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Published on: November 9, 2017
[Autoimmune Autonomic Ganglionopathy and Acute Autonomic Sensory Neuropathy]
1Department of Neurology, Faculty of Medicine, Academic Research Division, University of Toyama.
Autoimmune autonomic ganglionopathy (AAG) and acute autonomic sensory neuropathy (AASN) are distinct immune-mediated neuropathies. Despite differing autoantibodies, shared brain and endocrine symptoms suggest a potential common underlying pathophysiology.
Area of Science:
- Neurology
- Immunology
- Pathophysiology
Background:
- Autoimmune autonomic ganglionopathy (AAG) and acute autonomic sensory neuropathy (AASN) are immune-mediated neuropathies affecting nerve ganglia.
- AAG pathogenesis involves autoantibodies against the nicotinic ganglionic acetylcholine receptor (gAChR), which are absent in AASN.
- Clinical and laboratory findings currently separate AAG and AASN into distinct disease concepts.
Purpose of the Study:
- To explore the potential shared pathophysiology between AAG and AASN.
- To investigate the significance of extra-autonomic brain and endocrine symptoms in both conditions.
Main Methods:
- Review of clinical symptoms and laboratory findings in AAG and AASN patients.
- Analysis of autoantibody profiles, specifically gAChR antibodies.
- Comparative assessment of neurological, psychiatric, and endocrine manifestations.
Main Results:
- Autoantibodies against gAChR are characteristic of AAG but not AASN.
- Both AAG and AASN present with extra-autonomic brain symptoms, including psychiatric and personality changes.
- Endocrine disorders are observed in patients with both AAG and AASN.
Conclusions:
- Despite distinct immunological markers, the occurrence of similar extra-autonomic brain and endocrine symptoms suggests a shared pathophysiological basis between AAG and AASN.
- Further research is warranted to elucidate the common mechanisms underlying these immune-mediated neuropathies.
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