Journal of Neurology

, Volume 255, Issue 1, pp 1–10 | Cite as

MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain

A review of reported findings
  • A. Seewann
  • C. Enzinger
  • M. Filippi
  • F. Barkhof
  • A. Rovira
  • A. Gass
  • D. Miller
  • X. Montalban
  • A. Thompson
  • T. Yousry
  • M. Tintore
  • N. de Stefano
  • J. Palace
  • M. Rovaris
  • C. Polman
  • F. Fazekas
  • for the MAGNIMS network
REVIEW
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Abstract

Background

Idiopathic inflammatory demyelinating lesions (IIDL) of the brain usually present with a morphologic pattern characteristic of multiple sclerosis (MS). Atypical appearances of IIDLs also exist, however, and can pose significant diagnostic problems and uncertainty regarding prognosis and adequate therapy. We attempted to improve upon this situation by reviewing the literature.

Methods

We performed a PubMed search from January 1984 through December 2004 for articles in English reporting on IIDLs which had been considered as morphologically atypical (66 articles; 270 cases reported). From these publications 69 individual patient reports allowed the extraction of adequate information on magnetic resonance imaging (MRI) and associated disease characteristics.

Results

Reported atypical IIDLs most frequently manifested as large ring-like lesions (n = 27) which are now considered quite suggestive of an antibodymediated form of MS. Truly atypical IIDLs were less common and exhibited appearances which we termed megacystic (n = 8), Balolike (n = 11) and diffusely infiltrating (n = 11). Despite limitations imposed by the absence of original data the inter-rater agreement in defining these subtypes of atypical IIDLs was moderate to substantial (kappa 0.48–0.68) and we noted trends for their association with certain demographic, clinical and paraclinical variables.

Interpretation

We suggest that IIDLs reported as atypical in the literature can be segregated into several distinct subtypes based on their MRI appearance. The recognition of these patterns may be useful for the differential diagnosis and for a future classification. Because of the limitations inherent in our review this will have to be confirmed by a prospective registry.

Key words

magnetic resonance imaging idiopathic inflammatory demyelinating disorders atypical multiple sclerosis 
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Copyright information

© Steinkopff-Verlag 2007

Authors and Affiliations

  • A. Seewann
    • 1
    • 12
  • C. Enzinger
    • 1
  • M. Filippi
    • 2
  • F. Barkhof
    • 3
  • A. Rovira
    • 4
  • A. Gass
    • 5
  • D. Miller
    • 6
  • X. Montalban
    • 7
  • A. Thompson
    • 8
  • T. Yousry
    • 9
  • M. Tintore
    • 7
  • N. de Stefano
    • 10
  • J. Palace
    • 11
  • M. Rovaris
    • 2
  • C. Polman
    • 12
  • F. Fazekas
    • 1
  • for the MAGNIMS network
  1. 1.Dept. of NeurologyMedical University of GrazGrazAustria
  2. 2.Neuroimaging Research Unit, Dept. of NeurologyScientific Institute and University, Ospedale San RaffaeleMilanItaly
  3. 3.Dept. of RadiologyVU University Medical CentreAmsterdamThe Netherlands
  4. 4.Dept. of RadiologyHospitals Vall d'HebronBarcelonaSpain
  5. 5.Universitätsklinikum BaselBaselSwitzerland
  6. 6.Dept. of NeuroinflammationInstitute of Neurology, University College LondonLondonUK
  7. 7.Unitat de Neuroimmunologia ClinicaHospitals Vall d'HebronBarcelonaSpain
  8. 8.Dept. of NeurorehabilitationInstitute of Neurology, University College LondonLondonUK
  9. 9.Dept. of RadiologyInstitute of Neurology, University College LondonLondonUK
  10. 10.Dept. of Neurological and Behavioural SciencesUniversity of SienaSienaItaly
  11. 11.Dept. of Clinical NeurologyUniversity of OxfordOxfordUK
  12. 12.Dept. of NeurologyVU University Medical CentreAmsterdamThe Netherlands

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