2279 Severe Refractory Antibody-Positive Autoimmune Pandysautonomia Post-Covid19 Vaccinaton: a Case Report

Background COVID-19 vaccine-associated peripheral and central neuroimmunological disorders have been well described. We present the case of a 57 year old male who developed α3-ganglionic AChR antibody positive Autoimmune Autonomic Ganglionopathy (AAG) after completion of a two-dose course of mRNA (Cominarty) vaccination for COVID19. Results A previously hypertensive 57 year old Vietnamese male presented with the subacute sequential onset of severe constipation, urinary retention, erectile dysfunction, sudomotor failure, sicca symptoms, non-reactive pupils and severe orthostatic hypotension shortly after receiving the second dose of an mRNA vaccine against COVID19. Autonomic testing revealed severe cardiovagal, adrenergic and sudomotor impairment, and tonic 'half-mast' pupils with evidence of sympathetic and parasympathetic denervation. Nerve conduction studies were normal. Investigations for common causes of autonomic failure were non-contributory to a diagnosis. Pathological α3-ganglionic ACHR antibodies were positive in serumas detected by a new flow cytometric immunomodulation assay. Malignancy was excluded. The patient was diagnosed with severe, treatment resistant acute pandysautonomia (AAG). Conclusions While autonomic dysfunction has been previously reported post-COVID19 vaccination, to our knowledge this is the first reported case of antibody-positive AAG in this setting. The severity of this case is in marked contrast to the existing literature on idiopathic antibody-positive autoimmune pandysautonomia.