Atypical idiopathic inflammatory demyelinating lesions: prognostic implications and relation to multiple sclerosis
Open accessAbstract
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.
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While some patients presenting with atypical demyelinating lesions have a monophasic course, 30-60% develop MS.
“While some patients presenting with atypical demyelinating lesions have a monophasic course, 30-60% develop MS. 38,40”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [38]
The Results report that two-thirds or more of most lesion subgroups had no further attack during follow-up, supporting the possibility of a single-attack course. They also report new MS-typical MRI lesions in 29 of 69 patients (42%), but do not say that 30–60% developed a diagnosis of MS. The distinction between new lesions and an MS diagnosis limits support for that part of the claim. (LLM, conf=0.85)
Experimental — accuracy not yet measuredMRI might help predict outcome; the presence of typical MS lesions and a closed-ring enhancement pattern have been associated with a higher risk of MS.
“MRI might help predict outcome; the presence of typical MS lesions and a closed-ring enhancement pattern have been associated with a higher risk of MS. 38”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [38]
The Discussion (page unavailable) links additional MS-typical MRI lesions to a higher likelihood of having or developing MS. The Results and Discussion also report more subsequent attacks among patients with ring-like lesions, but the copy does not establish that a specifically closed-ring enhancement pattern predicts a higher risk of MS. (LLM, conf=0.87)
Experimental — accuracy not yet measuredOccasionally, MS presents with an atypical demyelinating lesion characterised by large size (>2cm diameter, sometimes with peri-lesional oedema and mass effect), enhancement pattern (open or closed-ring enhancement) or morphology (infiltrative or heterogeneous appearance including concentric rings).
“Occasionally, MS presents with an atypical demyelinating lesion characterised by their large size (>2cm diameter, sometimes with peri-lesional oedema and mass effect), enhancement pattern (open or closed-ring enhancement) or morphology (infiltrative or heterogeneous appearance including concentric rings).[38][39][40]”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [38]
The Introduction (page not available in our copy) says atypical demyelinating lesions can occur at the onset or during the course of relapsing-remitting disease and describes infiltrative, Baló-like and ring-like subtypes. It does not establish the claimed >2 cm threshold, peri-lesional oedema or mass effect, open versus closed-ring enhancement, or heterogeneous appearance. (LLM, conf=0.86)
Experimental — accuracy not yet measured
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Cites
MRI criteria for MS in patients with clinically isolated syndromes
Montalban, X., Tintoré, M., Swanton, J., Barkhof, F., Fazekas, F., Filippi, M., Frederiksen, J., Kappos, L., Palace, J., Polman, C., et al. (2010) · Neurology · doi:10.1212/wnl.0b013e3181cec45c · Cited by 252 · see citing works
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Research works printed but not linked: 15
- Polman C, Reingold S, Edan G, Filippi M, Hartung H, Kappos L, et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald criteria". Ann Neurol. 2005;58:840-6.
- Filippi M, Rocca M, Arnold D, Bakshi R, Barkhof F, De Stefano N, et al. EFNS guidelines on the use of neuroimaging in the management of multiple sclerosis. Eur J Neurol. 2006 Apr 2006;13(4):313-25.
- Rovira Cañellas A, Rovira Gols A, Río Izquierdo J, Tintoré Subirana M, Montalban Gairin X. Idiopathic inflammatory-demyelinating diseases of the central nervous system. Neuroradiology. 2007 May 2007;49(5):393-409.
- Seewann A, Enzinger C, Filippi M, Barkhof F, Rovira A, Gass A, et al. MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain : A review of reported findings. J Neurol. 2008;255(1):1-10.
- Poser C, Brinar V. The nature of multiple sclerosis. Clin Neurol Neurosurg. 2004;106(3):159-71.
- Pichiecchio A, Tavazzi E, Maccabelli G, Precupanu CM, Romani A, Roccatagliata L, et al. What insights have new imaging techniques given into aggressive forms of MS--different forms of MS or different from MS? Mult Scler. 2009 Mar;15(3):285-93.
- Kurtzke J. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983;33:1444-52.
- Llufriu S, Pujol T, Blanco Y, Hankiewicz K, Squarcia M, Berenguer J, et al. T2 hypointense rims and ring-enhancing lesions in MS. Mult Scler. 2010 Nov;16(11):1317-25.
- Chaodong W, Zhang KN, Wu XM, Gang H, Xie XF, Qu XH, et al. Balo's disease showing benign clinical course and co-existence with multiple sclerosis-like lesions in Chinese. Mult Scler. 2008 Apr;14(3):418-24.
- Altintas A, Petek B, Isik N, Terzi M, Bolukbasi F, Tavsanli M, et al. Clinical and radiological characteristics of tumefactive demyelinating lesions: follow-up study. Mult Scler. 2012 Oct;18(10):1448-53.
- Enzinger C, Strasser-Fuchs S, Ropele S, Kapeller P, Kleinert R, Fazekas F. Tumefactive demyelinating lesions: conventional and advanced magnetic resonance imaging. Mult Scler. 2005 Apr 2005;11(2):135-9.
- Cha S, Pierce S, Knopp EA, Johnson G, Yang C, Ton A, et al. Dynamic contrast-enhanced T2*-weighted MR imaging of tumefactive demyelinating lesions. AJNR Am J Neuroradiol. 2001 Jun-Jul;22(6):1109-16.
- Malhotra HS, Jain KK, Agarwal A, Singh MK, Yadav SK, Husain M, et al. Characterization of tumefactive demyelinating lesions using MR imaging and in-vivo proton MR spectroscopy. Mult Scler. 2009 Feb;15(2):193-203.
- Kiriyama T, Kataoka H, Taoka T, Tonomura Y, Terashima M, Morikawa M, et al. Characteristic neuroimaging in patients with tumefactive demyelinating lesions exceeding 30 mm. J Neuroimaging. 2011 Apr;21(2):e69-77.
- Saini J, Chatterjee S, Thomas B, Kesavadas C. Conventional and advanced magnetic resonance imaging in tumefactive demyelination. Acta Radiol. 2011 Dec 1;52(10):1159-68.
Cited by
Sorted by citations
Diagnosis of multiple sclerosis: progress and challenges
Brownlee, W. J., Hardy, T. A., Fazekas, F., & Miller, D. H. (2017) · The Lancet · doi:10.1016/s0140-6736(16)30959-x · Cited by 701 · see citing works
Self-citationAtypical inflammatory demyelinating syndromes of the CNS
Hardy, T. A., Reddel, S. W., Barnett, M. H., Palace, J., Lucchinetti, C. F., & Weinshenker, B. G. (2016) · The Lancet Neurology · doi:10.1016/s1474-4422(16)30043-6 · Cited by 161 · see citing works
Self-citation
- Authors
- Wallner-Blazek, M., Rovira, A., Fillipp, M., Rocca, M. A., Miller, D. H., Schmierer, K., Frederiksen, J., Gass, A., Gama, H., Tilbery, C. P., Rocha, A. J., Flores, J., Barkhof, F., Seewann, A., Palace, J., Yousry, T., Montalban, X., Enzinger, C., & Fazekas, F.
- Affiliations
- Medical University of Graz (AT)
- IRCCS Ospedale San Raffaele (IT)
- National Hospital for Neurology and Neurosurgery (GB)
- Queen Mary University of London (GB)
- University of Copenhagen (DK)
- University Hospital of Basel (CH)
- Irmandade da Santa Casa de Misericórdia de São Paulo (BR)
- Instituto Nacional de Neurología y Neurocirugía (MX)
- Amsterdam UMC Location Vrije Universiteit Amsterdam (NL)
- University of Oxford (GB)
- UCL Queen Square Institute of Neurology (GB)
- Corresponding author
- Fazekas, Franz
- ORCID
- Venue
- Journal of Neurology
- Published
- 2013-04-26
- Volume
- 260
- Issue
- 8
- Pages
- 2016-2022
- ISSN
- 0012-1037 (ISSN-L)
- 0340-5354
- 1432-1459
- OpenAlex
- W2145118705
- Cited by
- 84
- References
- 1
- Citations by year
- 2026: 3 · 2025: 3 · 2024: 9 · 2023: 7 · 2022: 3 · 2021: 15 · 2020: 4 · 2019: 6 · 2018: 13 · 2017: 8 · 2016: 4 · 2015: 5 · 2014: 4
- FWCI
- 3.25
- Type
- journal article
- Language
- en
- Update notices
- correction (2014-03-07)
- Open access
- green
- qmro.qmul.ac.uk
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Journal of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (publishedVersion)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (publishedVersion)
- Queen Mary Research Online (Queen Mary University of London) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Archivio Istituzionale della Ricerca - Università Vita-Salute San Raffaele (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Funders
- National Institute for Health and Care Research (NF-SI-0508-10058)
- Medical Research Council (MR/K000608/1)
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Ophthalmology › Ocular Diseases and Behçet’s Syndrome
- Keywords
- multiple sclerosis, infiltrative lesions, lesion classification, prognosis, clinical relapse, MRI lesion characteristics, demyelinating disease
- MeSH terms
- Adolescent, Adult, Age Factors, Brain, Female, Follow-Up Studies, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Multiple Sclerosis, Prognosis, Sex Factors, Databases, Factual, Disease Progression, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Young Adult
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