(benign periodic hypothermia)

Extremely rare.

Association of

-        agenesis of the corpus callosum (this characteristics is missing in some cases)

-        paroxysmal bouts of sweating and hypothermia of variable duration and frequency, and the cause or triggering factors of which are unknown.

The cause of this disturbance of thermoregulation is probably a congenital or acquired dysfunction (ischemic injury ?) of the thermoregulatory hypothalamic dopaminergic centers.

in case of sweating, one can observe major hyponatremia.

The spontaneous evolution is often favourable within a few months. Sometimes, treatment with a serotonin antagonist (cyproheptadine) or dopamine agonist (levodopa) is effective. Some cases of effective treatment with clonidine have been reported.

Anesthetic implications:

tendency to per and postoperative hypothermia; mental retardation; neurological disorders

References : 

-         Belcastro V, Striano P, Pierguidi L, Arnaboldi M, Tambasco N. 
Recurrent hypothermia with hyperhidrosis in two siblings: familial Shapiro syndrome variant. 
J Neurol 2012; 259: 756-8.

-         Owen AJ, Backman SB. 
Perioperative temperature changes in a patient with Shapiro syndrome.
Anesthesiology 2001; 95: 268-70.

Updated: September 2018