REVERSE SHAPIROS SYNDROME - A CASE OF AGENESIS OF CORPUS-CALLOSUM ASSOCIATED WITH PERIODIC HYPERTHERMIA

Objective: Investigation of the mechanism of the idiopathic periodic h yperthermia associated with agenesis of the corpus. callosum. Setting: Tertiary care referral center. Patient: Fourteen-year-old girl who wa s the first case of reverse Shapiro's syndrome (ie, agenesis of the co rpus callosum associated with periodic hyperthermia, as opposed to hyp othermia as described in the original report by Shapiro et al). Interv ention: Magnetic resonance imaging scans, endocrinological examination s for hypothalamus, electroencephalograms, and levodopa therapy. Main Outcome Measure: Body temperature. Result: No neurologic or physical a bnormalities were noted beside the callosal agenesis. Neuroimaging exa minations could not reveal any structural abnormality of the diencepha lon. Endocrinological examinations and electroencephalograms were foun d to be normal. The hyperthermia returned to normal by a small dose of levodopa, but a larger dose reversed the hyperthermia to hypothermia. Conclusions: These observations suggest that the hyperthermia observe d in this case may have been caused by the dopaminergic denervation of the hypothalamic thermoregulatory center, resulting in the supersensi tivity of its dopaminergic receptors.