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Background and Objectives: Identifying the etiologic diagnosis in patients presenting with myelopathy is essential in order to guide appropriate treatment and follow-up. We set out to examine the etiologic diagnosis after comprehensive clinical evaluation and diagnostic work-up in a large cohort of patients referred to our specialized myelopathy clinic, and to explore the demographic profiles and symptomatic evolution of specific etiologic diagnoses., Methods: In this retrospective study of patients referred to the Johns Hopkins Myelitis and Myelopathy Center between 2006 and 2021 for evaluation of "transverse myelitis", the final etiologic diagnosis determined after comprehensive evaluation in each patient was reviewed and validated. Demographic characteristics and temporal profile of symptom evolution were recorded., Results: Of 1193 included patients, 772 (65%) were determined to have an inflammatory myelopathy and 421 (35%) were determined to have a non-inflammatory myelopathy. Multiple sclerosis/clinically isolated syndrome (n = 221, 29%) and idiopathic myelitis (n = 149, 19%) were the most frequent inflammatory diagnoses, while spinal cord infarction (n = 197, 47%) and structural causes of myelopathy (n = 108, 26%) were the most frequent non-inflammatory diagnoses. Compared to patients with inflammatory myelopathies, patients with non-inflammatory myelopathies were more likely to be older, male and experience chronic symptom evolution (p < 0.001 for all). Hyperacute symptom evolution was most frequent in patients with spinal cord infarction (74%), while chronic symptom evolution was most frequent in patients with structural causes of myelopathy (81%), arteriovenous fistula or arteriovenous malformation (81%), myelopathy associated with rheumatologic disorder (71%), and sarcoidosis-associated myelopathy (61%)., Conclusions: Patients initially diagnosed with "transverse myelitis" are eventually found to have a more specific inflammatory or even non-inflammatory cause, potentially resulting in inappropriate treatment and follow-up. Demographic characteristics and temporal profile of symptom evolution may help inform a differential diagnosis in these patients. Etiological diagnosis of myelopathies would provide better therapeutic decisions., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Purpose: Low-flow spinal arteriovenous fistulas (SAVFs) with intradural venous drainage typically manifest with a progressive venous hypertensive myelopathy (VHM) in older patients. VHM is difficult to identify. MRI is often nonspecific, and many cases are initially misdiagnosed, most often as transverse myelitis. The workup of myelopathic patients frequently includes thoracic and/or abdominal contrast-enhanced CT (CECT) that are generally not reviewed by neuroradiologists. The purpose of this work was to investigate how often abnormal enhancing intracanalar structures corresponding to the draining veins of a low-flow SAVF were documented by CECT., Materials and Methods: We evaluated 92 consecutive patients with low-flow SAVFs and VHM treated at our institution between 2009 and 2018. The study group included 22 of these patients with at least one thoracoabdominal CECT available for review. The control group consisted of 20 consecutive myelopathy patients with negative angiography and at least one thoracoabdominal CECT. Intracanalar enhancing structures were classified either as (i) conspicuous or (ii) equivocal or absent., Results: One CECT in the study group was technically inadequate. Conspicuous intracanalar enhancing structures were observed in 20 of the remaining 21 patients with SAVFs (95.2%) and in 2 of 20 control patients (10%). None of the enhancing intracanalar structures was mentioned in official study reports., Conclusions: The presence of enhancing vascular structures within the spinal canal on thoracoabdominal CECT obtained during the workup of myelopathies appears to represent a powerful but currently underappreciated tool for the detection of low-flow SAVFs.

Objective: To determine the characteristic clinical and spinal MRI phenotypes of sarcoidosis-associated myelopathy (SAM), we analyzed a large cohort of patients with this disorder., Methods: Patients diagnosed with SAM at a single center between 2000 and 2018 who met the established criteria for definite and probable neurosarcoidosis were included in a retrospective analysis to identify clinical profiles, CSF characteristics, and MRI lesion morphology., Results: Of 62 included patients, 33 (53%) were male, and 30 (48%) were African American. SAM was the first clinical presentation of sarcoidosis in 49 patients (79%). Temporal profile of symptom evolution was chronic in 81%, with sensory symptoms most frequently reported (87%). CSF studies showed pleocytosis in 79% and CSF-restricted oligoclonal bands in 23% of samples tested. Four discrete patterns of lesion morphology were identified on spine MRI: longitudinally extensive myelitis (n = 28, 45%), short tumefactive myelitis (n = 14, 23%), spinal meningitis/meningoradiculitis (n = 14, 23%), and anterior myelitis associated with areas of disc degeneration (n = 6, 10%). Postgadolinium enhancement was seen in all but 1 patient during the acute phase. The most frequent enhancement pattern was dorsal subpial enhancement (n = 40), followed by meningeal/radicular enhancement (n = 23) and ventral subpial enhancement (n = 12). In 26 cases (42%), enhancement occurred at locations with coexisting structural changes (e.g., spondylosis)., Conclusions: Recognition of the clinical features (chronically evolving myelopathy) and distinct MRI phenotypes (with enhancement in a subpial and/or meningeal pattern) seen in SAM can aid diagnosis of this disorder. Enhancement patterns suggest that SAM may have a predilection for areas of the spinal cord susceptible to mechanical stress., (Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

A 42-year-old man, returning to Europe after a 2-month stay in China, reported cough and runny nose. Five days later, he developed neck discomfort and rapidly evolving weakness, spreading from his arms to the facial, bulbar and neck muscles, and then the legs. He developed dysphagia and breathlessness, and was intubated in the emergency department. Cerebrospinal fluid showed mildly elevated protein. On day 2 he had fixed dilated pupils, with absent oculocephalic reflexes, and a flaccid upper limb paralysis. MR scans of the brain and spinal cord were normal. The early features of descending weakness, bulbar involvement and fixed dilated pupils made it difficult to distinguish between Guillain-Barré syndrome and botulism, and early investigations were inconclusive. We initially gave both botulinum antitoxin and intravenous immunoglobulin, but initially delayed plasma exchange as this would have removed the botulinum antitoxin. Diagnostic testing for botulism has low sensitivity, so we urge preemptive treatment if it is clinically suspected. [ABSTRACT FROM AUTHOR]