Autoimmune Autonomic Ganglionopathy Presenting as Constipation

Abstract

Autoimmune autonomic ganglionopathy (AAG) is a rare post-ganglionic disorder that causes a range of symptoms, often including gastrointestinal disorders. Patients may be seropositive or seronegative for antibodies against the nicotinic acetylcholine receptor. Here, we describe the case of a 56-year-old woman with a previous diagnosis of sensorimotor peripheral neuropathy who presented with severe constipation that was not responsive to laxative therapy. The evaluation showed diffuse colonic hypomotility, rectal hypersensitivity, and type IV pelvic floor dysfunction. The patient was diagnosed 10 months after the presentation as having seronegative AAG, and she responded well to treatment with intravenous methylprednisolone and apheresis.

A patient with neuropathy and ataxia: what do I have to consider?

“…Autoimmune autonomic ganglionopathy is uncommon and principally a postganglionic disorder believed to be due to antibodies targeting the nicotinic acetylcholine receptor. These antibodies may be idiopathic or part of a paraneoplastic process [63]. Dysautonomia is often a feature of MSAc [59 ▪ ] and may be seen in SCA1, 2, 3 [64], 27B [65], FA [66] and CANVAS [67,68] amongst others.…”