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Autonomic dysfunction and adrenocortical unresponsiveness to ACTH
G Makari1, W H Hoffman, J E Carroll
1Department of Neurology, Medical College of Georgia, Augusta 30912-3760.
Journal of Child Neurology
|July 1, 1988
Summary
A child presented with adrenal insufficiency due to ACTH insensitivity and autonomic dysfunction. Progressive parasympathetic denervation is suggested as the cause of these combined symptoms.
Area of Science:
- Pediatric Endocrinology
- Autonomic Neurology
- Adrenal Disorders
Background:
- Adrenocortical insufficiency can arise from various causes, including ACTH resistance.
- Autonomic dysfunction presents with a range of symptoms affecting involuntary bodily functions.
Observation:
- A pediatric case exhibited unresponsiveness to ACTH, indicating adrenal insufficiency.
- The patient displayed symptoms of autonomic dysfunction: cold extremities, reduced lacrimation, esophageal achalasia, pupillary abnormalities, and an abnormal histamine response.
Findings:
- The constellation of symptoms points towards a progressive denervation affecting the parasympathetic nervous system.
- This parasympathetic denervation is hypothesized to underlie the observed adrenocortical unresponsiveness.
Implications:
- This case highlights a potential link between autonomic nervous system dysfunction and adrenal hormone regulation in children.
- Understanding this connection may offer new diagnostic and therapeutic avenues for pediatric endocrine and neurological disorders.