Seronegative and seropositive autoimmune autonomic ganglionopathy (AAG): Same clinical picture, same response to immunotherapy

Two patients with a syndrome of pandisautonomia with clinical criteria of AAG are provided. Both patients present a similar clinical picture and response to immunosuppressive treatment. One of them has positive antibodies against the ganglionic nicotinic acetylcholine (gAChr) and the other does not. This brief article serves to reflect the spectrum of AAG, at a clinical level, in laboratory tests and in the response to immunotherapy, independently of the presence of positive gAChr antibodies. [Display omitted] •The autoimmune autonomic ganglionopathy (AAG) is an extremely rare form of dysautonomia•Ganglionic nicotinic acetylcholine receptor antibodies (gAChr) are detected in about 50% of the patients•The neuro-hormonal study combined with cardiac MIBG scintigraphy may help to differentiate it from a patient with PAF•AAG responds to immunotherapy, independently of the presence of positive antibodies (gAChr)