Author Of 1 Presentation
YI02.04 - Comparison of clinical characterization, risk of relapses and antibody dynamics between children and adults with MOGAD
- A. Cobo Calvo
- A. Ruiz
- F. Rollot
- G. Arrambide
- R. Deschamps
- E. Maillart
- C. Papeix
- B. Audoin
- A. Lépine
- H. Maurey
- H. Zephir
- D. Biotti
- J. Ciron
- F. Durand-Dubief
- N. Collongues
- X. Ayrignac
- P. Labauge
- P. Meyer
- E. Thouvenot
- B. Bourre
- A. Montcuquet
- M. Cohen
- P. Horellou
- M. Tintore
- J. De Seze
- S. Vukusic
- K. Deiva
- R. Marignier
Abstract
Background
To predict the clinical course of myelin oligodendrocyte glycoprotein (MOG)-antibody (Ab)-associated disease (MOGAD) is essential to guide treatment recommendations.
Objectives
We aimed to 1) compare clinical features and disease course, and 2) to evaluate the association of MOG-Ab dynamics and relapses, between children and adults with MOGAD.
Methods
Retrospective study evaluating clinical features of 98 children and 266 adults with MOGAD, between January 2014 and September 2019. To analyse relapses over the whole disease course, a Cox regression analysis for recurrent time-to-event data was performed, introducing treatment as time-dependent covariate. To evaluate dynamics, delta mean fluorescence intensity ratio signal (ΔMFIratio) of MOG-Ab was measured in patients with a minimum time elapsed between two samples of 4 months.
Results
Median age at onset of symptoms was 10.9 (interquartile range 5.4-14.3) years in children and 36.2 (27.7-47.6) in adults. Isolated optic neuritis was the most frequent clinical presentation both in children (40.8%) and adults (55.9%), p=0.013, and acute disseminated encephalomyelitis syndrome was more frequent in children (36.7% vs. 5.6%; p<0.001). Compared to adults, children displayed a better recovery (EDSS ≥3.0 at last follow-up reached only by 10 of 97 [10.3%] vs. 66/247 [26.7%], p<0.001).
In the multivariate analysis, adults were at higher risk of relapse than children (Hazard ratio 1.41, 95%Confidence interval [CI] 1.12-1.78; p=0.003). Among the 124 participants evaluated for MOG-Ab dynamics, 36.3% became seronegative, 60.5% decrease and 3.2% increase the ΔMFIratio. At two years, 64.2% (95%CI 40.9-86.5) of non-relapsing children became MOG-Ab negative compared to 14.1% (95%CI 4.7-38.3) of relapsing ones, log-rank p<0.001, with no differences observed between non-relapsing and relapsing adults, log-rank p=0.280.
Conclusions
MOGAD differs in its clinical presentation at onset, showing a progressive shift in the clinical features across age-groups. Compared to children, adults have a higher risk of relapses and a worse functional recovery. Finally, children with monophasic disease became MOG-Ab negative earlier than relapsing ones, but not in adults. Considering these differences, management and treatment guidelines should be considered independently in children and adults.
Author Of 1 Presentation
P0246 - Clinical relevance of Kappa Free Light Chain index to evaluate intrathecal IgG synthesis: prospective study compared to oligoclobal bands (ID 1489)
Abstract
Background
An early and specific diagnosis of MS leads to an early treatment to better control the disease and its progression. Importance of immunoglobulin G (Ig G) synthesis in cerebrospinal fluid (CSF) has been re-emphasized for multiple sclerosis (MS) diagnosis since Mc Donald 2017 criteria. This synthesis is currently measured through the oligoclonal bands (OCB) using isoelectric focusing (ISE) technic, which is a time-consuming, expensive and may be difficult to interpret. Measurement of kappa free light chain (KFLC) in CSF and serum could offer a faster, standardized and less expensive way to evaluate the intrathecal Ig G synthesis.
Objectives
Primary objective is to test the relevance of KFLC index measurement in the diagnosis of MS compared with OCB measurement, using sensitivity and specificity of KFLC index in the diagnosis of MS. Secondary objective is to calculate a KFLC index threshold defining the IgG intrathecal synthesis. Methods: This is a prospective study including consecutively patients admitted in MS centre and/or other department of neurology at Lille University Hospital, who needed to undergo CSF lumbar puncture for CSF analysis. OCB and KFLC indexes were performed for all of them. KFLC index measured by turbidimetric assay on a SPAplus@ (The Binding Site Birmingham, UK).
Methods
We plan to include 250 patients in the study. Preliminary results concern 94 patients: 28 MS patients fulfilling 2017 MS criteria, 9 patients presenting CIS, 15 patients having other inflammatory disease (OID), 42 patients having non inflammatory disease (NOID). All MS patients who had OCB in CSF presented a KFLC index with a median value of 104.9 [6.43-649.7]; 3 MS patients who did not have OCB in CSF presented a median KFLC of 21.6 [10.1-23.4]. Height OID patients and 2 NOID patients had OCB in CSF; OID patients had a median KFLC value of 3.71 [1.7-600.8] and NOID of 3.2 [1.1-162.2].
Results
We plan to include 250 patients in the study. Preliminary results concern 94 patients: 28 MS patients fulfilling 2017 MS criteria, 9 patients presenting CIS, 15 patients having other inflammatory disease (OID), 42 patients having non inflammatory disease (NOID). All MS patients who had OCB in CSF presented a KFLC index with a median value of 104.9 [6.43-649.7]; 3 MS patients who did not have OCB in CSF presented a median KFLC of 21.6 [10.1-23.4]. Height OID patients and 2 NOID patients had OCB in CSF; OID patients had a median KFLC value of 3.71 [1.7-600.8] and NOID of 3.2 [1.1-162.2].
Conclusions
KFLC index seems to be more sensitive than OCB in the diagnosis of MS. Sensitivity, specificity and threshold of KFLC index defining the intrathecal synthesis will be evaluated with the next patients studied.
Presenter Of 1 Presentation
P0246 - Clinical relevance of Kappa Free Light Chain index to evaluate intrathecal IgG synthesis: prospective study compared to oligoclobal bands (ID 1489)
Abstract
Background
An early and specific diagnosis of MS leads to an early treatment to better control the disease and its progression. Importance of immunoglobulin G (Ig G) synthesis in cerebrospinal fluid (CSF) has been re-emphasized for multiple sclerosis (MS) diagnosis since Mc Donald 2017 criteria. This synthesis is currently measured through the oligoclonal bands (OCB) using isoelectric focusing (ISE) technic, which is a time-consuming, expensive and may be difficult to interpret. Measurement of kappa free light chain (KFLC) in CSF and serum could offer a faster, standardized and less expensive way to evaluate the intrathecal Ig G synthesis.
Objectives
Primary objective is to test the relevance of KFLC index measurement in the diagnosis of MS compared with OCB measurement, using sensitivity and specificity of KFLC index in the diagnosis of MS. Secondary objective is to calculate a KFLC index threshold defining the IgG intrathecal synthesis. Methods: This is a prospective study including consecutively patients admitted in MS centre and/or other department of neurology at Lille University Hospital, who needed to undergo CSF lumbar puncture for CSF analysis. OCB and KFLC indexes were performed for all of them. KFLC index measured by turbidimetric assay on a SPAplus@ (The Binding Site Birmingham, UK).
Methods
We plan to include 250 patients in the study. Preliminary results concern 94 patients: 28 MS patients fulfilling 2017 MS criteria, 9 patients presenting CIS, 15 patients having other inflammatory disease (OID), 42 patients having non inflammatory disease (NOID). All MS patients who had OCB in CSF presented a KFLC index with a median value of 104.9 [6.43-649.7]; 3 MS patients who did not have OCB in CSF presented a median KFLC of 21.6 [10.1-23.4]. Height OID patients and 2 NOID patients had OCB in CSF; OID patients had a median KFLC value of 3.71 [1.7-600.8] and NOID of 3.2 [1.1-162.2].
Results
We plan to include 250 patients in the study. Preliminary results concern 94 patients: 28 MS patients fulfilling 2017 MS criteria, 9 patients presenting CIS, 15 patients having other inflammatory disease (OID), 42 patients having non inflammatory disease (NOID). All MS patients who had OCB in CSF presented a KFLC index with a median value of 104.9 [6.43-649.7]; 3 MS patients who did not have OCB in CSF presented a median KFLC of 21.6 [10.1-23.4]. Height OID patients and 2 NOID patients had OCB in CSF; OID patients had a median KFLC value of 3.71 [1.7-600.8] and NOID of 3.2 [1.1-162.2].
Conclusions
KFLC index seems to be more sensitive than OCB in the diagnosis of MS. Sensitivity, specificity and threshold of KFLC index defining the intrathecal synthesis will be evaluated with the next patients studied.