Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Acute-onset autoimmune autonomic ganglionopathy remarkably effective in intravenous high-dose immunoglobulin therapy

Keishu Murakami, M.D.1j, Jinsoo Koh, M.D., Ph.D.1j, Maiko Takahashi, M.D.1j and Hidefumi Ito, M.D., Ph.D.1j

1) Department of Neurology, Wakayama Medical University

A 77-year-old woman developed acute onset of orthostatic hypotension, urinary retention, and constipation. Neurological examination on admission showed severe orthostatic hypotension accompanied by syncope, mydriatic pupils, and attenuation of light reflexes with no abnormalities in other neurological systems. Autonomic testing revealed denervation hypersensitivity in norepinephrine (NE) intravenous infusion test and 0.125% pilocarpine instillation test, low NE in the serum, and decreased amount of sweating in quantitative sudomotor axon reflex test. These findings indicated dysfunction of postganglionic autonomic nerves. Autoimmune autonomic ganglionopathy (AAG) was diagnosed due to the presence of anti-ganglionic acetylcholine receptors. The patient was given intravenous high-dose immunoglobulin therapy (IVIg), improving orthostatic hypotension, urinary retention, and constipation. Previous reports indicated that the response to IVIg varied from case to case. Thus, this case suggests that IVIg is effective in acute-onset AAG cases.
Full Text of this Article in Japanese PDF (571K)

(CLINICA NEUROL, 61: 687|691, 2021)
key words: autoimmune autonomic ganglionopathy, intravenous high-dose immuneoglobulin therapy, acute-onset

(Received: 21-Apr-21)