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Abstract Details

Clinical-pathological correlation of progressive solitary sclerosis: a case report
Multiple Sclerosis
P9 - Poster Session 9 (5:30 PM-6:30 PM)
12-003
Progressive solitary sclerosis is a rare unifocal demyelinating CNS disorder with an insidious course of cumulative disability. This atypical disorder is thought to be a variant of multiple sclerosis.
To review the clinical course and post-mortem pathology in an individual case of progressive solitary sclerosis.
Case review.
A 42 year old transgender man presented in 2011 with right hemibody paresthesias and normal spinal cord imaging. He returned in 2012 with gait imbalance, and MRI brain demonstrated an isolated non-enhancing T2/FLAIR hyperintense lesion at the cervico-medullary junction. He developed two distinct relapses involving the corticospinal tract in 2013, one of which was steroid-responsive. Despite trials of dimethyl fumarate, and then rituximab infusions, he progressed to requiring a walker in 2013. He required manual wheelchair in 2015, and then progressed to tetraparesis necessitating power wheelchair by 2018. His workup – including CSF oligoclonal band, serum anti-aquaporin-4 antibodies, and PET imaging of body and brain – was negative. Multiple MRIs from April 2012 to August 2019 did not demonstrate interval change of his solitary lesion, nor accumulation of further lesions in the central neuroaxis. He underwent medical assistance in dying in July 2021. Post-mortem neuropathology revealed a demarcated area of myelin loss with largely intact axons with rare microglia primarily at the edge of the lesion, similar to inactive multiple sclerosis plaques.
To our knowledge, this is the first reported case of progressive solitary sclerosis fitting the proposed clinical phenotype in addition to pathological confirmation of MS-like lesions, but within a single lesion of the brain and spinal cord.
Authors/Disclosures
Nathan Chu, MD
PRESENTER
Dr. Chu has nothing to disclose.
Sumit Das, MD (University of Alberta) Dr. Das has nothing to disclose.
Penelope Sybol Smyth, MD (University of Alberta) Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis Pharmaceuticals. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi Genzyme. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche Canada. Dr. Smyth has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Short Term Exceptional Drug Therapy Program Alberta. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen-Idec. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion Pharmaceuticals. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi Genzyme. Dr. Smyth has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis Pharmaceuticals.