Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses

Zalewski, Nicholas L.; Flanagan, Eoin P.; Keegan, B. Mark

doi:10.1212/wnl.0000000000004796

Cited by 84 publications

(66 citation statements)

References 23 publications

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“…Due to its rarity, there are few reliable estimates of its incidence; however, previous studies showed that SCI accounted for 1.2% of all strokes . Recent studies have shown that myelopathy related to ischemic diseases accounts for 14–18% of patients with transverse myelitis, suggesting the underdiagnosis of SCI . Clinically, three main characteristic symptoms could differentiate SCI from other etiologies of nontraumatic myelopathy (e.g., a hyperacute temporal course, new onset of back pain, and flaccid weakness) .…”

Section: Introductionmentioning

confidence: 99%

The etiologies and prognosis associated with spinal cord infarction

Hsu

Cheng

Liao

et al. 2019

Ann Clin Transl Neurol

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Background This study aims to investigate the etiology and prognosis of spinal cord infarction (SCI). Methods Over a period of 16 years, we retrospectively analyzed 31 patients with SCI. Demographic features and symptom presentations were carefully documented. Etiology‐specific MRI features, such as the length and distribution of the lesions and owl’s eyes sign, were recorded and analyzed to determine their associations with the clinical signs/symptoms. Results In total, seven patients had aortic or vertebral artery dissections. We divided the patients with SCI into two groups: those with or without vessel dissection. Among SCI patients, the onset age was younger, and the proportion of patients with long‐segment lesions and posterior pattern involvement on axial view was higher in the group with dissection than in the group without dissection (all P < 0.05). The lesions were frequently located in the upper cervical or lower thoracic‐lumbar regions, and the lengths of the lesions were associated with 1‐month outcomes, suggesting that artery dissection may contribute to the longitudinal and posterior extension of SCI. In contrast, among patients without dissection, the range of longitudinal extensions of in spans of vertebral bodies was broader (range, 1–8). A higher proportion of patients had focal pain adjacent to the lesion (P = 0.05) and a poorer 1‐month outcome (P = 0.04) in the long‐segment lesion group than in the short‐segment lesion group. Conclusions A detailed history and the use of modern imaging tools may help clinicians search for vessel dissection and other etiologies, evaluate the spatial extension of lesions in SCI, and predict prognosis.

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Section: Introductionmentioning

confidence: 99%

The etiologies and prognosis associated with spinal cord infarction

Hsu

Cheng

Liao

et al. 2019

Ann Clin Transl Neurol

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“…The risk of recurrence trended to be higher in the group of patients with delayed immunosuppressive treatment (32%) than in the group of patients with early immunosuppressive treatment (9%). Before starting a longer term immunosuppression, neurologists must be very stringent, trying to exclude difficult differential diagnosis [9] such as neurosarcoidosis, spinal cord infarction [10], or dural fistula. Neurologists need to be aware of evocative MRI patterns of alternative diseases: leptomeningeal enhancement or trident pattern of enhancement in neurosarcoidosis, compressive aspect or mass lesion in a spinal tumor, patchy anterior horn T2 hyperintensities in a spinal cord infarction, and serpiginous vessels in a spinal dural arteriovenous fistula, as proposed in series of idiopathic transverse myelitis [9].…”

Section: Discussionmentioning

confidence: 99%

“…Before starting a longer term immunosuppression, neurologists must be very stringent, trying to exclude difficult differential diagnosis [9] such as neurosarcoidosis, spinal cord infarction [10], or dural fistula. Neurologists need to be aware of evocative MRI patterns of alternative diseases: leptomeningeal enhancement or trident pattern of enhancement in neurosarcoidosis, compressive aspect or mass lesion in a spinal tumor, patchy anterior horn T2 hyperintensities in a spinal cord infarction, and serpiginous vessels in a spinal dural arteriovenous fistula, as proposed in series of idiopathic transverse myelitis [9]. In this study, to avoid misdiagnosis, inclusion criteria were stringent, and MRI was performed following the OFSEP protocol, centrally analyzed by neuroradiological experts in the field, which is a guarantee of quality.…”

Section: Discussionmentioning

confidence: 99%

“…Among the 29 patients receiving maintenance therapies, five patients had several successive maintenance treatments (3 patients with 2 different treatments and 2 patients with 3 different treatments). The main used drugs were: mycophenolate mofetil (16) and rituximab (9). Other immunosuppressants included: azathioprine (5), cyclophosphamide (3), mitoxantrone (1), methotrexate (1), and infliximab (1).…”

Section: Evaluation Of Maintenance Therapiesmentioning

confidence: 99%

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Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

Maillart

Durand‐Dubief²,

Louapre

et al. 2020

J Neuroinflammation

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Background: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. Methods: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. Results: Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). Conclusions:A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.

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“…It is important to not label a myelopathy as "transverse myelitis" or "idiopathic transverse myelitis" (ITM) unless a patient clearly meets the defined diagnostic criteria for ITM with comprehensive exclusion of other identifiable causes of myelopathy since premature diagnostic conclusions may potentially be harmful to patient outcome. 1,2…”

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confidence: 99%

Autoimmune and Paraneoplastic Myelopathies

Zalewski

Flanagan

2018

Semin Neurol

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Prompt recognition of an inflammatory myelopathy is critical, as a specific diagnosis and management plan allows for optimal patient outcomes. Many treatment options are now available for autoimmune and paraneoplastic myelopathies, but specific management strategies and expected prognosis vary widely depending on the underlying etiology. An understanding of the relevant clinical details, imaging findings, and other diagnostic information that can help achieve a specific myelopathy diagnosis and treatment plan is essential for all neurologists, given the variety of contexts in which myelopathies are encountered. We provide an outline of the diagnostic evaluation and treatment of various inflammatory myelopathies seen in autoimmune and paraneoplastic diseases, including multiple sclerosis, aquaporin-4 immunoglobulin G (IgG) seropositive neuromyelitis optica spectrum disorder, sarcoidosis, myelin oligodendrocyte glycoprotein IgG associated disease, and other rare inflammatory myelopathies; we also highlight common mimickers of inflammatory myelopathies.

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Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses

Abstract: The majority of patients with suspected ITM have an alternative specific myelopathy diagnosis. A presumptive diagnosis of ITM can lead to premature diagnostic conclusions affecting patient treatment.

Cited by 84 publications

References 23 publications

The etiologies and prognosis associated with spinal cord infarction

The etiologies and prognosis associated with spinal cord infarction

Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

Autoimmune and Paraneoplastic Myelopathies

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