Balo's concentric sclerosis: An atypical demyelinating disease in pediatrics

Mult Scler Relat Disord. 2020 Sep:44:102198. doi: 10.1016/j.msard.2020.102198. Epub 2020 May 22.

Abstract

Background: Baló's concentric sclerosis (BCS) is a rare subtype of tumefactive demyelinating disease with characteristic radiological and pathological features. In the medical literature, less than 10 BCS cases have been reported in the pediatric population.

Case: We report the case of a 5-year-old boy who presented to the emergency department with 2 days of left-sided weakness. Magnetic resonance imaging (MRI) revealed 3 tumefactive lesions; further diagnostic studies included MRI spectroscopy, lumbar puncture and biopsy. A final diagnosis of Baló concentric sclerosis was made. He received intravenous methylprednisolone at 30 mg/kg for 5 days, plasma exchange treatment and immunoglobulin G course (2 g/kg/day). The patient was discharged in good condition and asymptomatic; after 8 months of follow-up, he has not presented with new symptoms.

Conclusion: Baló's concentric sclerosis (BCS) is a rare variant of tumefactive demyelinating disease with only a handful of cases reported in the pediatric population. It poses a diagnostic challenge and therapeutic enigma, since it is difficult to distinguish from a central nervous system (CNS) neoplasm, infection or other CNS lesions on magnetic resonance imaging (MRI). Our case along with those reported in the literature, highlights the importance of considering BCS as a potential differential diagnosis when assessing tumefactive lesions. Distinguishing tumefactive demyelinating lesions from malignancy or infection is critical for proper patient management and to avoid unnecessary medical or surgical interventions.

Keywords: Balo's Concentric Sclerosis; Child; Demyelinating disease; Tumefactive demyelinating lesion.

Publication types

  • Case Reports

MeSH terms

  • Brain / diagnostic imaging
  • Child
  • Child, Preschool
  • Diffuse Cerebral Sclerosis of Schilder* / diagnostic imaging
  • Diffuse Cerebral Sclerosis of Schilder* / therapy
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use
  • Pediatrics*
  • Radiography

Substances

  • Methylprednisolone