Defining the natural history of tumefactive demyelination: A retrospective cohort of 257 patients

• Published in: Annals of clinical and translational neurology 2023-09, Vol.10 (9), p.1544-1555
• Main Authors: Fereidan-Esfahani, Mahboubeh, Decker, Paul A, Weigand, Stephen D, Lopez Chiriboga, Alfonso S, Flanagan, Eoin P, Tillema, Jan-Mendelt, Lucchinetti, Claudia F, Eckel-Passow, Jeanette E, Tobin, W Oliver
• Format: Article
• Language: English
• Subjects: Antibodies; Aquaporins; Biopsy; Glycoproteins; Immunoglobulins; Magnetic resonance imaging; Multiple sclerosis; Patients
• ISSN: 2328-9503; 2328-9503
• DOI: 10.1002/acn3.51844

To describe demographic, clinical, and radiographic features of tumefactive demyelination (TD) and identify factors associated with severe attacks and poor outcomes. Retrospective review of TD cases seen at Mayo Clinic, 1990-2021. Of 257 patients with TD, 183/257 (71%) fulfilled the 2017 multiple sclerosis (MS) McDonald criteria at the last follow-up, 12/257 (5%) had myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), 0 had aquaporin-4-IgG seropositive neuromyelitis optic spectrum disorders (AQP4+ NMOSD), and 62/257 (24%) were cryptogenic. Onset before age 18 was present in 18/257 (7%). Female to male ratio was 1.3:1. Cerebrospinal fluid oligoclonal (CSF) bands were present in 95/153 (62%). TD was the first demyelinating attack in 176/257 (69%). At presentation, 59/126 (47%) fulfilled Barkhof criteria for dissemination in space, 59/100 (59%) had apparent diffusion coefficient (ADC) restriction, and 57/126 (45%) had mass effect. Despite aggressive clinical presentation at onset, 181/257 (70%) of patients remained fully ambulatory (Expanded Disability Status Scale [EDSS] ≤4) after a 3.0-year median follow-up duration. Severe initial attack-related disability (EDSS ≥4) was more common in patients with motor symptoms (81/143 vs. 35/106, p