Utility of the CNS Demyelinating Disease Panel in Patients Presenting with Atypical Demyelinating Attacks
Manisha Ramprasad1, Alessandro Serra2, Hesham Abboud2
1Case Western Reserve University School of Medicine, 2University Hospitals Cleveland Medical Center
Objective:
To evaluate predictors of a positive CNS demyelinating disease panel (CDDP) in patients with atypical demyelinating attacks at a tertiary neuroimmunology clinic.
Background:
CDDP is a serum cell-based assay for antibodies against aquaporin-4 water channel (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG). CDDP is recommended for evaluating atypical demyelinating attacks not suggestive of MS, but its clinical utility has not been fully explored.
Design/Methods:
We retrospectively evaluated patients referred to a tertiary neuroimmunology clinic who were tested for CDDP at Mayo Clinic Laboratories as part of a workup for an atypical demyelinating attack.
Results:
From 2017 to 2024, 66 patients were tested for CDDP for an atypical demyelinating attack. Twenty-nine patients (43% of total) tested positive for MOG-IgG (including 8 patients deemed to have false positive antibody); 19 patients (28.4%) tested positive for AQP4-IgG (no false positives), and 18 patients (26.9%) tested negative for both. The rate of true positive panel was 60% with a sensitivity of 100% and specificity of 69%. Longitudinally-extensive optic neuritis predicted true positive MOG-IgG. Transverse myelitis, bowel dysfunction, tonic spasms, and spinal tremor predicted positive AQP4-IgG. Eye pain, focal dystonia, and a high IgG synthesis rate predicted a negative panel. Among patients presenting with atypical optic neuritis at testing (20, 29.9% of total), predictors of a true positive MOG-IgG test included steroid-responsiveness and normal spine MRI. Among patients presenting with atypical transverse myelitis (17, 25.8%), predictors of positive AQP4-IgG included bowel dysfunction and tonic spasms.
Conclusions:
Among patients presenting with atypical demyelinating attacks, the rate of true positive CDDP is high (60%) with high sensitivity and specificity. Among those, predictors of a negative test include painful optic neuritis, focal dystonia, and high IgG synthesis rate. Predictors of positive AQP4-IgG include transverse myelitis, tonic spasms, spinal tremor, and bowel dysfunction. Predictors of true positive MOG-IgG include longitudinally-extensive optic neuritis and steroid-responsiveness.
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