Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric Sclerosis

Neurology. 2021 Jul 27;97(4):e414-e422. doi: 10.1212/WNL.0000000000012230. Epub 2021 May 19.


Objective: To describe clinical, radiologic, and pathologic features of Baló concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases.

Methods: Retrospective review of BCS cases from US and Australian tertiary care centers.

Results: We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis-suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within 1 month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially nonenhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on apparent diffusion coefficient, diffusion-weighted imaging, and gadolinium-enhanced imaging was observed in 67% (22/33). Aquaporin-4 immunoglobulin G (n = 26) and myelin oligodendrocyte glycoprotein immunoglobulin G (n = 21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3-100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19-49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III).

Conclusions: BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aquaporin 4 / immunology
  • Brain / diagnostic imaging*
  • Child
  • Diffuse Cerebral Sclerosis of Schilder / diagnostic imaging*
  • Diffuse Cerebral Sclerosis of Schilder / drug therapy
  • Diffuse Cerebral Sclerosis of Schilder / pathology
  • Female
  • Humans
  • Immunoglobulin G / blood
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Myelin-Oligodendrocyte Glycoprotein / immunology
  • Neuroimaging
  • Retrospective Studies
  • Young Adult


  • Aquaporin 4
  • Immunoglobulin G
  • Myelin-Oligodendrocyte Glycoprotein