Clinical, Radiologic, and Prognostic Features of Myelitis Associated with Myelin Oligodendrocyte Glycoprotein Autoantibody

Divyanshu Dubey, Sean J Pittock, Karl N. Krecke, Padraig P. Morris, Elia Sechi, Nicholas L. Zalewski, Brian G Weinshenker, Eslam Shosha, Claudia F Lucchinetti, James P. Fryer, A. Sebastian Lopez-Chiriboga, John Chen, Jiraporn Jitprapaikulsan, Andrew McKeon, Avi Gadoth, B Mark Keegan, Jan-Mendelt Tillema, Elie Naddaf, Marc C. Patterson, Kevin Messacar & 2 others Kenneth L. Tyler, Eoin Flanagan

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment. Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS). Design, Setting, and Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Fifty-four patients met inclusion criteria of (1) clinical myelitis; (2) MOG-IgG positivity; and (3) medical records available. We excluded 145 patients without documented myelitis. Myelitis of AQP4-IgG (n = 46) and MS (n = 26) were used for comparison. Main Outcomes and Measures: Outcome variables included modified Rankin score and need for gait aid. A neuroradiologist analyzed spine magnetic resonance imaging of patients with MOG-IgG and control patients blinded to diagnosis. Results: Of 54 included patients with MOG-IgG myelitis, the median age was 25 years (range, 3-73 years) and 24 were women (44%). Isolated transverse myelitis was the initial manifestation in 29 patients (54%), and 10 (19%) were initially diagnosed as having viral/postviral acute flaccid myelitis. Cerebrospinal fluid-elevated oligoclonal bands occurred in 1 of 38 (3%). At final follow-up (median, 24 months; range, 2-120 months), 32 patients (59%) had developed 1 or more relapses of optic neuritis (n = 31); transverse myelitis (n = 7); or acute disseminated encephalomyelitis (n = 1). Clinical features favoring MOG-IgG myelitis vs AQP4-IgG or MS myelitis included prodromal symptoms and concurrent acute disseminated encephalomyelitis. Magnetic resonance imaging features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H sign) and lack of enhancement. Longitudinally extensive T2 lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (37 of 47 [79%] vs 28 of 34 [82%]; P =.52) but not found in MS. Multiple spinal cord lesions and conus involvement were more frequent with MOG-IgG than AQP4-IgG but not different from MS. Wheelchair dependence at myelitis nadir occurred in one-third of patients with MOG-IgG and AQP4-IgG but never with MS, although patients with MOG-IgG myelitis recovered better than those with AQP4-IgG. Conclusions and Relevance: Myelitis is an early manifestation of MOG-IgG-related disease and may have a clinical phenotype of acute flaccid myelitis. We identified a variety of clinical and magnetic resonance imaging features that may help clinicians identify those at risk in whom MOG-IgG should be tested..

Original languageEnglish (US)
JournalJAMA Neurology
DOIs
StateAccepted/In press - Jan 1 2018

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Myelin-Oligodendrocyte Glycoprotein
Myelitis
Autoantibodies
Immunoglobulin G
Aquaporin 4
Multiple Sclerosis
Transverse Myelitis
Acute Disseminated Encephalomyelitis
Magnetic Resonance Imaging

ASJC Scopus subject areas

  • Clinical Neurology

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Clinical, Radiologic, and Prognostic Features of Myelitis Associated with Myelin Oligodendrocyte Glycoprotein Autoantibody. / Dubey, Divyanshu; Pittock, Sean J; Krecke, Karl N.; Morris, Padraig P.; Sechi, Elia; Zalewski, Nicholas L.; Weinshenker, Brian G; Shosha, Eslam; Lucchinetti, Claudia F; Fryer, James P.; Lopez-Chiriboga, A. Sebastian; Chen, John; Jitprapaikulsan, Jiraporn; McKeon, Andrew; Gadoth, Avi; Keegan, B Mark; Tillema, Jan-Mendelt; Naddaf, Elie; Patterson, Marc C.; Messacar, Kevin; Tyler, Kenneth L.; Flanagan, Eoin.

In: JAMA Neurology, 01.01.2018.

Research output: Contribution to journalArticle

Dubey, Divyanshu ; Pittock, Sean J ; Krecke, Karl N. ; Morris, Padraig P. ; Sechi, Elia ; Zalewski, Nicholas L. ; Weinshenker, Brian G ; Shosha, Eslam ; Lucchinetti, Claudia F ; Fryer, James P. ; Lopez-Chiriboga, A. Sebastian ; Chen, John ; Jitprapaikulsan, Jiraporn ; McKeon, Andrew ; Gadoth, Avi ; Keegan, B Mark ; Tillema, Jan-Mendelt ; Naddaf, Elie ; Patterson, Marc C. ; Messacar, Kevin ; Tyler, Kenneth L. ; Flanagan, Eoin. / Clinical, Radiologic, and Prognostic Features of Myelitis Associated with Myelin Oligodendrocyte Glycoprotein Autoantibody. In: JAMA Neurology. 2018.
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title = "Clinical, Radiologic, and Prognostic Features of Myelitis Associated with Myelin Oligodendrocyte Glycoprotein Autoantibody",
abstract = "Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment. Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS). Design, Setting, and Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Fifty-four patients met inclusion criteria of (1) clinical myelitis; (2) MOG-IgG positivity; and (3) medical records available. We excluded 145 patients without documented myelitis. Myelitis of AQP4-IgG (n = 46) and MS (n = 26) were used for comparison. Main Outcomes and Measures: Outcome variables included modified Rankin score and need for gait aid. A neuroradiologist analyzed spine magnetic resonance imaging of patients with MOG-IgG and control patients blinded to diagnosis. Results: Of 54 included patients with MOG-IgG myelitis, the median age was 25 years (range, 3-73 years) and 24 were women (44{\%}). Isolated transverse myelitis was the initial manifestation in 29 patients (54{\%}), and 10 (19{\%}) were initially diagnosed as having viral/postviral acute flaccid myelitis. Cerebrospinal fluid-elevated oligoclonal bands occurred in 1 of 38 (3{\%}). At final follow-up (median, 24 months; range, 2-120 months), 32 patients (59{\%}) had developed 1 or more relapses of optic neuritis (n = 31); transverse myelitis (n = 7); or acute disseminated encephalomyelitis (n = 1). Clinical features favoring MOG-IgG myelitis vs AQP4-IgG or MS myelitis included prodromal symptoms and concurrent acute disseminated encephalomyelitis. Magnetic resonance imaging features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H sign) and lack of enhancement. Longitudinally extensive T2 lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (37 of 47 [79{\%}] vs 28 of 34 [82{\%}]; P =.52) but not found in MS. Multiple spinal cord lesions and conus involvement were more frequent with MOG-IgG than AQP4-IgG but not different from MS. Wheelchair dependence at myelitis nadir occurred in one-third of patients with MOG-IgG and AQP4-IgG but never with MS, although patients with MOG-IgG myelitis recovered better than those with AQP4-IgG. Conclusions and Relevance: Myelitis is an early manifestation of MOG-IgG-related disease and may have a clinical phenotype of acute flaccid myelitis. We identified a variety of clinical and magnetic resonance imaging features that may help clinicians identify those at risk in whom MOG-IgG should be tested..",
author = "Divyanshu Dubey and Pittock, {Sean J} and Krecke, {Karl N.} and Morris, {Padraig P.} and Elia Sechi and Zalewski, {Nicholas L.} and Weinshenker, {Brian G} and Eslam Shosha and Lucchinetti, {Claudia F} and Fryer, {James P.} and Lopez-Chiriboga, {A. Sebastian} and John Chen and Jiraporn Jitprapaikulsan and Andrew McKeon and Avi Gadoth and Keegan, {B Mark} and Jan-Mendelt Tillema and Elie Naddaf and Patterson, {Marc C.} and Kevin Messacar and Tyler, {Kenneth L.} and Eoin Flanagan",
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T1 - Clinical, Radiologic, and Prognostic Features of Myelitis Associated with Myelin Oligodendrocyte Glycoprotein Autoantibody

AU - Dubey, Divyanshu

AU - Pittock, Sean J

AU - Krecke, Karl N.

AU - Morris, Padraig P.

AU - Sechi, Elia

AU - Zalewski, Nicholas L.

AU - Weinshenker, Brian G

AU - Shosha, Eslam

AU - Lucchinetti, Claudia F

AU - Fryer, James P.

AU - Lopez-Chiriboga, A. Sebastian

AU - Chen, John

AU - Jitprapaikulsan, Jiraporn

AU - McKeon, Andrew

AU - Gadoth, Avi

AU - Keegan, B Mark

AU - Tillema, Jan-Mendelt

AU - Naddaf, Elie

AU - Patterson, Marc C.

AU - Messacar, Kevin

AU - Tyler, Kenneth L.

AU - Flanagan, Eoin

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment. Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS). Design, Setting, and Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Fifty-four patients met inclusion criteria of (1) clinical myelitis; (2) MOG-IgG positivity; and (3) medical records available. We excluded 145 patients without documented myelitis. Myelitis of AQP4-IgG (n = 46) and MS (n = 26) were used for comparison. Main Outcomes and Measures: Outcome variables included modified Rankin score and need for gait aid. A neuroradiologist analyzed spine magnetic resonance imaging of patients with MOG-IgG and control patients blinded to diagnosis. Results: Of 54 included patients with MOG-IgG myelitis, the median age was 25 years (range, 3-73 years) and 24 were women (44%). Isolated transverse myelitis was the initial manifestation in 29 patients (54%), and 10 (19%) were initially diagnosed as having viral/postviral acute flaccid myelitis. Cerebrospinal fluid-elevated oligoclonal bands occurred in 1 of 38 (3%). At final follow-up (median, 24 months; range, 2-120 months), 32 patients (59%) had developed 1 or more relapses of optic neuritis (n = 31); transverse myelitis (n = 7); or acute disseminated encephalomyelitis (n = 1). Clinical features favoring MOG-IgG myelitis vs AQP4-IgG or MS myelitis included prodromal symptoms and concurrent acute disseminated encephalomyelitis. Magnetic resonance imaging features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H sign) and lack of enhancement. Longitudinally extensive T2 lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (37 of 47 [79%] vs 28 of 34 [82%]; P =.52) but not found in MS. Multiple spinal cord lesions and conus involvement were more frequent with MOG-IgG than AQP4-IgG but not different from MS. Wheelchair dependence at myelitis nadir occurred in one-third of patients with MOG-IgG and AQP4-IgG but never with MS, although patients with MOG-IgG myelitis recovered better than those with AQP4-IgG. Conclusions and Relevance: Myelitis is an early manifestation of MOG-IgG-related disease and may have a clinical phenotype of acute flaccid myelitis. We identified a variety of clinical and magnetic resonance imaging features that may help clinicians identify those at risk in whom MOG-IgG should be tested..

AB - Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment. Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS). Design, Setting, and Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Fifty-four patients met inclusion criteria of (1) clinical myelitis; (2) MOG-IgG positivity; and (3) medical records available. We excluded 145 patients without documented myelitis. Myelitis of AQP4-IgG (n = 46) and MS (n = 26) were used for comparison. Main Outcomes and Measures: Outcome variables included modified Rankin score and need for gait aid. A neuroradiologist analyzed spine magnetic resonance imaging of patients with MOG-IgG and control patients blinded to diagnosis. Results: Of 54 included patients with MOG-IgG myelitis, the median age was 25 years (range, 3-73 years) and 24 were women (44%). Isolated transverse myelitis was the initial manifestation in 29 patients (54%), and 10 (19%) were initially diagnosed as having viral/postviral acute flaccid myelitis. Cerebrospinal fluid-elevated oligoclonal bands occurred in 1 of 38 (3%). At final follow-up (median, 24 months; range, 2-120 months), 32 patients (59%) had developed 1 or more relapses of optic neuritis (n = 31); transverse myelitis (n = 7); or acute disseminated encephalomyelitis (n = 1). Clinical features favoring MOG-IgG myelitis vs AQP4-IgG or MS myelitis included prodromal symptoms and concurrent acute disseminated encephalomyelitis. Magnetic resonance imaging features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H sign) and lack of enhancement. Longitudinally extensive T2 lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (37 of 47 [79%] vs 28 of 34 [82%]; P =.52) but not found in MS. Multiple spinal cord lesions and conus involvement were more frequent with MOG-IgG than AQP4-IgG but not different from MS. Wheelchair dependence at myelitis nadir occurred in one-third of patients with MOG-IgG and AQP4-IgG but never with MS, although patients with MOG-IgG myelitis recovered better than those with AQP4-IgG. Conclusions and Relevance: Myelitis is an early manifestation of MOG-IgG-related disease and may have a clinical phenotype of acute flaccid myelitis. We identified a variety of clinical and magnetic resonance imaging features that may help clinicians identify those at risk in whom MOG-IgG should be tested..

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