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Altered norepinephrine metabolism in Shapiro's syndrome.
J A Sanfield1, O A Linares, D D Cahalan
1Department of Internal Medicine, University of Michigan, Ann Arbor.
Archives of Neurology
|January 1, 1989
Summary
This study investigated Shapiro's syndrome, finding elevated norepinephrine levels due to increased release and decreased clearance. Clonidine therapy improved norepinephrine kinetics and reduced hypothermia episodes.
Area of Science:
- Neuroendocrinology
- Autonomic Nervous System Physiology
- Rare Disease Pathophysiology
Background:
- Spontaneous periodic hypothermia (Shapiro's syndrome) is a rare disorder characterized by recurrent episodes of hypothermia and associated autonomic dysfunction.
- Elevated plasma norepinephrine (NE) levels are frequently observed in patients with Shapiro's syndrome, but the underlying mechanisms remain unclear.
Observation:
- A 66-year-old female patient with Shapiro's syndrome was studied to elucidate the mechanisms of increased plasma NE.
- Compartmental analysis of NE kinetics revealed an increased NE release rate into the extravascular compartment.
- Decreased NE clearance and volume of distribution in the intravascular compartment were also observed in the patient compared to controls.
Findings:
- The study identified increased NE release and decreased NE clearance as the primary contributors to elevated plasma NE levels in Shapiro's syndrome.
- Clonidine therapy demonstrated a significant reduction in the frequency of diaphoretic episodes.
- Clonidine treatment was associated with a decrease in NE release rate and improvements in NE clearance and volume of distribution.
Implications:
- Understanding NE kinetics provides crucial insights into the pathophysiology of Shapiro's syndrome.
- Clonidine shows promise as an effective therapeutic agent for managing Shapiro's syndrome by modulating NE kinetics.
- Further research into autonomic dysfunction in rare hypothermic disorders is warranted.