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Abstract Details

Clinical Characterization of Neurofascin-155 IgG4 and Contactin-1 IgG Dual Positive Neuropathies
Autoimmune Neurology
P4 - Poster Session 4 (11:45 AM-12:45 PM)
14-003
To evaluate the frequency and characterize the clinical phenotype of patients positive for both neurofascin-155 and contactin-1 antibodies.
Clinical features associated with neurofascin-155 IgG4 or contactin-1 IgG seropositive autoimmune nodopathies have been described. However, clinical description of those with neurofascin-155 and contactin-1 autoantibody dual positive is lacking
Patients were identified through service neural autoantibody evaluation at Mayo Clinic Neuroimmunology Laboratory. Clinical information was gathered from the managing provider.
Among 1194 patients tested for autoimmune nodopathy panel between August 2022 and October 2023, 10 (0.8%; 1 female, 9 male) were positive for both antibodies. Clinical information was available for six patients. Median age of symptom onset was 64 years (range: 33-82). All patients were clinically and electrodiagnostically suspected to have chronic inflammatory demyelinating polyneuropathy. Median time from symptom onset to nadir was four months (range: 1.5-6). Among patients who underwent CSF evaluation (n=4), median nucleated cell count was 2.5 cells/mm3 (range: 1-9), and median protein was 255 mg/dL (range: 59-627). All nerve conduction studies (NCS) demonstrated polyradiculoneuropathy phenotype. Only one NCS was purely demyelinating but the majority (5/6, 83%) had features of demyelination and co-existing axonal loss. Additional symptoms included neuropathic pain (3, 50%), cranial neuropathy (3, 50%), dysautonomia (2, 33%), ataxia (5, 83%) and tremors (2, 33%). Membranous glomerulonephropathy was confirmed in one (17%). Median modified Rankin score at nadir was 5 (range: 3-5) and following immunotherapy including rituximab was 1 (range: 1-5) at the last follow up (n=5), with three improving by >1 point
Neurofascin-155 and contactin-1 autoantibody dual seropositivity is rarely encountered. Our study demonstrates these patients have clinical features and treatment response resembling other neurofascin-155/contactin-1 IgG autoimmune nodopathies. Additional experimental studies are needed to evaluate the clinical and analytical specificity of these antibody results.
Authors/Disclosures
Shemonti Hasan, MD
PRESENTER 		Dr. Hasan has nothing to disclose.
Haidara Kherbek (Mayo Clinic) Haidara Kherbek has nothing to disclose.
Pallab Sarker (Mayo Clinic) Pallab Sarker has nothing to disclose.
Yoonjae Nam (Mayo Clinic) No disclosure on file
Aaron L. Berkowitz, MD, PhD, FAAN Dr. Berkowitz has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for AAN. Dr. Berkowitz has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for McGraw-Hill 好色先生. Dr. Berkowitz has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for various law firms. Dr. Berkowitz has received publishing royalties from a publication relating to health care. Dr. Berkowitz has received publishing royalties from a publication relating to health care. Dr. Berkowitz has received publishing royalties from a publication relating to health care. Dr. Berkowitz has received publishing royalties from a publication relating to health care. Dr. Berkowitz has received publishing royalties from a publication relating to health care. Dr. Berkowitz has received personal compensation in the range of $500-$4,999 for serving as a Content creator with Clinical problem Solvers. Dr. Berkowitz has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant with Thieme Publisher.
Alan D. Salgado, MD Dr. Salgado has nothing to disclose.
Kyle A. Wuthrich, DO (USF Health Neurology) Dr. Wuthrich has nothing to disclose.
Christopher J. Klein, MD, FAAN (Mayo Clinic) Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Pharma.
John R. Mills, MD, PhD (Mayo Clinic) The institution of Dr. Mills has received research support from Werfen Diagnostics. Dr. Mills has received intellectual property interests from a discovery or technology relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic) The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.