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[Acute-onset autoimmune autonomic ganglionopathy remarkably effective in intravenous high-dose immunoglobulin therapy].
- Source :
-
Rinsho shinkeigaku = Clinical neurology [Rinsho Shinkeigaku] 2021 Oct 28; Vol. 61 (10), pp. 687-691. Date of Electronic Publication: 2021 Sep 25. - Publication Year :
- 2021
-
Abstract
- 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.
- Subjects :
- Aged
Constipation
Female
Humans
Hypotension, Orthostatic diagnosis
Hypotension, Orthostatic drug therapy
Hypotension, Orthostatic etiology
Immunization, Passive
Peripheral Nervous System Diseases
Urinary Retention
Autoimmune Diseases diagnosis
Autoimmune Diseases drug therapy
Immunoglobulins, Intravenous administration & dosage
Immunoglobulins, Intravenous therapeutic use
Primary Dysautonomias
Subjects
Details
- Language :
- Japanese
- ISSN :
- 1882-0654
- Volume :
- 61
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- Rinsho shinkeigaku = Clinical neurology
- Publication Type :
- Academic Journal
- Accession number :
- 34565756
- Full Text :
- https://doi.org/10.5692/clinicalneurol.cn-001631