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Abstract Details

Acetylcholine Receptor Antibodies in a patient with Sensory Neuropathy
Neuromuscular and Clinical Neurophysiology (EMG)
P15 - Poster Session 15 (12:00 PM-1:00 PM)
1-002

To report a case of axonal sensory motor neuropathy with persistent high titers of binding and modulating acetylcholine receptor (AChR) and striational (Str) muscle antibodies (Ab).

 A 68-year-old man presented with progressive sensory complaints devloping over few years beginning in his feet including painful paresthesias and the sensation that his feet were swollen. His neurologic examination showed absence of vibratory sensibility in his toes, present although reduced at ankles. Pain and light touch were normal on his face and lower extremities and sensation was normal in his upper extremities. Over the next 8 years there was no progression in symptoms or changes in his examination. He’s never had complaints of weakness or motor symtoms or findings referable to his cranial nerves over subsequent followup. 

Information regarding patient obtained via retrospective review of electronic medical records.
ANA was positive (1:2560; homogeneous pattern), as was ENA (SS-B antibodies were 67.8, normal <24). His ESR and CRP have always been normal as have multiple examinations for a serum paraprotein. A paraneoplastic panel was obtained with AChR binding antibody of 5.58 nmol/l (normal <0.02), modulating Ab of 100% (normal 0-20%) and Str antibody of 1:3840 (normal < 1:120). Eight years after onset of symptoms he continues to demonstrate ANA, SS-B antibodies and Str, AChR binding and modulating Ab. Chest CT on two occasions 5 and 8 years after onset of symptoms showed no evidence of thymoma or other neoplastic process. 
The presence of antibodies to nicotinic muscle acetylcholine receptor is very sensitive and highly specific serologic test for myasthenia gravis (MG). They are rarely observed in patients with other autoimmune diseases and have often been transient. High titer modulating Ab do not seem to have been reported. Cases such as this, although rare, emphasize the importance of ensuring that autoantibodies fit the clinical picture. 
Authors/Disclosures
Shitiz K. Sriwastava, MBBS (UT Health Houston)
PRESENTER
Dr. Sriwastava has nothing to disclose.
Robert P. Lisak, MD, FÂé¶¹´«Ã½Ó³»­ (Wayne State Univ Sch of Med, Dept of Neuro) Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argnex. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for GLG Consulting. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Third Bridge Consulting. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Clearview Consulting. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alivar Therapeutics. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ANI Pharmaceuticals. Dr. Lisak has received personal compensation in the range of $0-$499 for serving as a Consultant for Coleman Research. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunic. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Guidepoint Consulting. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Tedus Consulting. Dr. Lisak has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for COUR Pharma. The institution of Dr. Lisak has received research support from NINDS. The institution of Dr. Lisak has received research support from Argenx. The institution of Dr. Lisak has received research support from Ra Pharmaceuticals. Dr. Lisak has received publishing royalties from a publication relating to health care. Dr. Lisak has received publishing royalties from a publication relating to health care.