title: Atypical idiopathic inflammatory demyelinating lesions: prognostic implications and relation to multiple sclerosis.
abstract: 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.
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Mirja
authors.initials: M
authors.lastname: Wallner-Blazek
authors.forename: Alex
authors.initials: A
authors.lastname: Rovira
authors.forename: Massimo
authors.initials: M
authors.lastname: Fillipp
authors.forename: Mara A
authors.initials: MA
authors.lastname: Rocca
authors.forename: David H
authors.initials: DH
authors.lastname: Miller
authors.forename: Klaus
authors.initials: K
authors.lastname: Schmierer
authors.forename: Jette
authors.initials: J
authors.lastname: Frederiksen
authors.forename: Achim
authors.initials: A
authors.lastname: Gass
authors.forename: Hugo
authors.initials: H
authors.lastname: Gama
authors.forename: Charles P
authors.initials: CP
authors.lastname: Tilbery
authors.forename: Antonio J
authors.initials: AJ
authors.lastname: Rocha
authors.forename: José
authors.initials: J
authors.lastname: Flores
authors.forename: Frederik
authors.initials: F
authors.lastname: Barkhof
authors.forename: Alexandra
authors.initials: A
authors.lastname: Seewann
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Palace
authors.forename: Tarek
authors.initials: T
authors.lastname: Yousry
authors.forename: Xavier
authors.initials: X
authors.lastname: Montalban
authors.forename: Christian
authors.initials: C
authors.lastname: Enzinger
authors.forename: Franz
authors.initials: F
authors.lastname: Fazekas
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001921:Brain / Q000473:pathology; D016208:Databases, Factual; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000145:classification / Q000175:diagnosis / Q000473:pathology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000145:classification / Q000175:diagnosis / Q000473:pathology*; D011379:Prognosis; D012737:Sex Factors; D055815:Young Adult