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Abstract Details

11-year Follow-Up of a Case of Autoimmune Autonomic Ganglionopathy
Autonomic Disorders
P03 - (-)
024
BACKGROUND: AAG is an immune-mediated autonomic disorder usually presenting as acute pandysautonomia. The pathophysiology has been well characterized owing to the discovery of nicotinic ganglionic acetylcholine receptor antibodies in patients with AAG, though at least half of cases presents without detectable autoantibodies. Little is known about the long-term prognosis of AAG in general and seronegative AAG in particular.
DESIGN/METHODS: We followed a case of seronegative AAG for 11 years. In addition to routine clinical and laboratory assessments, standardized autonomic testing and standardized assessment of autonomic symptoms (composite autonomic symptom score, COMPASS) were completed.
RESULTS: The patient presented with subacute onset of abdominal pain, vomiting, bloating, constipation, orthostatic hypotension, heat intolerance, urinary retention, and erectile dysfunction. Autonomic reflex testing showed pandysautonomia (CASS score=9 out of 10). Immunomodulatory treatment resulted in negligible benefit and was discontinued. Upon re-evaluation one year later, most symptoms had modestly improved. CASS score was 7 related to improvement in cardiovagal function. Thermoregulatory sweat test showed global anhidrosis except for small islands of preserved sweating. By eleven years after symptom onset, most symptoms had improved markedly and the patient had returned to near normal functioning. Remaining symptoms included erectile dysfunction and lack of thermoregulatory abilities. COMPASS had improved from 61.3 (year 1) to 34 (year 11). There was continued severe sudomotor failure, improvement of cardiovascular adrenergic function, and normalization of cardiovagal function (CASS=5).
CONCLUSIONS: 11-year follow-up in a patient with seronegative AAG reveals definite but incomplete improvement of autonomic function and symptoms. Sudomotor and erectile function remained markedly impaired. This confirms prior short-term follow-up reports of an overall favorable prognosis but often incomplete recovery. The long duration of selected deficits would suggest structural impairment beyond functional blockade of ganglionic function.
Authors/Disclosures
Tonette Gehrking
PRESENTER 		Tonette Gehrking has nothing to disclose.
Michael D. Geschwind, MD, PhD, FAAN (UCSF) Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Brainstorm Cell Therapeutics, Inc.. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Walter Grubb. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gerson Lehrman Group. Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Reata Pharmaceuticals, Inc..
No disclosure on file
Robert D. Fealey, MD (Mayo Clinic/Dept of Neuro) No disclosure on file
Phillip A. Low, MD, FAAN (Mayo Clinic) Dr. Low has nothing to disclose.
Wolfgang Singer, MD, FAAN (Mayo Clinic) Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. The institution of Dr. Singer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Lundbeck. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ionis. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Yoda. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Theravance. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ferrer. The institution of Dr. Singer has received research support from NIH. The institution of Dr. Singer has received research support from FDA. The institution of Dr. Singer has received research support from Michael J. Fox Foundation. Dr. Singer has received intellectual property interests from a discovery or technology relating to health care.