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Abstract Details

Clinical Characteristics of Acquired Chronic Autoimmune Polyneuropathies Associated with Nodal/Paranodal Autoantibodies at UCSD Neuromuscular Center
Neuromuscular and Clinical Neurophysiology (EMG)
Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)
074

To describe the clinical and electrophysiologic characteristics of patients with chronic autoimmune polyneuropathy associated with anti-nodal/paranodal autoantibodies and gather evidence for their potential role as biomarkers of clinical phenotype and response to treatment. 
Recent studies have demonstrated a potential link between chronic inflammatory demyelinating polyneuropathy (CIDP) and autoantibodies against the nodal/paranodal proteins targeting neurofascin 155, neurofascin 140/186, contactin 1. These antibodies could be responsible for differences in temporal evolution and severity of motor or sensory symptoms. Certain subgroups of CIDP harboring these antibodies may not respond well to immunomodulatory therapy. Further research is needed to evaluate specific correlations with regards to clinical, electrophysiological features as well as preferred treatment method in these individuals. 
A review of patients with chronic autoimmune (demyelinating and axonal type) polyneuropathy at UCSD neuromuscular clinic from October 2019 until March 2020 were evaluated for presence of antibodies with testing at Washington University Neuromuscular Laboratory. Demographic, clinical, electrodiagnostic information, and treatment response of this group were analyzed
Five of twenty-one patients tested positive for the antibodies.  Three of these had predominantly distal motor > sensory involvement while the other two had length dependent large fiber sensory deficits with neuropathic pain.  Primary demyelinating features was present in three and axonal pathology in two of them.  Four patients failed to respond to IVIG.  One of these patients had a mild response to high dose steroids with IVIG. Two patients experienced objective clinical improvement with Rituximab infusions
Although the clinical significance and pathogenicity of these autoantibodies remain uncertain, they could be objective markers of autoimmune neuropathies and guide treatment.  Research involving independent large studies are needed to address these questions.  
Authors/Disclosures
Jonathan Peters, MD (UCSD Neurosciences Department)
PRESENTER 		Dr. Peters has nothing to disclose.
Wali Qayoumi, MD (UCSD Neurology) Dr. Qayoumi has received personal compensation in the range of $0-$499 for serving as a Consultant for Atheneum. Dr. Qayoumi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Guidepoint.
Betul B. Gundogdu, MD (UCSD) Dr. Gundogdu has nothing to disclose.