Related Experiment Videos
Pure autonomic failure in association with human alpha-synucleinopathy
1Department of Neurology, Chiba University School of Medicine, 1-8-1 Inohana, Chuo-ku, 260-8670, Chiba, Japan. arai@med.m.chiba-u.ac.jp
Neuroscience Letters
|December 8, 2000
Summary
Pure autonomic failure is linked to alpha-synuclein protein deposits in the peripheral nervous system. This study identifies it as a Lewy body disease affecting autonomic nerves.
Area of Science:
- Neuropathology
- Autonomic Neuroscience
Background:
- Pure autonomic failure (PAF) is a neurodegenerative disorder affecting the autonomic nervous system.
- The role of alpha-synuclein in the pathogenesis of PAF has not been previously investigated using immunohistochemistry.
Observation:
- Autopsy examination revealed pre- and post-ganglionic lesions in both sympathetic and parasympathetic nervous systems in a case of PAF.
- Abundant Lewy bodies and Lewy neurites were observed, particularly within the sympathetic nervous system.
Findings:
- Immunohistochemical analysis using an anti-alpha-synuclein antibody demonstrated that these inclusions are immunoreactive to alpha-synuclein.
- Alpha-synuclein immunoreactivity was more intense in the halos than in the cores of Lewy bodies, and prominent at the edges of swollen neurites.
- The substantia nigra was preserved, and cortical Lewy bodies were absent.
Implications:
- These findings classify pure autonomic failure as a Lewy body type alpha-synucleinopathy.
- This expands the spectrum of alpha-synucleinopathies to include peripheral autonomic nervous system dysfunction, alongside conditions like Parkinson's disease and dementia with Lewy bodies.