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 M. Wingerchuk, Neeraj Kumar, Eoin P. Flanagan

Research output: Contribution to journalArticlepeer-review

22 Scopus citations

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)
Pages (from-to)218-225
Number of pages8
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume88
Issue number3
DOIs
StatePublished - Mar 1 2017

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health

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