Atypical inflammatory demyelinating lesions and atypical multiple sclerosis

Rev Neurol (Paris). 2018 Jun;174(6):408-418. doi: 10.1016/j.neurol.2018.03.007. Epub 2018 Apr 16.


Atypical idiopathic inflammatory demyelinating disorders (IIDDs) of the brain have long been known to be disorders closely related to multiple sclerosis (MS), despite having distinctive clinical and radiological characteristics. Originally, they mostly corresponded to acute-onset variants of MS that classically had poor prognoses, such as Baló's concentric sclerosis, Marburg variant of MS and Schilder's disease, and their relationship with MS was based on their shared pathological findings and the co-occurrence of these variants in patients with typical MS. More recently, other atypical disorders, such as solitary sclerosis, have also been described as belonging to the MS spectrum, raising the question of their links with MS. Meanwhile, multiple MS mimics have been described and need to be considered in the differential diagnosis of MS. In addition, thorough characterization of these atypical entities, including advanced MRI and biological studies, is now warranted to further improve their management.

Keywords: Acute hemorrhagic leukoencephalitis; Atypical demyelinating lesions; Balo's concentric sclerosis; Solitary sclerosis; Tumefactive demyelination.

Publication types

  • Review

MeSH terms

  • Demyelinating Diseases / classification
  • Demyelinating Diseases / complications
  • Demyelinating Diseases / diagnosis*
  • Diagnosis, Differential
  • Diffuse Cerebral Sclerosis of Schilder / complications
  • Diffuse Cerebral Sclerosis of Schilder / diagnosis
  • Encephalitis / complications*
  • Encephalitis / diagnosis
  • Humans
  • Magnetic Resonance Imaging
  • Multiple Sclerosis / classification*
  • Multiple Sclerosis / complications
  • Multiple Sclerosis / diagnosis*
  • Multiple Sclerosis / pathology