8749.2008.03136.x. PMID: 19018840, PMCID: PMC2704249.

Panicker JN, Nagaraja D, Kovoor JM, Subbakrishna DK. Descriptive study of acute disseminated

encephalomyelitis and evaluation of functional outcome predictors. J Postgrad Med. 2010; 56(1): 12-6. doi:

4103/0022-3859.62425. PMID: 20393243. 21) Ben Achour N, Ben Waddey O, Kraoua I, Benrhouma H, Klaa H, Rouissi A, et al. Acute disseminated

encephalomyelitis in Tunisia: Report of a pediatric cohort. Rev Neurol (Paris). 2015; 171(12): 882-90. doi:

1016/j.neurol.2015.09.011. PMID: 26573333.

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. doi: 10.1055/s-2003-42208.

PMID: 12973660.

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. PMID:

Jayakrishnan MP, Krishnakumar P. Clinical profile of acute disseminated encephalomyelitis in children. J

Pediatr Neurosci. 2010; 5(2): 111-4. doi: 10.4103/1817-1745.76098. PMID: 21559154, PMCID:

PMC3087985.

Sundar U, Shrivastava MS. Acute disseminated encephalomyelitis--a prospective study of clinical profile

and in-hospital outcome predictors. J Assoc Physicians India. 2012; 60: 21-6. PMID: 22799110.

Govender R, Wieselthaler NA, Ndondo A, Wilmshurst JM. Acquired demyelinating disorders of childhood

in the Western Cape, South Africa. J Child Neurol. 2010; 25(1): 48-56. doi: 10.1177/0883073809336294.

PMID: 19494357.

Christensen PS, Østergaard JR. Acute disseminated encephalomyelitis. Definition, treatment, prognosis and

evidence. Ugeskr Laeger. 2008; 170(21): 1839-42. PMID: 18492453.

Leake JA, Albani S, Kao AS, Senac MO, Billman GF, Nespeca MP, et al. Acute disseminated

encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J. 2004;

(8): 756-64. doi: 10.1097/01.inf.0000133048.75452.dd. PMID: 15295226.

Beatty C, Bowler RA, Farooq O, Dudeck L, Ramasamy D, Yeh EA, et al. Long-Term Neurocognitive,

Psychosocial, and Magnetic Resonance Imaging Outcomes in Pediatric-Onset Acute Disseminated

Encephalomyelitis. Pediatr Neurol. 2016; 57: 64-73. doi: 10.1016/j.pediatrneurol.2016.01.003. PMID:

Shilo S, Michaeli O, Shahar E, Ravid S. Long-term motor, cognitive and behavioral outcome of acute

disseminated encephalomyelitis. Eur J Paediatr Neurol. 2016; 20(3): 361-7. doi:

1016/j.ejpn.2016.01.008.

Published

2022-03-07

Issue

Section

Articles