First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability

doi:10.1016/j.jpeds.2003.10.056

The Journal of Pediatrics

Volume 144, Issue 2, February 2004, Pages 246-252

https://doi.org/10.1016/j.jpeds.2003.10.056 Get rights and content

Abstract

Objectives

To evaluate prognostic factors for second attack and for disability in children presenting with an initial episode of central nervous system (CNS) demyelination.

Study design

A cohort of 296 children having a first episode of acute CNS inflammatory demyelination was studied by survival analysis.

Results

The average follow-up was 2.9±3 years. At the end of the follow-up, 57% of patients had a diagnosis of multiple sclerosis (MS), 29% had a monophasic acute disseminated encephalomyelitis, and 14% had a single focal episode. The rate of a second attack was (1) higher in patients with age at onset ≥10 years (hazard ratio, 1.67; 95% CI, 1.04-2.67), MS-suggestive initial MRI (1.54; 1.02-2.33), or optic nerve lesion (2.59; 1.27-5.29); and (2) lower in patients with myelitis (0.23; 0.10-0.56) or mental status change (0.59; 0.33-1.07). Of patients with a second attack, 29% had an initial diagnosis of acute disseminated encephalomyelitis. At the end of the follow-up period, 90% of patients had no or minor disability. Occurrence of severe disability was associated with a polysymptomatic onset (3.25; 1.16-11.01), sequelae after the first attack (26.65; 9.42-75.35), further relapses (1.49; 1.16-1.92), and progressive MS (3.57; 1.21-8.72).

Conclusions

Risk of second attack of CNS demyelination is higher in older patients and lower in patients with mental status change. Risk of disability is higher in polysymptomatic and relapsing patients.

Section snippets

Subjects

The cohort was identified through the French Neuropediatric Society. We included children under 16 years of age who had a first neurologic episode compatible with a first attack or the progressive onset of an inflammatory demyelinating disease of the CNS, identified between January 1985 (the date after which all patients could have MRI at the first attack) and June 2001. Patients were followed up during routine clinical visits from the onset of their condition until December 2001, and the

Results

The cohort included 296 children with a mean follow-up period of 2.9±3 years (median, 1.9 years; range, 0.5 to 14.9), including 61 of 296 (21%) who were followed for more than 5 years. The characteristics of the group and those related to the final diagnosis at the end of the follow-up period are reported in Table I. The age at onset was significantly different among the three groups of patients, patients with ADEM being the youngest. MRI at onset was performed in all patients (brain: 264/296

Discussion

This study applies wide inclusion criteria not related only to ADEM or MS but to a first acute inflammatory event of demyelination of the CNS in childhood, to assess long-term evolution without bias caused by mode of inclusion. To improve the accuracy of data (history before the onset, MRI at the first attack) we limited the date of eligibility and maintained a homogeneous geographic area. The cohort is representative of patients that are referred to neuropediatric centers for a first episode

Acknowledgements

We thank the participants of the KIDMUS study group. We also thank A. Kezouh, PhD, for statistical advice (Mc Gill University, Canada).

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^☆: Supported by grants from the Association pour la Recherche sur la Sclérose En Plaques (ARSEP, France). S. Suissa is the recipient of a Distinguished Senior Scientist award from the Canadian Institute of Health Research (CIHR).

^∗: A complete list of members can be found in the Appendix.

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First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability☆

Abstract

Objectives

Study design

Results

Conclusions

Section snippets

Subjects

Results

Discussion

Acknowledgements

Multiple sclerosis in childhood: clinical profile in 125 patients

J Pediatr

Multiple sclerosis in childhood: clinical features of 149 cases

Mult Scler

Course and prognosis of multiple sclerosis assessed by the computerized data processing of 349 patients

Brain

The natural history of multiple sclerosis: a geographically based study, I: clinical course and disability

Brain

Early onset MS under the age of 16: clinical and paraclinical features

Acta Neurol Scand

Sadovnick D, and the UBC MS Clinic Neurologists. Early onset multiple sclerosis: a longitudinal study

Neurology

Course and prognosis in early-onset MS: comparison with adult-onset forms

Neurology

Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children

Brain

Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children

Neurology

Acute disseminated encephalomyelitis in children

Pediatrics

Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients

Neurology

EDMUS, a European Database for Multiple Sclerosis

J Neurol Neurosurg Psychiatry

New-diagnostic criteria for multiple sclerosis: guidelines for research protocols

Ann Neurol

Recommended diagnostic criteria for multiple sclerosis: guidelines from the international panel on the diagnosis of multiple sclerosis

Ann Neurol

MRI in the diagnosis of MS: a prospective study with comparison of clinical evaluation, evoked potentials, oligoclonal banding, and CT

Neurology