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Pediatric multiple sclerosis with primary progressive course--report of a retrospective cohort study in Iran

Masoud Etemadifar1, Parisa Afzali, Nasim Tabrizi

  • 1Department of Neurology, Medical School, Isfahan University of Medical Sciences, Isfahan, Islamic Republic of Iran.

Neuropediatrics
|December 21, 2012
PubMed

Insights

Primary progressive multiple sclerosis (PPMS) is rare in children, affecting 4.23% of pediatric MS cases in Isfahan. This study details the clinical and MRI features of pediatric PPMS, noting a more progressive course than in adults.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epidemiology

Background:

  • Multiple Sclerosis (MS) is a chronic autoimmune disease affecting the central nervous system.
  • Pediatric-onset MS (POMS) is less common than adult-onset MS, with varied clinical presentations.
  • The primary progressive (PP) subtype of MS is particularly rare in pediatric populations.

Purpose of the Study:

  • To determine the incidence rate of the primary progressive (PP) subtype of pediatric-onset multiple sclerosis (MS) in Isfahan, Iran.
  • To describe the clinical and paraclinical characteristics of pediatric primary progressive multiple sclerosis (PPMS) in this cohort.
  • To compare the disease course and MRI findings of pediatric PPMS with those reported in adult populations.

Main Methods:

  • Retrospective data retrieval from the Isfahan MS Society (IMSS) database.
  • Inclusion criteria: MS patients with onset symptoms before 18 years of age, diagnosed between April 2003 and August 2011.
  • Analysis of demographic data, clinical symptoms (including initial presentation), disease duration, Expanded Disability Status Scale (EDSS), progression index, cerebrospinal fluid (CSF) analysis, and magnetic resonance imaging (MRI) findings.

Main Results:

  • Out of 3,843 MS patients, 260 had pediatric onset; 11 (4.23%) exhibited a PPMS course.
  • Mean age at onset was 16 years, with a female predominance (2.66:1). Ataxia was the most frequent initial symptom.
  • MRI revealed periventricular lesions in all patients and spinal lesions in 9/11. Cerebral lesions were common, potentially more so than in adults. Parental smoking exposure was noted in 7 patients.

Conclusions:

  • Pediatric primary progressive multiple sclerosis (PPMS) is an uncommon subtype of pediatric MS.
  • Cerebral lesions on MRI appear more prevalent in pediatric PPMS compared to adult cases.
  • The PPMS course in children may be more aggressive and progressive than in adults.

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