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Abstract Details

NT5c1A Antibodies in Amyotrophic Lateral Sclerosis
Neuromuscular and Clinical Neurophysiology (EMG)
Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)
040

To describe a case series of eight patients with NT5c1A antibody positivity and subsequent diagnosis of Amyotrophic Lateral Sclerosis (ALS).

NT5c1A antibody is frequently seen in sporadic inclusion body myositis (IBM), with reported sensitivity and specificity of up to 70% and 92% respectively. While it may be seen in other systemic disorders such as Sjogren’s and systemic lupus erythematous, it is not seen in other neuromuscular disorders.
A retrospective chart review of eight patients with serum positive NT5C1A antibodies with an eventual diagnosis of motor neuron disease at the University of California, Irvine from January 2017 to August 2020 was performed.
In this cohort of eight patients, three were men and five were women, with the mean age of 70 years old. Ethnicities included Hispanic, Asian and Caucasian. Symptom duration at the time of initial consultation ranged from three months to three years. Presentations varied and included progressive dysarthria and dysphagia, proximal more than distal lower extremity weakness, symmetric or asymmetric distal more than proximal upper extremity weakness. Creatine kinase (CK) ranged from 44-257 U/L. Electrodiagnostic testing in five out of the eight patient revealed chronic and ongoing motor neuron changes in the regions tested, while two patients had short duration units with early recruitment pattern. Electrodiagnostic testing was not performed in one patient.

NT5c1A antibodies can be seen in individuals with ALS and further studies may be beneficial to determine the frequency of NT5c1A antibody positivity in this population.

Authors/Disclosures
Shadi Milani, DO
PRESENTER
Dr. Milani has nothing to disclose.
Michael C. Hunter, MD (Intermountain Healthcare) Dr. Hunter has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Hunter has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Amgen.
Manisha Kak Korb, MD Dr. Kak Korb has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Kak Korb has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CSL Behring. Dr. Kak Korb has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Kak Korb has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen.
Ali A. Habib, MD (University of California, Irvine) Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for argenx. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunovant. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech/Roche. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for NMD Pharma. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon/ Amgen. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Jansen/Johnson & Johnson. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMDSerono/Merck. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH/NINDS. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunis Biomedical. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffman-La Roche. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for argenx. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Jansen/Joohnson & Johnson. The institution of Dr. Habib has received research support from Alexion. The institution of Dr. Habib has received research support from Horizon/Amgen. The institution of Dr. Habib has received research support from Immunovant. The institution of Dr. Habib has received research support from UCB. The institution of Dr. Habib has received research support from argenx. The institution of Dr. Habib has received research support from CabalettaBio. The institution of Dr. Habib has received research support from Regeneron. The institution of Dr. Habib has received research support from Arcellx. The institution of Dr. Habib has received research support from Novartis. The institution of Dr. Habib has received research support from EMDSerono/Merck. The institution of Dr. Habib has received research support from Cour Pharmaceuticals.