ECTRIMS eLearning

Significance of aquaporin-4-immunoglobulin G in longitudinally extensive transverse myelitis
Author(s): ,
D Zelic
Affiliations:
Department of Neurology, Vejle Hospital, Vejle
,
H.P.B Skejoe
Affiliations:
Department of Radiology, Aleris-Hamlet Hospital, Copenhagen
,
S.T Lillevang
Affiliations:
Department of Clinical Immunology, Odense University Hospital
N Asgari
Affiliations:
Department of Neurology, Vejle Hospital, Vejle;Institutes of Molecular Medicine and Regional Research, University of Southern Denmark, Odense, Denmark
ECTRIMS Learn. Zelic D. 09/16/16; 145705; P1021
Dejan Zelic
Dejan Zelic
Contributions
Abstract

Abstract: P1021

Type: Poster

Abstract Category: Pathology and pathogenesis of MS - Imaging

Background: Imaging criteria have been incorporated into the proposed diagnostic criteria for neuromyelitis optica spectrum disorder (NMOSD) and the diagnosis may be made solely based on clinical and magnetic resonance imaging (MRI)-based analysis. A common manifestation of NMOSD is the longitudinal extensive transverse myelitis (LETM). The clinical impact of aquaporin-4-immunoglobulin G (AQP4-IgG) positivity in the NMOSD patients with LETM has only been sparsely studied.

Methods: A total of 30 NMOSD patients with LETM were identified in a database originating from a population-based retrospective case series with clinical and MRI follow-up. T2W, T1W with or without gadolinium (GD) and short tau inversion recovery sequences were analyzed in spinal cord imaging by a 1.5 Tesla scanner. The neuroradiologist was blinded to the clinical and serological results. AQP4-IgG was measured with a recombinant immunofluorescence assay and evaluated by a cell-based assay.

Results: Sixteen out of thirty NMOSD patients with LETM were AQP4-IgG positive (13 females, 81.2%) and fourteen were AQP4-IgG negative (8 females, 57.1%). The AQP4-IgG positive LETM group (12/16) had a significantly higher frequency of a long length LETM (≥5 vertebral segments involved) as compared to the AQP4-IgG negative patient group (2/14) (p< 0.001). Brainstem involvement and GD enhancement occurred more frequently in the AQP4-IgG positive NMOSD patients with LETM (p < 0.02), all females. At follow-up, six patients had recurrent LETM (all females), five of them were AQP4-IgG positive. Focal spinal cord atrophy at the site of previous LETM was only seen in AQP4-IgG negative patients. Five out of six patients with general spinal cord atrophy were AQP4-IgG positive, all females.

Conclusion: AQP4-IgG positive NMOSD patients with LETM had more frequent occurrence of long length LETM, recurrent LETM, more brainstem involvement and GD enhancement, thus suggesting that the MRI of the spinal cord may be useful in evaluation of NMOSD disease activity as well as in the diagnostic work-up.

Disclosure: Dejan Zelic: nothing to disclose

Hanne Pernille Bro Skejoe: nothing to disclose

Soeren Thue Lillevang: nothing to disclose

Nasrin Asgari: nothing to disclose

Study funding: The Danish Multiple Sclerosis Society, The Danish Foundation for Neurological Research, ​The Jacobsen Foundation, ​The Lundbeck Foundation, ​The Region of Southern Denmark Research Fund, and The University of Southern Denmark.​

Abstract: P1021

Type: Poster

Abstract Category: Pathology and pathogenesis of MS - Imaging

Background: Imaging criteria have been incorporated into the proposed diagnostic criteria for neuromyelitis optica spectrum disorder (NMOSD) and the diagnosis may be made solely based on clinical and magnetic resonance imaging (MRI)-based analysis. A common manifestation of NMOSD is the longitudinal extensive transverse myelitis (LETM). The clinical impact of aquaporin-4-immunoglobulin G (AQP4-IgG) positivity in the NMOSD patients with LETM has only been sparsely studied.

Methods: A total of 30 NMOSD patients with LETM were identified in a database originating from a population-based retrospective case series with clinical and MRI follow-up. T2W, T1W with or without gadolinium (GD) and short tau inversion recovery sequences were analyzed in spinal cord imaging by a 1.5 Tesla scanner. The neuroradiologist was blinded to the clinical and serological results. AQP4-IgG was measured with a recombinant immunofluorescence assay and evaluated by a cell-based assay.

Results: Sixteen out of thirty NMOSD patients with LETM were AQP4-IgG positive (13 females, 81.2%) and fourteen were AQP4-IgG negative (8 females, 57.1%). The AQP4-IgG positive LETM group (12/16) had a significantly higher frequency of a long length LETM (≥5 vertebral segments involved) as compared to the AQP4-IgG negative patient group (2/14) (p< 0.001). Brainstem involvement and GD enhancement occurred more frequently in the AQP4-IgG positive NMOSD patients with LETM (p < 0.02), all females. At follow-up, six patients had recurrent LETM (all females), five of them were AQP4-IgG positive. Focal spinal cord atrophy at the site of previous LETM was only seen in AQP4-IgG negative patients. Five out of six patients with general spinal cord atrophy were AQP4-IgG positive, all females.

Conclusion: AQP4-IgG positive NMOSD patients with LETM had more frequent occurrence of long length LETM, recurrent LETM, more brainstem involvement and GD enhancement, thus suggesting that the MRI of the spinal cord may be useful in evaluation of NMOSD disease activity as well as in the diagnostic work-up.

Disclosure: Dejan Zelic: nothing to disclose

Hanne Pernille Bro Skejoe: nothing to disclose

Soeren Thue Lillevang: nothing to disclose

Nasrin Asgari: nothing to disclose

Study funding: The Danish Multiple Sclerosis Society, The Danish Foundation for Neurological Research, ​The Jacobsen Foundation, ​The Lundbeck Foundation, ​The Region of Southern Denmark Research Fund, and The University of Southern Denmark.​

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