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Abnormal vascular reflexes in Charcot-Marie-Tooth disease
Journal of Neurology, Neurosurgery, and Psychiatry
|April 1, 1980
Summary
Autonomic neuropathy, affecting the nervous system
Area of Science:
- Neurology
- Autonomic Neuroscience
Background:
- Charcot-Marie-Tooth disease (CMT) is a progressive, inherited peripheral neuropathy.
- Autonomic dysfunction can occur in CMT, impacting involuntary bodily functions.
Purpose of the Study:
- To investigate the presence and extent of vascular reflex abnormalities in patients with Charcot-Marie-Tooth disease.
- To explore the association between autonomic neuropathy and CMT.
Main Methods:
- Vascular reflexes were assessed in 17 adult CMT patients.
- Tests included the Valsalva manoeuvre and responses to standing (pulse rate, systolic blood pressure).
- Histological examination of bowel tissue was performed in one patient.
Main Results:
- Six out of 17 patients (35%) exhibited abnormal vascular reflexes, suggestive of autonomic neuropathy.
- Histological findings revealed giant nerve fibre bundles in the myenteric plexus, indicating autonomic neuronal involvement.
- Review of existing literature supports an autonomic component in CMT.
Conclusions:
- A significant proportion of Charcot-Marie-Tooth disease patients may experience autonomic neuropathy.
- Autonomic dysfunction should be considered in the comprehensive assessment and management of CMT.
- Further research is warranted to elucidate the mechanisms and clinical implications of autonomic involvement in CMT.