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What are the clinical and neuroimaging findings of patients with a spontaneous spinal cord infarction (SCI), and can diagnostic criteria be established to facilitate an accurate diagnosis?
Of 133 patients with a spontaneous SCI, 102 (77%) reached nadir within 12 hours, while others had a stuttering decline. Magnetic resonance imaging shows confirmatory (eg, vertebral body infarct) and supportive findings while excluding other etiologies; cerebrospinal fluid was usually noninflammatory (92%).
The validation of spinal cord infarction diagnostic criteria supports their utility.
Spinal cord infarction (SCI) is often disabling, and the diagnosis can be challenging without an inciting event (eg, aortic surgery). Patients with a spontaneous SCI are often misdiagnosed as having transverse myelitis. Diagnostic criteria for SCI are lacking, hindering clinical care and research.
To describe the characteristics of spontaneous SCI and propose diagnostic criteria.
Design, Setting, and Participants
An institution-based search tool was used to identify patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1997 to December 2017 with a spontaneous SCI. Patients provided written consent to use their records for research. Participants were 18 years and older with a diagnosis of spontaneous SCI (n = 133), and controls were selected from a database of alternative myelopathy etiologies for validation of the proposed diagnostic criteria (n = 280).
Main Outcomes and Measures
A descriptive analysis of SCI was performed and used to propose diagnostic criteria, and the criteria were validated.
Of 133 included patients with a spontaneous SCI, the median (interquartile range) age at presentation was 60 (52-69) years, and 101 (76%) had vascular risk factors. Rapid onset of severe deficits reaching nadir within 12 hours was typical (102 [77%]); some had a stuttering decline (31 [23%]). Sensory loss occurred in 126 patients (95%), selectively affecting pain/temperature in 49 (39%). Initial magnetic resonance imaging (MRI) spine results were normal in 30 patients (24%). Characteristic MRI T2-hyperintense patterns included owl eyes (82 [65%]) and pencil-like hyperintensity (50 [40%]); gadolinium enhancement (37 of 96 [39%]) was often linear and located in the anterior gray matter. Confirmatory MRI findings included diffusion-weighted imaging/apparent diffusion coefficient restriction (19 of 29 [67%]), adjacent dissection/occlusion (16 of 82 [20%]), and vertebral body infarction (11 [9%]). Cerebrospinal fluid showed mild inflammation in 7 of 89 patients (8%). Diagnostic criteria was proposed for definite, probable, and possible SCI of periprocedural and spontaneous onset. In the validation cohort (n = 280), 9 patients (3%) met criteria for possible SCI, and none met criteria for probable SCI.
Conclusions and Relevance
This large series of spontaneous SCIs provides clinical, laboratory, and MRI clues to SCI diagnosis. The diagnostic criteria proposed here will aid clinicians in making the correct diagnosis and ideally improve future care for patients with SCI. The validation of these criteria supports their utility in the evaluation of acute myelopathy.
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Corresponding Author: Nicholas L. Zalewski, MD, Mayo Clinic, 200 1st St SW, Rochester, MN 55905 (email@example.com).
Accepted for Publication: July 14, 2018.
Published Online: September 24, 2018. doi:10.1001/jamaneurol.2018.2734
Author Contributions: Drs Zalewski and Flanagan had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Concept and design: Zalewski, Rabinstein, Kaufmann, Blessing, Flanagan.
Acquisition, analysis, or interpretation of data: Zalewski, Krecke, Brown, Wijdicks, Weinshenker, Kaufmann, Morris, Aksamit, Bartleson, Lanzino, Flanagan.
Drafting of the manuscript: Zalewski, Morris.
Critical revision of the manuscript for important intellectual content: All authors.
Statistical analysis: Zalewski.
Administrative, technical, or material support: Kaufmann, Morris, Bartleson.
Supervision: Rabinstein, Wijdicks, Morris, Aksamit, Lanzino, Flanagan.
Conflict of Interest Disclosures: Dr Weinshenker receives royalties from RSR Ltd, University of Oxford, Hospices Civils de Lyon, and MVZ Labor PD Dr Volkmann und Kollegen GbR for a patent of neuromyelitis optica–IgG as a diagnostic test for neuromyelitis optica and related disorders; serves as a member of an adjudication committee for clinical trials in neuromyelitis optica being conducted by MedImmune and Alexion Pharmaceuticals; is a consultant for Caladrius Biosciences and BrainStorm Cell Therapeutics regarding potential clinical trials for neuromyelitis optica; and serves as a member of a data safety monitoring committee for clinical trials conducted by Novartis. Dr Lanzino is a consultant for Medtronic.
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