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Clonidine therapy for Shapiro's syndrome.

B R Walker¹, J A Anderson, C R Edwards

¹University of Edinburgh, Department of Medicine, Western General Hospital.

The Quarterly Journal of Medicine

|March 1, 1992

Summary

Shapiro's syndrome, a rare condition, involves corpus callosum agenesis, sweating, and hypothermia. Clonidine effectively treated a patient by normalizing central temperature regulation, suggesting a hypothalamic action.

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Area of Science:

Neuroscience
Endocrinology
Genetics

Background:

Shapiro's syndrome is a rare neurological disorder characterized by agenesis of the corpus callosum, episodic hyperhidrosis, and hypothermia.
The exact pathophysiology remains unclear, but it involves central thermoregulation defects.

Observation:

A 25-year-old male patient presented with symptoms consistent with Shapiro's syndrome.
Investigations revealed a central defect in temperature regulation with a lowered hypothalamic set-point.
No evidence of epilepsy, sympathetic dysfunction, or inappropriate vasopressin release was found.

Findings:

Therapy with clonidine, an alpha 2-adrenoceptor agonist, led to symptom remission.
Symptoms recurred upon clonidine withdrawal and resolved again upon reinitiation.
Clonidine treatment normalized central temperature regulation, suggesting a hypothalamic mechanism.

Implications:

Clonidine may be an effective treatment for Shapiro's syndrome by acting on hypothalamic thermoregulation.
These findings could inform therapeutic strategies for other temperature dysregulation disorders, such as perimenopausal flushing.

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