Related Experiment Videos
Pure progressive autonomic failure presenting severe orthostatic hypotension
1First Department of Internal Medicine, Oita Medical University, Japan.
Internal Medicine (Tokyo, Japan)
|February 1, 1993
Summary
This study describes a rare case of pure autonomic failure (PAF) in a woman with chronic progressive autonomic dysfunction. Findings suggest PAF is a distinct syndrome of autonomic nervous system degeneration.
Area of Science:
- Neurology
- Autonomic Neuroscience
Background:
- Pure autonomic failure (PAF) is a rare condition characterized by progressive autonomic nervous system dysfunction.
- Distinguishing PAF from other autonomic neuropathies is crucial for accurate diagnosis and management.
Observation:
- A 59-year-old woman presented with chronic progressive autonomic failure, including orthostatic hypotension and dyshidrosis.
- Neurological examination revealed no signs of Parkinsonism, cerebellar, or peripheral neuropathy.
- Autonomic function tests and sural nerve biopsy indicated significant postganglionic sympathetic and parasympathetic dysfunction.
Findings:
- The patient's orthostatic hypotension was attributed to decreased total peripheral resistance without a compensatory heart rate increase upon standing.
- The observed dysfunctions were primarily postganglionic, affecting both sympathetic and parasympathetic systems.
Implications:
- This case supports the recognition of pure autonomic failure as a distinct clinical syndrome.
- Systematic degeneration of the autonomic nervous system underlies pure autonomic failure.
- Understanding the specific mechanisms of orthostatic hypotension in PAF is vital for patient care.