Autoimmune autonomic ganglionopathy manifesting as acute-onset orthostatic hypotension in a patient undergoing peritoneal dialysis

Hara, Masatoshi; Yamada, S.; Nakamura, Yuuri; Oka, Hideaki; Kamimura, Taro; Nakane, Shunya; Tsuruya, Kazuhiko; Harada, Atsumi

doi:10.1007/s13730-015-0180-3

Cited by 3 publications

(2 citation statements)

References 20 publications

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“…However, some patients develop orthostatic hypotension that is caused by dysfunction of the ANS, not by diabetic or amyloidosis-related neuropathy. We encountered a case of acute-onset orthostatic hypotension with hypospadias and erectile dysfunction in a 56-year-old man with a 17-year history of peritoneal dialysis [77]. gAChR antibodies were detected in the serum, and he was accordingly diagnosed with AAG.…”

Section: Uremic Neuropathymentioning

confidence: 99%

Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis

Nakane,

Koike,

Hayashi

et al. 2024

IJMS

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Autoimmune autonomic ganglionopathy (AAG) is a disease of autonomic failure caused by ganglionic acetylcholine receptor (gAChR) autoantibodies. Although the detection of autoantibodies is important for distinguishing the disease from other neuropathies that present with autonomic dysfunction, other factors are important for accurate diagnosis. Here, we provide a comprehensive review of the clinical features of AAG, highlighting differences in clinical course, clinical presentation, and laboratory findings from other neuropathies presenting with autonomic symptoms. The first step in diagnosing AAG is careful history taking, which should reveal whether the mode of onset is acute or chronic, followed by an examination of the time course of disease progression, including the presentation of autonomic and extra-autonomic symptoms. AAG is a neuropathy that should be differentiated from other neuropathies when the patient presents with autonomic dysfunction. Immune-mediated neuropathies, such as acute autonomic sensory neuropathy, are sometimes difficult to differentiate, and therefore, differences in clinical and laboratory findings should be well understood. Other non-neuropathic conditions, such as postural orthostatic tachycardia syndrome, chronic fatigue syndrome, and long COVID, also present with symptoms similar to those of AAG. Although often challenging, efforts should be made to differentiate among the disease candidates.

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Section: Uremic Neuropathymentioning

confidence: 99%

Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis

Nakane,

Koike,

Hayashi

et al. 2024

IJMS

Self Cite

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“…Studies on anti-N-methyl-D-aspartate receptor encephalitis potential relapses are limited and opposed, data on autoimmune autonomic ganglionopathy and myalgic encephalomyelitis/chronic fatigue syndrome are again limited, and so are data on autoimmune hypophysitis—with a case report showing high CRP possibly in relapse [ 749 , 750 , 751 , 752 , 753 ]. Moreover, CRP can be elevated in Guillain–Barré syndrome, in which it can possibly be a risk factor for disease severity, particularly in adults [ 754 , 755 ].…”

Section: Current Evidence On C-reactive Protein and Potential Conditionsmentioning

confidence: 99%

C-Reactive Protein: Pathophysiology, Diagnosis, False Test Results and a Novel Diagnostic Algorithm for Clinicians

Mouliou

2023

Diseases

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The current literature provides a body of evidence on C-Reactive Protein (CRP) and its potential role in inflammation. However, most pieces of evidence are sparse and controversial. This critical state-of-the-art monography provides all the crucial data on the potential biochemical properties of the protein, along with further evidence on its potential pathobiology, both for its pentameric and monomeric forms, including information for its ligands as well as the possible function of autoantibodies against the protein. Furthermore, the current evidence on its potential utility as a biomarker of various diseases is presented, of all cardiovascular, respiratory, hepatobiliary, gastrointestinal, pancreatic, renal, gynecological, andrological, dental, oral, otorhinolaryngological, ophthalmological, dermatological, musculoskeletal, neurological, mental, splenic, thyroid conditions, as well as infections, autoimmune-supposed conditions and neoplasms, including other possible factors that have been linked with elevated concentrations of that protein. Moreover, data on molecular diagnostics on CRP are discussed, and possible etiologies of false test results are highlighted. Additionally, this review evaluates all current pieces of evidence on CRP and systemic inflammation, and highlights future goals. Finally, a novel diagnostic algorithm to carefully assess the CRP level for a precise diagnosis of a medical condition is illustrated.

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

et al. 2021

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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.

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Autoimmune autonomic ganglionopathy manifesting as acute-onset orthostatic hypotension in a patient undergoing peritoneal dialysis

Cited by 3 publications

References 20 publications

Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis

Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis

C-Reactive Protein: Pathophysiology, Diagnosis, False Test Results and a Novel Diagnostic Algorithm for Clinicians

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

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