Abstract Details

Title Autoimmune Dysautonomia and Ataxia due to Ganglionic Acetylcholine Receptor Autoantibodies

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (12:00 PM-1:00 PM)

Poster/Presentation
Number 15-007

Objective

To report a rare case of a young woman presenting with dysautonomia and cerebellar ataxia due to ganglionic acetylcholine receptor antibodies causing autoimmune autonomic gangionopathy (AAG).

Background

AAG is a rare subacute disorder associated with antibodies against ganglionic acetylcholine receptors. Ganglionic acetylcholine receptor antibodies have been reported to also be associated with other neurological disorders unrelated to the autonomic nervous system, such as ataxia.

Design/Methods Our patient is a 26 year old female who in March 2018 began having lightheadedness, stiffness in her neck and shoulder girdle, and difficulty lifting her arms. She also felt like just touch on her skin would cause muscle spasms, and muscle spasms would occur triggered by movement. She had frequent muscle spasms of the arms >legs, left>right, which caused her to fall a couple of times. She actually needed a walker at one point because of the leg stiffness. She had slurring of speech and limb ataxia. She was having to strain to urinate and also had constipation. She had abnormal sweating, and tended to sweat a lot at night. Cold especially triggered some spasms. Her exam was notable for gait imbalance, slurring of speech, and excessive saliva.

Results Laboratory evaluation revealed an elevated α3-AChR Ab at 62 pmol/L (normal <53 pmol/L) with no other autoantibodies or infectious etiology detected. Thorough screening revealed no evidence of associated malignancy. Paraneoplastic panel otherwise only showed fluorescence on monkey cerebellum substrate, and IgG index was positive in CSF. She was subsequently started on intravenous immunoglobulin treatment.

Conclusions AAG is a clinically heterogeneous disease with variable presentation, particularly in those with lower antibody titers. This case suggests including α3-AChR Ab in the evaluation of dysautonomia and cerebellar dysfunction in a young adult, and highlights the importance of further understanding α3-AChR within the brain.

Authors/Disclosures
Duarte G. Machado, MD, F�鶹��ýӳ�� (Hartford Healthcare Neurology) PRESENTER	Dr. Machado has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Supernus. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurocrine. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbvie. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Adamas. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Ipsen. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Kyowa Kirin. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merz. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Neurocrine. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sunovion. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva.
Alison L. Carlson, NP (Chase Movement Disorders Center)	No disclosure on file

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