Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders

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

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Abstract

Objective We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause. Methods We reviewed spinal cord MRIs for ringenhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement. We identified two groups of control patients with: (1) longitudinally extensive myelopathy of other cause (n=66) and (2) myelitis in the context of a concurrent or subsequent diagnosis of multiple sclerosis (MS) from a populationbased cohort (n=30). Results Ring-enhancement was detected in 50 of 156 (32%) myelitis episodes in 41 patients (83% single; 17% multiple attacks). Ring-enhancement was noted on sagittal and axial images in 36 of 43 (84%) ring enhancing myelitis episodes and extended a median of two vertebral segments (range, 1-12); in 21 of 48 (44%) ring enhancing myelitis episodes, the ring extended greater than or equal to three vertebrae. Ringenhancement was accompanied by longitudinally extensive (greater than or equal to three vertebral segments) T2-hyperintensity in 44 of 50 (88%) ring enhancing myelitis episodes. One case of a spinal cord biopsy during ring-enhancing myelitis revealed tissue vacuolation and loss of AQP4 immunoreactivity with preserved axons. The clinical characteristics of ringenhancing myelitis episodes did not differ from non-ringenhancing episodes. Ring-enhancing spinal cord lesions were more common in NMOSD than other causes of longitudinally extensive myelopathy (50/156 (32%) vs 0/66 (0%); p≥0.001) but did not differ between NMOSD and MS (50/156 (32%) vs 6/30 (20%); p=0.20). Conclusions 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.

Original languageEnglish (US)
JournalJournal of Neurology, Neurosurgery and Psychiatry
DOIs
StateAccepted/In press - Dec 2 2016

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Neuromyelitis Optica
Myelitis
Spinal Cord
Aquaporin 4
Spinal Cord Diseases
Multiple Sclerosis
Ring
Lesion
Cord
Immunoglobulin G
Axons
Spine

ASJC Scopus subject areas

  • Surgery
  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Psychiatry and Mental health

Cite this

@article{0111b03b583e4fff97cef6d6147e1082,
title = "Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders",
abstract = "Objective We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause. Methods We reviewed spinal cord MRIs for ringenhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement. We identified two groups of control patients with: (1) longitudinally extensive myelopathy of other cause (n=66) and (2) myelitis in the context of a concurrent or subsequent diagnosis of multiple sclerosis (MS) from a populationbased cohort (n=30). Results Ring-enhancement was detected in 50 of 156 (32{\%}) myelitis episodes in 41 patients (83{\%} single; 17{\%} multiple attacks). Ring-enhancement was noted on sagittal and axial images in 36 of 43 (84{\%}) ring enhancing myelitis episodes and extended a median of two vertebral segments (range, 1-12); in 21 of 48 (44{\%}) ring enhancing myelitis episodes, the ring extended greater than or equal to three vertebrae. Ringenhancement was accompanied by longitudinally extensive (greater than or equal to three vertebral segments) T2-hyperintensity in 44 of 50 (88{\%}) ring enhancing myelitis episodes. One case of a spinal cord biopsy during ring-enhancing myelitis revealed tissue vacuolation and loss of AQP4 immunoreactivity with preserved axons. The clinical characteristics of ringenhancing myelitis episodes did not differ from non-ringenhancing episodes. Ring-enhancing spinal cord lesions were more common in NMOSD than other causes of longitudinally extensive myelopathy (50/156 (32{\%}) vs 0/66 (0{\%}); p≥0.001) but did not differ between NMOSD and MS (50/156 (32{\%}) vs 6/30 (20{\%}); p=0.20). Conclusions 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.",
author = "Zalewski, {Nicholas L.} and Morris, {Padraig P.} and Weinshenker, {Brian G} and Lucchinetti, {Claudia F} and Yong Guo and Pittock, {Sean J} and Krecke, {Karl N.} and Kaufmann, {Timothy J} and Wingerchuk, {Dean Marko} and Neeraj Kumar and Eoin Flanagan",
year = "2016",
month = "12",
day = "2",
doi = "10.1136/jnnp-2016-314738",
language = "English (US)",
journal = "Journal of Neurology, Neurosurgery and Psychiatry",
issn = "0022-3050",
publisher = "BMJ Publishing Group",

}

TY - JOUR

T1 - Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders

AU - Zalewski, Nicholas L.

AU - Morris, Padraig P.

AU - Weinshenker, Brian G

AU - Lucchinetti, Claudia F

AU - Guo, Yong

AU - Pittock, Sean J

AU - Krecke, Karl N.

AU - Kaufmann, Timothy J

AU - Wingerchuk, Dean Marko

AU - Kumar, Neeraj

AU - Flanagan, Eoin

PY - 2016/12/2

Y1 - 2016/12/2

N2 - Objective We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause. Methods We reviewed spinal cord MRIs for ringenhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement. We identified two groups of control patients with: (1) longitudinally extensive myelopathy of other cause (n=66) and (2) myelitis in the context of a concurrent or subsequent diagnosis of multiple sclerosis (MS) from a populationbased cohort (n=30). Results Ring-enhancement was detected in 50 of 156 (32%) myelitis episodes in 41 patients (83% single; 17% multiple attacks). Ring-enhancement was noted on sagittal and axial images in 36 of 43 (84%) ring enhancing myelitis episodes and extended a median of two vertebral segments (range, 1-12); in 21 of 48 (44%) ring enhancing myelitis episodes, the ring extended greater than or equal to three vertebrae. Ringenhancement was accompanied by longitudinally extensive (greater than or equal to three vertebral segments) T2-hyperintensity in 44 of 50 (88%) ring enhancing myelitis episodes. One case of a spinal cord biopsy during ring-enhancing myelitis revealed tissue vacuolation and loss of AQP4 immunoreactivity with preserved axons. The clinical characteristics of ringenhancing myelitis episodes did not differ from non-ringenhancing episodes. Ring-enhancing spinal cord lesions were more common in NMOSD than other causes of longitudinally extensive myelopathy (50/156 (32%) vs 0/66 (0%); p≥0.001) but did not differ between NMOSD and MS (50/156 (32%) vs 6/30 (20%); p=0.20). Conclusions 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.

AB - Objective We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause. Methods We reviewed spinal cord MRIs for ringenhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement. We identified two groups of control patients with: (1) longitudinally extensive myelopathy of other cause (n=66) and (2) myelitis in the context of a concurrent or subsequent diagnosis of multiple sclerosis (MS) from a populationbased cohort (n=30). Results Ring-enhancement was detected in 50 of 156 (32%) myelitis episodes in 41 patients (83% single; 17% multiple attacks). Ring-enhancement was noted on sagittal and axial images in 36 of 43 (84%) ring enhancing myelitis episodes and extended a median of two vertebral segments (range, 1-12); in 21 of 48 (44%) ring enhancing myelitis episodes, the ring extended greater than or equal to three vertebrae. Ringenhancement was accompanied by longitudinally extensive (greater than or equal to three vertebral segments) T2-hyperintensity in 44 of 50 (88%) ring enhancing myelitis episodes. One case of a spinal cord biopsy during ring-enhancing myelitis revealed tissue vacuolation and loss of AQP4 immunoreactivity with preserved axons. The clinical characteristics of ringenhancing myelitis episodes did not differ from non-ringenhancing episodes. Ring-enhancing spinal cord lesions were more common in NMOSD than other causes of longitudinally extensive myelopathy (50/156 (32%) vs 0/66 (0%); p≥0.001) but did not differ between NMOSD and MS (50/156 (32%) vs 6/30 (20%); p=0.20). Conclusions 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.

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JO - Journal of Neurology, Neurosurgery and Psychiatry

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SN - 0022-3050

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