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Pathologic-MRI Findings in Atypical IIDD (IIDD)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT03121105
Recruitment Status : Completed
First Posted : April 19, 2017
Last Update Posted : May 7, 2018
Strasbourg University Hospital
Rennes University Hospital
University Hospital, Bordeaux
University Hospital, Limoges
Hospices Civils de Lyon
Nimes University Hospital
Colmar Hospital
Perpignan Hospital
Information provided by (Responsible Party):
University Hospital, Montpellier

Brief Summary:
Our objective is to describe the pathologic and MRI findings in a series of patients with presumed demyelinating lesion of the central nervous system.

Condition or disease
Idiopathic Inflammatory Demyelinating Disorders of the Central Nervous System

Detailed Description:

Idiopathic inflammatory demyelinating disorders (IIDD) are a group of diseases with distinct clinical and magnetic resonnance imaging (MRI) features, the most frequent being Multiple Sclerosis (MS). MS can present with atypical MRI features that can be misleading. Neuromyelitis Optica Spectrum Disorder (NMOSD) is the main differential diagnosis. It has been demonstrated that some patient with NMOSD can also present with brain lesion that is sometimes difficult to diagnose. In this context, a retrospective series identifies 18 patients with centreal nervous system atypical demyelination and 1) pathological evidence of astrocytopathy and 2) immunohistochemistry demonstrating decrease of aqp4 binding. The aim of the study is to describe the radiological and pathological characteristics of a series of patients with pathologicaly proven atypical demyelination that underwent biopsy for diagnostic uncertainties.

This is a retrospective multicenter study. Inclusion criteria are: 1) Acute or subacute onset of neurological deficit, 2) Brain biopsy performed for diagnostic uncertainties revealing an active demyelinating lesion, 3) no known diagnosis of MS or NMOSD at the time of the biopsy and 4) no alternative diagnosis identified during the disease course.

All the medical records of the patients will be reviewed and the following data will be recorded: previous medical history including previous neurologic relapses, gender, age at onset, clinical symptoms at onset and diagnosis at last follow-up according to current diagnosis criteria for MS and NMOSD.

Brain MRI scanners will be analysed. The investigators will mainly focus on T1-, T2-, T2 gradient echo-, fluid- attenuated inversion recovery- and diffusion-weighted images. The following data will be recorded: number of lesions, location (cortical, juxtacortical, juxtaventricular, corpus callosum involvement, posterior fossa involvement), presence of a peripheral hyperintense/hypointense rim (on T2 sequence), and type of gadolinium enhancement (peripheral open or closed ring, central homogeneous or heterogeneous). The presence of oedema will be recorded and mass effect will be analysed. According to the classification of atypical demyelinating lesions (MAGNIMS group), patients will be classified as having either infiltrative, megacystic, balo-like, ring-like lesions or unclassified. Three neuropathologists (BL, BL and VR) blinded to the MRI and clinical datas will perform all the pathologic evaluations. Paraffin-embedded sections have been stained using hematoxylin & eosin, Luxol fast blue. Primary antibodies specific fot GFAP, CD3, CD8, CD20 and CD68 were used in routine practice. Additional immunohistochemical studies will be done using primary antiobodies specific for IgG and Aquaporin-4. The investigators will specifically look at the presence of 1) morphologic features suggestive of either MS (Creutzfeldt cells) or NMOSD (dystrophic astrocytes, myelin vacuolation, vascular hyalinization) 2) negative aquaporin-4 staining (suggestive of NMOSD) and 3) macrophages containing GFAP positive or Luxol fast blue positive debris.

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Study Type : Observational
Actual Enrollment : 25 participants
Observational Model: Cohort
Time Perspective: Retrospective
Official Title: A Pathologic-MRI Reappraisal of Patients With Atypical Idiopathic Inflammatory Demyelinating Disorders
Actual Study Start Date : January 1, 2017
Actual Primary Completion Date : December 1, 2017
Actual Study Completion Date : December 31, 2017

Primary Outcome Measures :
  1. pathological [ Time Frame: 1 day ]
    pathological results: morphologic changes and specific immunostaining

Secondary Outcome Measures :
  1. MRI (magnetic resonnance Imaging) [ Time Frame: 1 day ]
    MRI results: characteristics of the MRI lesions

Information from the National Library of Medicine

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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Probability Sample
Study Population
25 patients with cerebral MRI lesions suggestive of demyelinating lesions

Inclusion Criteria:

  • Presence of brain lesion in MRI suggestive of demyelinating lesions
  • Biopsy analysis revealed active inflammation with active demyelination
  • No known diagnosis of MS or Devic's disease or other diagnosis

Exclusion Criteria:

- NO

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT03121105

Sponsors and Collaborators
University Hospital, Montpellier
Strasbourg University Hospital
Rennes University Hospital
University Hospital, Bordeaux
University Hospital, Limoges
Hospices Civils de Lyon
Nimes University Hospital
Colmar Hospital
Perpignan Hospital
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Study Director: xavier AYRIGNAC, MD University Hospital, Montpellier

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Responsible Party: University Hospital, Montpellier Identifier: NCT03121105     History of Changes
Other Study ID Numbers: RECHMPL17_0078
First Posted: April 19, 2017    Key Record Dates
Last Update Posted: May 7, 2018
Last Verified: March 2017
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: Undecided
Plan Description: NC

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by University Hospital, Montpellier:
Idiopathic Inflammatory demyelinating disorders of the central nervous system
Multiple Sclerosis
Neuromyelitis Optica
Additional relevant MeSH terms:
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Demyelinating Diseases
Central Nervous System Diseases
Nervous System Diseases