Recurrent autonomic and sensory neuropathy in a patient with anti-ganglionic acetylcholine receptor antibodies

Abstract

We report a case of recurrent neuropathy with predominant autonomic and sensory involvement whose serum was positive for anti-ganglionic acetylcholine receptor (anti-gAChR) antibodies, a diagnostic marker of autoimmune autonomic ganglionopathy. An 11-year-old girl complained of numbness and limb pain after gastroenteritis. Although hyperalgesia and autonomic dysfunctions, such as orthostatic intolerance and gastrointestinal dysmotility subsequently developed, these symptoms faded after a few days. Similar sensory and autonomic impairments recurred three times within 12 months after the first episode. The sensory and autonomic symptoms were rapidly ameliorated by the administration of intravenous immunoglobulin (IVIg) at the second and third relapse; however, the symptoms persisted even after the administration of IVIg at the fourth relapse. The residual symptoms disappeared after methylprednisolone pulse therapy. The patient's serum was found to be positive for anti-gAChR antibodies at the second relapse, and was negative after methylprednisolone pulse therapy. Further studies are needed to clarify the efficacy of treatment and the nosological position in the spectrum of neuropathies that are associated with autonomic and sensory impairments. Keywords: Ganglionomic neuronal nicotinic acetylcholine receptors, Orthostatic hypotension, Acute autonomic and sensory neuropathy, Autoimmune autonomic ganglionopathy, Autoimmune diseases