Atypical idiopathic inflammatory demyelinating lesions: Prognostic implications and relation to multiple sclerosis

Mirja Wallner-Blazek, Alex Rovira, Massimo Fillipp, Mara A. Rocca, David H. Miller, Klaus Schmierer, Jette Frederiksen, Achim Gass, Hugo Gama, Charles P. Tilbery, Antonio J. Rocha, José Flores, Frederik Barkhof, Alexandra Seewann, Jacqueline Palace, Tarek Yousry, Xavier Montalban, Christian Enzinger, Franz Fazekas

Research output: Contribution to journalArticlepeer-review


Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be classified according to previously suggested radiologic characteristics and how this classification relates to prognosis. Searching the databases of eight tertiary referral centres we identified 90 adult patients (61 women, 29 men; mean age 34 years) with ≥1 AIIDL. We collected their demographic, clinical and magnetic resonance imaging data and obtained follow-up (FU) information on 77 of these patients over a mean duration of 4 years. The AIIDLs presented as a single lesion in 72 (80 %) patients and exhibited an infiltrative (n = 35), megacystic (n = 16), Baló (n = 10) or ring-like (n = 16) lesion appearance in 77 (86 %) patients. Additional multiple sclerosis (MS)-typical lesions existed in 48 (53 %) patients. During FU, a further clinical attack occurred rarely (23-35 % of patients) except for patients with ring-like AIIDLs (62 %). Further attacks were also significantly more often in patients with coexisting MS-typical lesions (41 vs. 10 %, p <0.005). New AIIDLs developed in six (7 %), and new MS-typical lesions in 29 (42 %) patients. Our findings confirm the previously reported subtypes of AIIDLs. Most types confer a relatively low risk of further clinical attacks, except for ring-like lesions and the combination with MS-typical lesions.

Original languageEnglish
Pages (from-to)2016-2022
Number of pages7
JournalJournal of Neurology
Issue number8
Publication statusPublished - Aug 2013


  • Atypical lesions
  • MRI
  • Multiple sclerosis
  • Prognosis
  • Tumefactive lesions

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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