TY - JOUR
T1 - Retinal Changes in Double-Antibody Seronegative Neuromyelitis Optica Spectrum Disorders
AU - Oertel, Frederike C
AU - Zimmermann, Hanna G
AU - Motamedi, Seyedamirhosein
AU - Bereuter, Charlotte
AU - Manthey, Luca Magdalena
AU - Ashtari, Fereshteh
AU - Kafieh, Rahele
AU - Dehghani, Alireza
AU - Pourazizi, Mohsen
AU - Pandit, Lekha
AU - D'Cunha, Anitha
AU - Aktas, Orhan
AU - Albrecht, Philipp
AU - Ringelstein, Marius
AU - Martinez-Lapiscina, Elena H
AU - Sanchez Dalmau, Bernardo F
AU - Villoslada, Pablo
AU - Asgari, Nasrin
AU - Marignier, Romain
AU - Cobo-Calvo, Alvaro
AU - Leocani, Letizia
AU - Pisa, Marco
AU - Radaelli, Marta
AU - Palace, Jacqueline
AU - Roca-Fernandez, Adriana
AU - Leite, Maria Isabel S
AU - Sharma, Srilakshmi
AU - De Seze, Jerome
AU - Senger, Thomas
AU - Yeaman, Michael R
AU - Smith, Terry J
AU - Cook, Lawrence J
AU - Brandt, Alexander U
AU - Paul, Friedemann
AU - Guthy-Jackson Charitable Foundation
PY - 2024/9
Y1 - 2024/9
N2 - BACKGROUND AND OBJECTIVES: To systematically describe the clinical picture of double-antibody seronegative neuromyelitis optica spectrum disorders (DN-NMOSD) with specific emphasis on retinal involvement.METHODS: Cross-sectional data of 25 people with DN-NMOSD (48 eyes) with and without a history of optic neuritis (ON) were included in this study along with data from 25 people with aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD, 46 eyes) and from 25 healthy controls (HCs, 49 eyes) for comparison. All groups were matched for age and sex and included from the collaborative retrospective study of retinal optical coherence tomography (OCT) in neuromyelitis optica (CROCTINO). Participants underwent OCT with central postprocessing and local neurologic examination and antibody testing. Retinal neurodegeneration was quantified as peripapillary retinal nerve fiber layer thickness (pRNFL) and combined ganglion cell and inner plexiform layer thickness (GCIPL).RESULTS: This DN-NMOSD cohort had a history of [median (inter-quartile range)] 6 (5; 9) attacks within their 5 ± 4 years since onset. Myelitis and ON were the most common attack types. In DN-NMOSD eyes after ON, pRNFL (p < 0.001) and GCIPL (p = 0.023) were thinner compared with eyes of HCs. Even after only one ON episode, DN-NMOSD eyes already had considerable neuroaxonal loss compared with HCs. In DN-NMOSD eyes without a history of ON, pRNFL (p = 0.027) and GCIPL (p = 0.022) were also reduced compared with eyes of HCs. However, there was no difference in pRNFL and GCIPL between DN-NMOSD and AQP4-NMOSD for the whole group and for subsets with a history of ON and without a history of ON-as well as between variances of retinal layer thicknesses.DISCUSSION: DN-NMOSD is characterized by severe retinal damage after ON and attack-independent retinal neurodegeneration. Most of the damage occurs during the first ON episode, which highlights the need for better diagnostic markers in DN-NMOSD to facilitate an earlier diagnosis as well as for effective and early treatments. In this study, people with DN-NMOSD presented with homogeneous clinical and imaging findings potentially suggesting a common retinal pathology in these patients.
AB - BACKGROUND AND OBJECTIVES: To systematically describe the clinical picture of double-antibody seronegative neuromyelitis optica spectrum disorders (DN-NMOSD) with specific emphasis on retinal involvement.METHODS: Cross-sectional data of 25 people with DN-NMOSD (48 eyes) with and without a history of optic neuritis (ON) were included in this study along with data from 25 people with aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD, 46 eyes) and from 25 healthy controls (HCs, 49 eyes) for comparison. All groups were matched for age and sex and included from the collaborative retrospective study of retinal optical coherence tomography (OCT) in neuromyelitis optica (CROCTINO). Participants underwent OCT with central postprocessing and local neurologic examination and antibody testing. Retinal neurodegeneration was quantified as peripapillary retinal nerve fiber layer thickness (pRNFL) and combined ganglion cell and inner plexiform layer thickness (GCIPL).RESULTS: This DN-NMOSD cohort had a history of [median (inter-quartile range)] 6 (5; 9) attacks within their 5 ± 4 years since onset. Myelitis and ON were the most common attack types. In DN-NMOSD eyes after ON, pRNFL (p < 0.001) and GCIPL (p = 0.023) were thinner compared with eyes of HCs. Even after only one ON episode, DN-NMOSD eyes already had considerable neuroaxonal loss compared with HCs. In DN-NMOSD eyes without a history of ON, pRNFL (p = 0.027) and GCIPL (p = 0.022) were also reduced compared with eyes of HCs. However, there was no difference in pRNFL and GCIPL between DN-NMOSD and AQP4-NMOSD for the whole group and for subsets with a history of ON and without a history of ON-as well as between variances of retinal layer thicknesses.DISCUSSION: DN-NMOSD is characterized by severe retinal damage after ON and attack-independent retinal neurodegeneration. Most of the damage occurs during the first ON episode, which highlights the need for better diagnostic markers in DN-NMOSD to facilitate an earlier diagnosis as well as for effective and early treatments. In this study, people with DN-NMOSD presented with homogeneous clinical and imaging findings potentially suggesting a common retinal pathology in these patients.
KW - Humans
KW - Neuromyelitis Optica/diagnostic imaging
KW - Female
KW - Male
KW - Adult
KW - Cross-Sectional Studies
KW - Middle Aged
KW - Tomography, Optical Coherence
KW - Aquaporin 4/immunology
KW - Retrospective Studies
KW - Autoantibodies/blood
KW - Retina/diagnostic imaging
U2 - 10.1212/NXI.0000000000200273
DO - 10.1212/NXI.0000000000200273
M3 - Article
C2 - 38941573
SN - 2332-7812
VL - 11
JO - Neurology: Neuroimmunology and NeuroInflammation
JF - Neurology: Neuroimmunology and NeuroInflammation
IS - 5
M1 - e200273
ER -