Extremely rare. Association of agenesis of the corpus callosum with thermoregulation disorders presenting as in form of febrile crises the cause of which  cannot be determined. The cause is probably a malfunction of the thermoregulatory dopaminergic centers at the level of the hypothalamus.

Sometimes, treatment with dopaminergic agonists (levodopa) or serotoninergic antagonists (cyproheptadine) is effective.

Anesthetic implications:

tendency to per- and postoperative hyperthermia; mental retardation; neurologic problems

References : 

-        Lin K-L, Wang H-S. 
Reverse Shapiro’s syndrome : an unusual cause of fever of unknown origin. 
Brain Dev 2005; 27: 455-7.

-         Guha S, Guha G, Hashmi MA. 
A case of periodic fever with corpus callosum agenesis : reverse Shapiro’s syndrome.
J Pediatr Neurosc 2012; 7: 150-4.

Updated: October 2018