@article{d18efc8a4b014891b75d3c2ac2f820ac,
title = "Progressive motor impairment from “critical” demyelinating lesions of the cervicomedullary junction",
abstract = "Background: Progressive motor impairment anatomically associated with a “critical” lesion has been described in primary demyelinating disease. Most “critical” lesions occur within the spinal cord. Objective: To describe the clinical and radiological features of “critical” lesions of the cervicomedullary junction (CMJ). Methods: Observational study on people presenting with a CMJ lesion associated with primary demyelinating disease-related progressive motor impairment. Clinical data were extracted by chart review. Brain and spinal cord magnetic resonance images were reviewed to characterize the CMJ lesion and determine additional demyelination burden. Results: Forty-one people were included: 29 (71%) had progression from onset and 12 (29%) had a relapse onset (secondary progressive) course. Most had progressive hemiparesis (21 (51%)) or progressive quadriparesis (15 (37%)) with a median Expanded Disability Status Scale (EDSS) of 5.5 (2.0–8.5) at last follow-up. No “critical” CMJ lesion enhanced; most were bilateral (25 (61%)). Brain magnetic resonance images were otherwise normal in 16 (39%) or with a restricted demyelination burden in 15 (37%). Cervical and thoracic cord MRIs were without additional lesions in 25 (61%) and 22/37 (59%), respectively. Conclusion: CMJ “critical” lesions can correlate with progressive motor impairment even with few or no additional magnetic resonance imaging (MRI) lesions. Lesion location is an important determinant of progressive motor impairment in demyelinating disease.",
keywords = "Multiple sclerosis, cervicomedullary junction, demyelinating disease, disability, motor impairment, progressive multiple sclerosis",
author = "Jackson-Tarlton, {Caitlin S.} and Flanagan, {Eoin P.} and Messina, {Steven Anthony} and Benan Barakat and Rowaid Ahmad and Kantarci, {Orhun H.} and Weinshenker, {Brian G.} and Keegan, {B. Mark}",
note = "Funding Information: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: C.S.J.-T. reports no disclosures; E.P.F. has served on advisory boards for Alexion, Genentech, and Horizon Therapeutics; has received speaker honoraria from Pharmacy Times; received royalties from UpToDate; was a site primary investigator in a randomized clinical trial on Inebilizumab in neuromyelitis optica spectrum disorder run by Medimmune/Viela-Bio/Horizon Therapeutics; has received funding from the NIH; is a member of the medical advisory board of the MOG project; and is an editorial board member of the Journal of the Neurological Sciences and Neuroimmunology Reports. S.A.M. reports no disclosures. B.B. reports no disclosures. R.A. reports no disclosures. O.H.K. received speaker honoraria 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. B.G.W. 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, served on an adjudication committee for clinical trials in NMO being conducted by MedImmune and Alexion, and consulted for Chugai and Mitsubishi Tanabe regarding a clinical trial for NMO. BMK is funded by the Applebaum Family and is a member of the Center for Multiple Sclerosis and Autoimmune Research Mayo Clinic, receives publishing royalties for Common Pitfalls in Multiple Sclerosis and CNS Demyelinating Diseases, and is an Editorial Board member of Multiple Sclerosis and Related Disorders. Publisher Copyright: {\textcopyright} The Author(s), 2022.",
year = "2022",
doi = "10.1177/13524585221114438",
language = "English (US)",
journal = "Multiple Sclerosis",
issn = "1352-4585",
publisher = "SAGE Publications Ltd",
}