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Autonomic cardiovascular reflexes in Wilson's disease
Kirsty Bhattacharya1, Miodrag Velickovic, Michael Schilsky
1Department of Neurology, Mount Sinai School of Medicine, New York, NY 10029, USA.
Summary
Autonomic nervous system impairment in Wilson's disease is linked to central nervous system damage. Cardiovascular reflex abnormalities suggest central autonomic neuron involvement in this rare genetic disorder.
Area of Science:
- Neurology
- Cardiology
- Autonomic Neuroscience
Background:
- Wilson's disease is a rare genetic disorder causing copper accumulation.
- Autonomic dysfunction can occur in Wilson's disease, but its neurological basis is unclear.
Purpose of the Study:
- To investigate autonomic cardiovascular function in patients with Wilson's disease.
- To correlate autonomic testing results with central nervous system (CNS) involvement.
Main Methods:
- Evaluated autonomic cardiovascular reflexes in 14 Wilson's disease patients.
- Assessed the severity of central nervous system abnormalities in participants.
Main Results:
- Four patients exhibited abnormal autonomic testing results.
- Three of these four patients also had severe CNS abnormalities.
- Ten patients had normal cardiovascular reflexes, with only two showing severe CNS deficits.
Conclusions:
- Autonomic impairment in Wilson's disease is associated with central nervous system involvement.
- Findings suggest that damage to central autonomic neurons contributes to autonomic dysfunction in Wilson's disease.