ArticleJournal of neurology2019
Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions.
Article in Journal of neurology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 14 citations in OpenAlex.
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- Comparison of Simoa, high‑sensitivity ELISA, and CLIA for serum neurofilament light chain quantification in multiple sclerosis.Scientific reports · 2025Article
- Tumor-like Lesions in Primary Angiitis of the Central Nervous System: The Role of Magnetic Resonance Imaging in Differential Diagnosis.Diagnostics (Basel, Switzerland) · 2024Review
- Immunopathology of Tumefactive Demyelinating Lesions-From Idiopathic to Drug-Related Cases.Frontiers in neurology · 2022Review
- A Comprehensive Updated Review on Magnetic Nanoparticles in Diagnostics.Nanomaterials (Basel, Switzerland) · 2021Review
- Article
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Authors and funding
30 authors at 12 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe 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.
objectivesOur objectives were to describe radiological and pathological characteristics of patients with acute inflammatory demyelinating lesions.
methodsWe retrospectively identified patients with brain biopsy performed for diagnostic uncertainty revealing a demyelinating lesion. A complete clinical, biological, radiological and pathological analysis was performed.
resultsTwenty 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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