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CASE REPORT
Year : 2010  |  Volume : 58  |  Issue : 3  |  Page : 452-456

Inflammatory demyelinating disorders of childhood: Experience with six children


1 Department of Pediatrics, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India
2 Department of Neurology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India

Date of Acceptance01-Feb-2010
Date of Web Publication17-Jul-2010

Correspondence Address:
Arathi Srinivasan
Kanchi Kamakoti CHILDS Trust Hospital, 12-A, Nageswara Road, Nungambakkam, Chennai - 600 034
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.65751

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 » Abstract 

Demyelinating disorders of the central nervous system (CNS) in children pose diagnostic and prognostic difficulties in clinical practice. In this report, we describe our experience with inflammatory demyelinating disorders of CNS in six children, classified as per the proposed criteria by the Pediatric Multiple Sclersois Study Group. We emphasize the importance of appropriate diagnosis and follow-up to distinguish transient inflammatory demyelinating diseases from chronic inflammatory demyelinating diseases.


Keywords: Acute disseminated, demyelinations, encephalomyelitis, encephalopathy, multiple sclerosis


How to cite this article:
Srinivasan A, Sankar J, Ganapathi K. Inflammatory demyelinating disorders of childhood: Experience with six children. Neurol India 2010;58:452-6

How to cite this URL:
Srinivasan A, Sankar J, Ganapathi K. Inflammatory demyelinating disorders of childhood: Experience with six children. Neurol India [serial online] 2010 [cited 2021 Sep 27];58:452-6. Available from: https://www.neurologyindia.com/text.asp?2010/58/3/452/65751



 » Introduction Top


Acute disseminated encephalomyelitis (ADEM) is defined as a first clinical event presenting as a polysymptomatic encephalopathy with acute or sub acute onset, showing focal or multifocal hyper intense lesions predominantly affecting central nervous system (CNS) white matter. There should be no previous clinical episode or magnetic resonance imaging (MRI) evidence of demyelinating event. [1] Though classically ADEM is a monophasic illness, variants such as recurrent and multiphasic ADEM have been described. Recurrent ADEM implies a new demyelinating event occurring within three months after the initial event or at least four weeks after completing steroids with a recapitulation of prior illness, where as multiphasic ADEM differs in involving new areas of the brain. [1] ADEM per se has a favorable outcome but some of them progress to have a chronic demyelinating disease with relapses and remissions. Thus dilemma does exist regarding prognostication and the probability of developing a chronic demyelinating illness in patients with ADEM. Our experience in this case series reveals the difficulties faced during the evaluation of these cases.


 » Case Report Top


Based on the clinical and neuroimaging findings six children with first CNS demyelinating event were identified to have ADEM as per the criteria defined by the International Pediatric Multiple Sclerosis (MS) Study group. All of them underwent cerebrospinal fluid (CSF) analysis and neuroimaging. CSF analysis showed mild increase in proteins in all the six children and mild lymphocytic pleocytosis in one. MRI was abnormal in all the six children revealing hyper intense white matter lesions. All of them were treated with high dose intravenous methyl prednisolone (MPS) for five days followed by oral steroids which was tapered and stopped. There was a good clinical improvement with intravenous steroid and four children had no residual neurological deficit. Two children had a relapse six months later, one of whom was diagnosed to have recurrent ADEM and the other subsequently diagnosed as a case of MS as per the proposed criteria. The details are given in [Table 1] and [Table 2].


 » Discussion Top


At the initial presentation all the six children fulfilled the diagnostic criteria of ADEM as per the consensus definition. [1] Neuroimaging is the investigation of choice in ADEM, [2] lesions are typically large, multifocal, hyper intense and asymmetric involving the white matter. [3] Frequently, typical symmetrical grey matter involvement of thalami and basal ganglia are also seen. [4],[5] Sequential MRI plays an important role in confirming the diagnosis of ADEM which may show either partial or complete resolution of the lesions. [2] All our patients showed typical lesions of ADEM on MRI [Figure 1]. Full recovery has been reported in 50% to 80% of patients with the use of high dose intravenous methyl prednisolone for five days followed by oral steroid tapering over four to six weeks. [5],[6],[7] All the six children in our case series showed a rapid recovery with good neurological improvement on steroids. Though good recovery is often the case, subtle neurocongnitive deficits can be seen in these children on longterm follow-up. [8]

Clinically differentiating the first episode of MS from ADEM is often difficult. Diagnosis of pediatric MS requires multiple episodes of CNS demyelination separated in time and space with "positive MRI". [9] As per the pediatric MS study group, the second demyelinating event not meeting the criteria for recurrent or multiphasic ADEM would qualify for the initial event for pediatric MS and new T2 MRI lesions developing at least three months from the second event or a new clinical event developing at least three months subsequent to the second event are required for the diagnosis of pediatric MS. [1] In this series one of the child (Case 5), during the follow-up had met these criteria to be reclassified as a case of pediatric MS.

The McDonald diagnostic criteria for MS proposed in 2001 [9] have been revised and the imaging requirements have been liberalized. [10] However, the adult-based MRI criteria regarding dissemination in space are not sensitive in children. In view of differences in the distribution of lesions in children, specific modifications are proposed to this adult-based MRI criteria for better diagnostic utility in children. [11] To diagnose pediatric MS, at least two of the three criteria have to fulfilled: (1) ≥ 5 T2 lesions (2) ≥ 2 periventricular lesions (3) ≥ 1 brainstem lesion. Recently, MRI criteria have been proposed to differentiate the first attack of MS from ADEM. [12] When these criteria were applied in these six children, one child (Case 5) satisfied the criteria for the diagnosis of MS [Table 2]. These criteria require evaluation in a large sample of children for validation.

To summarize, in children it is difficult to differentiate ADEM and MS at the initial presentation. The major inflammatory demyelinating disorders of CNS in childhood are summarized in [Table 3]. [1] An approach to the childhood demyelinating disorders based on the recommendations by the Pediatric MS Study Group is given in [Figure 2]. [1]

 
 » References Top

1.Krupp LB, Banwell B, Tenembaum S; for the International Pediatric MS Study Group. Consensus definitions proposed for pediatric multiple sclerosis. Neurology 2007;68:S7-12.  Back to cited text no. 1      
2.Tenembaum S, Chitnis T, Ness J, Hahn JS; for the International Pediatric MS Study Group. Acute Disseminated Encephalomyelitis. Neurology 2007;68:S23-36.   Back to cited text no. 2      
3.Wingerchuck DM. Postinfectious encephalomyelitis. Curr Neurol Neurosci Rep 2003;3:256-64.  Back to cited text no. 3      
4.Baum PA, Barkovich AJ, Koch TK, Berg BO. Deep grey matter involvement in children with acute disseminating encephalomyelitis. AJNR Am J Neuroradiol 1994;15:1275-83.  Back to cited text no. 4  [PUBMED]    
5.Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long term follow-up study of 84 pediatric patients. Neurology 2002;59:1224-31.   Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001;56:1308-12.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis,multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000;123:2407-22.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Hahn CD, Miles BS, MacGregor DL, Blaser SI, Banwell BL, Hetherington CR. Neurocognitive outcome after acute disseminated encephalomyelitis. Pediatr Neurol 2003;29:117-23.   Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121-7.  Back to cited text no. 9  [PUBMED]    
10.Polman HC, Reingold CS, Gilles E, Filippi M, Hartung HP, Kappos L, et al. Diagnostic Criteria for Multiple Sclerosis: 2005 Revisions to the "McDonald Criteria". Ann Neurol 2005;58:840-6.  Back to cited text no. 10      
11.Callen DJ, Shroff MM, Branson HM, Lotze T, Li DK, Stephens D, et al. MRI in the diagnosis of pediatric multiple sclerosis. Neurology 2009;72:961-7.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Callen DJ, Shroff M, Branson HM, Li DK, Lotze T, Stephens D, et al. Role of MRI in the differentiation of ADEM from MS in children. Neurology 2009;72:968-73.  Back to cited text no. 12      


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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