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01-07-2019 | Magnetic Resonance Imaging | Original Communication

Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions

Authors: Xavier Ayrignac, Valérie Rigau, Benoit Lhermitte, Thierry Vincent, Nicolas Menjot de Champfleur, Clarisse Carra-Dalliere, Mahmoud Charif, Nicolas Collongues, Jérôme de Seze, Sonia Hebbadj, Guido Ahle, Hélène Oesterlé, François Cotton, Françoise Durand-Dubief, Romain Marignier, Sandra Vukusic, Frédéric Taithe, Mikael Cohen, Anne-Marie Guennoc, Anne Kerbrat, Gilles Edan, Béatrice Carsin-Nicol, Thibaut Allou, Denis Sablot, Eric Thouvenot, Aurélie Ruet, Laurent Magy, Marie-Paule Boncoeur-Martel, Pierre Labauge, Stéphane Kremer

Published in: Journal of Neurology | Issue 7/2019

Abstract

Background

The diagnosis of atypical inflammatory demyelinating lesions can be difficult. Brain biopsy is often required to exclude neoplasms. Moreover, the relationship between these lesions and multiple sclerosis and NMOSD is not clear.

Objectives

Our objectives were to describe radiological and pathological characteristics of patients with acute inflammatory demyelinating lesions.

Methods

We retrospectively identified patients with brain biopsy performed for diagnostic uncertainty revealing a demyelinating lesion. A complete clinical, biological, radiological and pathological analysis was performed.

Results

Twenty patients (15 with a single lesion) were included. MRI disclosed a wide range of lesions including infiltrative lesions (40%), ring-like lesion (15%) Baló-like lesion (15%) and acute haemorrhagic leukoencephalitis (20%). In spite of a marked heterogeneity, some findings were common: a peripheral B1000 hyperintense rim (70%), a slight oedema with mild mass effect (75%) and an open-rim peripheral enhancement (75%). Histopathology revealed that all cases featured macrophages distributed throughout, extensive demyelination, axonal preservation and absence of haemorrhagic changes. In the majority of cases, macrophages were the predominant inflammatory infiltrate and astrocytes were reactive and dystrophic. Aquaporin-4 staining was systematically preserved. After a mean follow-up of 5 years (1–12), 16/20 patients had a diagnosis of monophasic acute atypical inflammatory demyelinating lesion. One patient was diagnosed with MS and 3 with AQP4 negative NMOSD.

Discussion

Although imaging findings in patients with atypical inflammatory demyelinating lesions are heterogeneous, some common features such as peripheral DWI hyperintense rim with open-rim enhancement and absence of oedema argue in favour of a demyelinating lesion and should preclude a brain biopsy. In this context, AQP4 staining is systematically preserved and argues against an AQP4-positive NMOSD. Moreover, long-term follow-up is characterized by low recurrence rate.

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Title: Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions
Authors: Xavier Ayrignac
Valérie Rigau
Benoit Lhermitte
Thierry Vincent
Nicolas Menjot de Champfleur
Clarisse Carra-Dalliere
Mahmoud Charif
Nicolas Collongues
Jérôme de Seze
Sonia Hebbadj
Guido Ahle
Hélène Oesterlé
François Cotton
Françoise Durand-Dubief
Romain Marignier
Sandra Vukusic
Frédéric Taithe
Mikael Cohen
Anne-Marie Guennoc
Anne Kerbrat
Gilles Edan
Béatrice Carsin-Nicol
Thibaut Allou
Denis Sablot
Eric Thouvenot
Aurélie Ruet
Laurent Magy
Marie-Paule Boncoeur-Martel
Pierre Labauge
Stéphane Kremer
Publication date: 01-07-2019
Publisher: Springer Berlin Heidelberg
Keywords: Magnetic Resonance Imaging
Magnetic Resonance Imaging
Multiple Sclerosis
Neuromyelitis Optica Spectrum Disease
Published in: Journal of Neurology / Issue 7/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09328-7

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions

Abstract

Background

Objectives

Methods

Results

Discussion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Objectives

Methods

Results

Discussion

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