Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders

Zalewski, Nicholas L.; Morris, Pearse; Weinshenker, Brian G.; Lucchinetti, Claudia F.; Guo, Yuxin; Pittock, Sean J.; Krecke, Karl N.; Kaufmann, Timothy J.; Wingerchuk, Dean M.; Kumar, Neeraj; Flanagan, Eoin P.

doi:10.1136/jnnp-2016-314738

Cited by 58 publications

(42 citation statements)

References 39 publications

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“…24,25 However, we did not observe any NMOSD-like bright spotty T2W lesions in the spinal cord in BD, 24,25 nor is the Bagel Sign observed in NMOSD. 24,25 However, we did not observe any NMOSD-like bright spotty T2W lesions in the spinal cord in BD, 24,25 nor is the Bagel Sign observed in NMOSD.…”

Section: Discussioncontrasting

confidence: 55%

“…Long myelopathy with a ring and/or patchy enhancement pattern without oligoclonal bands in CSF, as observed in patients with BD, 7-10 is similar to neuromyelitis optica spectrum disorders (NMOSD). 24,25 However, we did not observe any NMOSD-like bright spotty T2W lesions in the spinal cord in BD, 24,25 nor is the Bagel Sign observed in NMOSD. Although not all our patients underwent NMO-IgG testing, those that did were negative.…”

Section: Discussioncontrasting

confidence: 55%

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Myelopathy in Behçet's disease: The Bagel Sign

Uygunoğlu

Zeydan

Özgüler

et al. 2017

Annals of Neurology

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Section: Discussioncontrasting

confidence: 55%

Section: Discussioncontrasting

confidence: 55%

Myelopathy in Behçet's disease: The Bagel Sign

Uygunoğlu

Zeydan

Özgüler

et al. 2017

Annals of Neurology

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“…Enhancement patterns are usually homogeneous throughout the lesion (75%) or ring-enhancing (6-25%). [5][6][7] Gadolinium enhancement resolves within 8 weeks in approximately 95% of cases. 5 The cerebrospinal fluid (CSF) profile generally shows evidence of mild inflammation (0-50 cells, mild elevation in protein) and elevated oligoclonal bands and/or immunoglobulin G (IgG) index, although a normal CSF is seen in approximately 15% of cases historically.…”

Section: Multiple Sclerosismentioning

confidence: 99%

“…Myelitis lesions extending < 3 vertebral segments on MRI can be seen in approximately 15% of NMOSD cases, 20,21 but even these shorter lesions in NMOSD are generally still more extensive than a typical MS spinal lesion. Accompanying T1-weighted gadolinium enhancement helps distinguish a new lesion from an old lesion and occurs in the vast majority of cases, 7 and can also help differentiate typical AQP4-IgG seropositive NMOSD enhancement patterns (ring enhancement [►Fig. 1, C3 and C4] or patchy) 7,22 from other myelopathies with different contrast enhancement patterns.…”

Section: Aquaporin-4-igg Seropositive Neuromyelitis Optica Spectrum Dmentioning

confidence: 99%

“…Accompanying T1-weighted gadolinium enhancement helps distinguish a new lesion from an old lesion and occurs in the vast majority of cases, 7 and can also help differentiate typical AQP4-IgG seropositive NMOSD enhancement patterns (ring enhancement [►Fig. 1, C3 and C4] or patchy) 7,22 from other myelopathies with different contrast enhancement patterns. MRI may also give additional clues to AQP4-IgG seropositive NMOSD myelitis with central T2 hyperintensity on axial views (►Fig.…”

Section: Aquaporin-4-igg Seropositive Neuromyelitis Optica Spectrum Dmentioning

confidence: 99%

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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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Unique Gadolinium Enhancement Pattern in Spinal Dural Arteriovenous Fistulas

et al. 2018

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IMPORTANCE Spinal dural arteriovenous fistula (sDAVF) is often misdiagnosed as an inflammatory or a neoplastic myelopathy, often because of intraparenchymal gadolinium enhancement on magnetic resonance imaging (MRI); proper early diagnosis is important because deficits are reversible and a delay in treatment is associated with permanent morbidity. Tortuous flow voids on MRI are not universally present; thus, recognition of a unique gadolinium enhancement pattern may also aid in the early recognition and treatment of sDAVF.OBJECTIVE To describe a unique pattern of spinal cord gadolinium enhancement on MRI in sDAVF. DESIGN, SETTING, AND PARTICIPANTS This retrospective evaluation included pretreatmentMRIs from 80 patients referred to the Mayo Clinic, Rochester, Minnesota, from January 1, 1997, through December 31, 2017, with a confirmed diagnosis of sDAVF and a control group of 144 patients with alternative confirmed myelopathy diagnoses. All participants underwent a neurologic evaluation at the Mayo Clinic.MAIN OUTCOMES AND MEASURES Evidence of at least 1 focal geographic nonenhancing area within a long segment of intense holocord gadolinium enhancement (termed the missing-piece sign) on MRI. RESULTSOf 51 patients with an sDAVF and a pretreatment MRI with gadolinium enhancement, 44 (86%) had intraparenchymal contrast enhancement, and 19 of these patients (43%) displayed the characteristic missing-piece sign. Of these 19 patients, symptom onset occurred at a median age of 67 years (range, 27-80 years); 15 patients were men. Progressive myelopathy features affecting the lower extremities occurred during a median of 33 months (range, 1-84 months). Eleven patients (58%) received an alternative diagnosis before confirmation of sDAVF. Tortuous flow voids were present on T2-weighted MRI in 13 of 19 patients. More than 1 digital subtraction angiogram was required for 5 patients to confirm the diagnosis. The missing-piece sign was not seen in any patients from the control group.CONCLUSIONS AND RELEVANCE This unique gadolinium enhancement pattern in sDAVF was not found in a large control group of patients with other myelopathy. Identifying the missing-piece sign on MRI could potentially result in earlier time to angiography with improved outcomes for patients with an sDAVF.

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Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders

Abstract: Spinal cord ring-enhancement accompanies one-third of NMOSD myelitis episodes and distinguishes NMOSD from other causes of longitudinally extensive myelopathies but not from MS.

Cited by 58 publications

References 39 publications

Myelopathy in Behçet's disease: The Bagel Sign

Myelopathy in Behçet's disease: The Bagel Sign

Autoimmune and Paraneoplastic Myelopathies

Unique Gadolinium Enhancement Pattern in Spinal Dural Arteriovenous Fistulas

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