The Journal of Pediatrics
Volume 149, Issue 1 , Page 8, July 2006

Multiple sclerosis in children

  • Lauren B. Krupp, MD

      Affiliations

    • Corresponding Author InformationReprint requests: Lauren B. Krupp, MD, State University of New York, Department of Neurology, Health Science Center T12-020, Stony Brook, NY 11794-8121.

Director, National Pediatric MS Center, Department of Neurology, State University of New York, Stony Brook, NY

Article Outline

Abbreviations:  ADEM, Acute disseminated encephalomyelitis , CNS, Central nervous system , MRI, Magnetic resonance imaging , MS, Multiple sclerosis

 

Multiple sclerosis (MS) is an acquired immune-mediated demyelinating disorder of the nervous system that typically develops during early to mid-adulthood. However, it is estimated that 2% to 5% of all MS cases have onset before age 16 years.1, 2 For adults, prompt and accurate diagnosis of MS is frequently followed by initiation of treatment that might modify the subsequent course of disease. In fact, even in patients at high risk for MS, earlier rather than delayed initiation of treatment could favorably affect development of the disease.3

See related article, p 125

The availability of treatment increases the need for a swift and accurate diagnosis in children as well as in adults. Since the side effect profile of disease modifying therapy appears similar in children as in adults,4 more clinicians will want to offer treatment options to MS patients in the pediatric age range. Hence, tests which point to the possible diagnosis early in the course are valuable.

In this issue, Pohl et al5 demonstrate that multivodal evoked potentials can provide critical data needed for the diagnosis of MS. The authors found that evoked potential testing in 85 children presenting with a monofocal, clinically isolated syndrome demonstrated additional anatomic site involvement of the central nervous system (CNS). Such findings implicate involvement of the CNS in areas beyond those indicated by the history or neurological examination. Evidence for a disseminated disease process increases the likelihood of MS.

The diagnosis of MS rests on demonstration of dissemination in time and space of acute episodes of inflammatory CNS dysfunction that cannot be attributed to other systemic or neurologic disease. Dissemination in space refers to discrete regions of the CNS affected by the disease that can be defined by findings on neurologic examination.6

Neuroimaging criteria for adults with MS have been developed to facilitate the diagnosis.7 Magnetic resonance imaging (MRI) criteria specific for children with MS also would lead to an earlier diagnosis.8, 9 However, analysis of other paraclinical tests, such as evoked potential testing, is also of value. MRI testing in children can be complicated by the need for conscious sedation, which is associated with increased risk and might not always be available.

The demonstration by Pohl et al that multivodal evoked potentials can demonstrate multiple CNS areas affected by the demyelinating process provides critical data necessary for the dissemination in space criterion of the MS diagnosis. However, finding multifocal abnormalities with evoked potentials does not exclude other diagnoses (eg, acute disseminated encephalomyelitis, ADEM) and does not satisfy the dissemination in time criterion necessary for the MS diagnosis. Unfortunately, no biological marker has yet been identified which can determine whether an initial inflammatory demyelinating event represents a self limited disorder such as ADEM or constitutes the first episode of an on-going chronic and recurrent disease such as MS. Although ADEM and MS differ with respect to typical clinical, neuroimaging, and laboratory findings, many features of these disorders overlap. Only the subsequent clinical course reliably distinguishes ADEM from MS.

Fortunately, our understanding of pediatric MS and the ability to make the diagnosis more confidently and sooner is improving. Establishing that evoked potential testing can demonstrate multifocal CNS involvement in children with a single clinical event will be useful in diagnosing MS earlier than might be possible otherwise. Increasing the rapidity of MS diagnosis will enable clinicians to promptly initiate treatment to control the disease process and will also benefit families by providing a definitive answer, allowing early planning and adjustment to the diagnosis.

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References 

  1. Duquette P , Murray TJ , Pleines J , et al.   Multiple sclerosis in childhood (clinical profile in 125 patients) . J Pediatr . 1987;111:359–363
  2. Ghezzi A , Deplano V , Faroni J , et al.   Multiple sclerosis in childhood (clinical features of 149 cases) . Mult Scler . 1997;3:43–46
  3. Jacobs LD , Beck RW , Simon JH , Kinkel RP , Brownscheidle CM , Murray TJ , et al.   Intramuscular interferon beta-1a therapy initiated during a first demyelinating event in multiple sclerosis . N Engl J Med . 2000;43:898–904
  4. Pohl D , Rostasy K , Gartner J , Hanefeld F . Treatment of early-onset multiple sclerosis with subcutaneous interferon beta-1a . Neurology . 2005;64:888–890
  5. Pohl D , Rostasy K , Treiber-Held S , Brockmann K , Gärtner J , Hanefeld F . Pediatric multiple sclerosis (detection of clinically silent lesions by multivodal evoked potentials) . J Pediatr . 2006;149:125–127
  6. Poser CM , Paty DW , Scheinberg L , et al.   New diagnostic criteria for multiple sclerosis (guidelines for research protocols) . Ann Neurol . 1983;13:227–231
  7. McDonald WI , Compston A , Edan G , et al.   Recommended diagnostic criteria for multiple sclerosis (guidelines from the International Panel on the Diagnosis of Multiple Sclerosis) . Ann Neurol . 2001;50:121–127
  8. Mikaeloff Y , Suissa S , Vallee L , et al.   First episode of acute CNS inflammatory demyelination in childhood (prognostic factors for multiple sclerosis and disability) . J Pediatr . 2004;144:246–252
  9. Hahn CD , Shroff MM , Blaser SI , Banwell BL . MRI criteria for multiple sclerosis (evaluation in a pediatric cohort) . Neurology . 2004;62:806–808

PII: S0022-3476(06)00482-3

doi:10.1016/j.jpeds.2006.05.037

Refers to article:

  • Pediatric multiple sclerosis: Detection of clinically silent lesions by multimodal evoked potentials

    Daniela Pohl, Kevin Rostasy, Stephanie Treiber-Held, Knut Brockmann, Jutta Gärtner, Folker Hanefeld
    The Journal of Pediatrics July 2006 (Vol. 149, Issue 1, Pages 125-127)

The Journal of Pediatrics
Volume 149, Issue 1 , Page 8, July 2006

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