Contributions
Abstract: P460
Type: Poster Sessions
Abstract Category: Pathology and pathogenesis of MS - Neurodegeneration
Introduction: Progressive motor impairment anatomically attributable to a single demyelinating lesion is seen in patients with “progressive solitary sclerosis”. These “critical lesions” are generally prominent lesions located in the spinal cord along corticospinal tracts and associated with focal atrophy. Rare multiple sclerosis (MS) patients with exclusively unilateral motor progression may offer insight into the role of such critical lesions in the development of progression.
Objective: To determine if progressive exclusively unilateral motor impairment in MS can be attributable to a single critical lesion.
Methods: We included Mayo Clinic patients (1996-2017) who had: (1) ≥1 year of unilateral motor progression; (2) no contralateral pyramidal symptoms/signs; (3) >5 central nervous system demyelinating lesions; (4) fulfilled 2017 McDonald MS criteria. A blinded neuroradiologist evaluated MRI's and determined whether a single potential critical lesion could be identified based on MRI characteristics .
Results: Thirty-eight patients were included: primary progressive MS, 20 (53%); secondary progressive MS, 18 (47%). Median age at progression onset was 55 years (range, 39-73). Median EDSS was 5 (range, 2.5-7.5) at last follow-up (median, 119.5 months from symptom onset; range, 22-418). Progressive unilateral motor impairment was characterized by (face-sparing) hemiparesis or monoparesis. The likelihood of detecting at least one demyelinating lesion along the corticospinal tract to which the deficit could be attributed was higher (100%) than on the contralateral side (39%) (p< 0.0001). The neuroradiologist identified a single potential critical lesion in 26 cases (68%): cervical cord, 20; thoracic cord, 6. In all 26 the progressive motor deficits were attributable to the identified critical lesion. Of the remaining 12 patients, eight had two potential critical lesions along the same corticospinal tract to which the deficit localized.
Conclusion: In MS patients with unilateral motor progression, a single, recognizable critical lesion along corticospinal tracts may be responsible.
Disclosure: Dr. Keegan has research funded by Biogen, and receives publishing royalties for Common Pitfalls in Multiple Sclerosis and CNS Demyelinating Diseases. He is an Editorial Board member of Multiple Sclerosis and Related Disorders. Dr. Weinshenker receives royalties from RSR Ltd, Oxford University, Hospices Civil de Lyon, and MVZ Labor PD Dr. Volkmann und Kollegen GbR for a patent of NMO-IgG as a diagnostic test for NMO and related disorders. He serves as a member of an adjudication committee for clinical trials in NMO being conducted by VielaBio and Alexion pharmaceutical companies. He is a consultant for Caladrius Biosciences, Brainstorm Therapeutics, Roivant Sciences and Chugai Pharma regarding potential clinical trials for NMO. He serves as a member of a data safety monitoring committee for clinical trials conducted by Novartis. O.H. Kantarci received speaker honoraria (paid to Mayo Clinic) from Novartis and Biogen; performed a grant review for The National Multiple Sclerosis Society; and received research support from Biogen, the Multiple Sclerosis Society, the Mayo Foundation, and the Hilton Foundation. Drs. Sechi, Kaufmann and Flanagan have nothing to disclose.
Abstract: P460
Type: Poster Sessions
Abstract Category: Pathology and pathogenesis of MS - Neurodegeneration
Introduction: Progressive motor impairment anatomically attributable to a single demyelinating lesion is seen in patients with “progressive solitary sclerosis”. These “critical lesions” are generally prominent lesions located in the spinal cord along corticospinal tracts and associated with focal atrophy. Rare multiple sclerosis (MS) patients with exclusively unilateral motor progression may offer insight into the role of such critical lesions in the development of progression.
Objective: To determine if progressive exclusively unilateral motor impairment in MS can be attributable to a single critical lesion.
Methods: We included Mayo Clinic patients (1996-2017) who had: (1) ≥1 year of unilateral motor progression; (2) no contralateral pyramidal symptoms/signs; (3) >5 central nervous system demyelinating lesions; (4) fulfilled 2017 McDonald MS criteria. A blinded neuroradiologist evaluated MRI's and determined whether a single potential critical lesion could be identified based on MRI characteristics .
Results: Thirty-eight patients were included: primary progressive MS, 20 (53%); secondary progressive MS, 18 (47%). Median age at progression onset was 55 years (range, 39-73). Median EDSS was 5 (range, 2.5-7.5) at last follow-up (median, 119.5 months from symptom onset; range, 22-418). Progressive unilateral motor impairment was characterized by (face-sparing) hemiparesis or monoparesis. The likelihood of detecting at least one demyelinating lesion along the corticospinal tract to which the deficit could be attributed was higher (100%) than on the contralateral side (39%) (p< 0.0001). The neuroradiologist identified a single potential critical lesion in 26 cases (68%): cervical cord, 20; thoracic cord, 6. In all 26 the progressive motor deficits were attributable to the identified critical lesion. Of the remaining 12 patients, eight had two potential critical lesions along the same corticospinal tract to which the deficit localized.
Conclusion: In MS patients with unilateral motor progression, a single, recognizable critical lesion along corticospinal tracts may be responsible.
Disclosure: Dr. Keegan has research funded by Biogen, and receives publishing royalties for Common Pitfalls in Multiple Sclerosis and CNS Demyelinating Diseases. He is an Editorial Board member of Multiple Sclerosis and Related Disorders. Dr. Weinshenker receives royalties from RSR Ltd, Oxford University, Hospices Civil de Lyon, and MVZ Labor PD Dr. Volkmann und Kollegen GbR for a patent of NMO-IgG as a diagnostic test for NMO and related disorders. He serves as a member of an adjudication committee for clinical trials in NMO being conducted by VielaBio and Alexion pharmaceutical companies. He is a consultant for Caladrius Biosciences, Brainstorm Therapeutics, Roivant Sciences and Chugai Pharma regarding potential clinical trials for NMO. He serves as a member of a data safety monitoring committee for clinical trials conducted by Novartis. O.H. Kantarci received speaker honoraria (paid to Mayo Clinic) from Novartis and Biogen; performed a grant review for The National Multiple Sclerosis Society; and received research support from Biogen, the Multiple Sclerosis Society, the Mayo Foundation, and the Hilton Foundation. Drs. Sechi, Kaufmann and Flanagan have nothing to disclose.