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MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain

A review of reported findings

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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.

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Author notes

  1. A. Seewann

    Present address: Dept. of Neurology, VU University Medical Centre, Amsterdam, The Netherlands

Authors and Affiliations

  1. Dept. of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036, Graz, Austria

    A. Seewann, C. Enzinger & F. Fazekas M.D.

  2. Neuroimaging Research Unit, Dept. of Neurology, Scientific Institute and University, Ospedale San Raffaele, Milan, Italy

    M. Filippi & M. Rovaris

  3. Dept. of Radiology, VU University Medical Centre, Amsterdam, The Netherlands

    F. Barkhof

  4. Dept. of Radiology, Hospitals Vall d'Hebron, Barcelona, Spain

    A. Rovira

  5. Universitätsklinikum Basel, Basel, Switzerland

    A. Gass

  6. Dept. of Neuroinflammation, Institute of Neurology, University College London, London, UK

    D. Miller

  7. Unitat de Neuroimmunologia Clinica, Hospitals Vall d'Hebron, Barcelona, Spain

    X. Montalban & M. Tintore

  8. Dept. of Neurorehabilitation, Institute of Neurology, University College London, London, UK

    A. Thompson

  9. Dept. of Radiology, Institute of Neurology, University College London, London, UK

    T. Yousry

  10. Dept. of Neurological and Behavioural Sciences, University of Siena, Siena, Italy

    N. de Stefano

  11. Dept. of Clinical Neurology, University of Oxford, Oxford, UK

    J. Palace

  12. Dept. of Neurology, VU University Medical Centre, Amsterdam, The Netherlands

    C. Polman

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  1. A. Seewann
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Seewann, A., Enzinger, C., Filippi, M. et al. MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain. J Neurol 255, 1–10 (2008). https://doi.org/10.1007/s00415-007-0754-x

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  • DOI: https://doi.org/10.1007/s00415-007-0754-x

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