Highlights
- •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)
Abstract
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.
Graphical abstract
Graphical Abstract
Keywords
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Article info
Publication history
Published online: April 03, 2018
Accepted:
March 28,
2018
Received in revised form:
March 24,
2018
Received:
February 25,
2018
Identification
Copyright
© 2018 Elsevier B.V. All rights reserved.
