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. 2024 Feb:82:105408.
doi: 10.1016/j.msard.2023.105408. Epub 2024 Jan 5.

Clinical and radiological characteristics and outcomes of patients with recurrent or relapsing tumefactive demyelination

Irin Pervin  1 , Sudarshini Ramanathan  2 , Cecilia Cappelen-Smith  3 , Steve Vucic  1 , Stephen W Reddel  4 , Todd A Hardy  5
Affiliations
Free article

Clinical and radiological characteristics and outcomes of patients with recurrent or relapsing tumefactive demyelination

Irin Pervin et al. Mult Scler Relat Disord. 2024 Feb.
Free article

Abstract

Background: Relapsing or recurrent tumefactive demyelination is rare and has not been studied beyond individual case reports.

Objective: We examined the clinical course, neuroimaging, cerebrospinal fluid (CSF), treatment and outcomes of patients with recurrent tumefactive demyelinating lesions (TDLs).

Methods: We used PubMed to identify reports of recurrent TDLs and included the details of an additional, unpublished patient.

Results: We identified 18 cases (11F, 7 M). The median age at onset of the index TDL was 37 years (range 12-72) and most were solitary lesions 72 % (13/18). CSF-restricted oligoclonal bands (OCBs) were detected in 25 % (4/16). Only one of those tested (n = 13) was positive for AQP4-IgG. A moderate-to-marked treatment response (high dose corticosteroid with or without additional plasmapheresis, IVIg or disease modifying therapies) was evident in 89 % of treated patients. Median EDSS at the median follow-up of 36 months (range 6-144) was 2 (range 1-10). Most remained ambulatory (EDSS < 4 in 13/18), but 1 patient died.

Conclusion: The median age of patients with relapsing TDLs is similar to that of typical MS, but differences include a lower female:male sex ratio, larger lesions, and a comparative lack of CSF-restricted OCBs. Outcomes vary among this group of patients ranging from minimal disability through to death.

Keywords: ADEM; Balo concentric sclerosis; Demyelinating; MOGAD; MRI; Multiple sclerosis; NMOSD; Prognosis; Pseudotumoral; Pseudotumour; Therapy.

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Conflict of interest statement

Declaration of competing interest The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: IP has nothing to disclose. DSR has received research funding from the National Health and Medical Research Council (NHMRC, Australia), the Petre Foundation, the Brain Foundation (Australia), the Royal Australasian College of Physicians, and the University of Sydney. She is supported by an NHMRC Investigator Grant (GNT2008339). She serves as a consultant on an advisory board for UCB and Limbic Neurology, and has been an invited speaker for Biogen, Excemed and Limbic Neurology. She is on the editorial board of Neurology: Neuroimmunology and Neuroinflammation. She is on the Advisory Board and/or received consulting fees from Sanofi Genzyme, Biogen and Merck relating to treatments for multiple sclerosis. C.C-S and SV has no conflicts of interest or disclosures. SWR has received honoraria for travel support, trial payments, research and clinical support to the neurology department from NHMRC, NBA, MAA, Lambert Initiative, Beeren foundation, anonymous donors; and from pharmaceutical / biological companies: Biogen, CSL, Genzyme, Grifols, Octapharma, Merck, Novartis, Roche, Sanofi. Additional interests and potential conflicts of interest include: Co-founder / shareholder of Medical Safety Systems, National IVIG Governance Advisory Council & Specialist Working Group Australia (Neurology) (paid), Australian Medical Services Advisory Committee ad-hoc sub-committee on IVIG (paid), Australian Technical Advisory Group on Immunisation Varicella Zoster working party (unpaid), Medical advisor (unpaid) to various patient and advocacy groups. SWR is not aware of all devices and medications that operate under FDA rules so cannot affirm that unlabelled uses are discussed. TAH has received honoraria for talks, advisory boards or support for scientific meetings from Bayer-Schering, Novartis, Biogen Idec, Merck, Teva, Merck, Alexion, Bristol Myers Squibb and Sanofi-Genzyme. He is Co-Editor of Advances in Clinical Neuroscience and Rehabilitation and serves on the editorial boards of Journal of Neuroimmunology and Frontiers in Neurology.

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