A unique episodic hypothermia, though apparently extremely rare, is currently attracting considerable attention because of its distinctive clinical and pathological features. Also known as recurrent, relapsing, spontaneous, and periodic, this hypothermia usually begins in childhood or adolescence. Characteristically, a sudden bout of profuse sweating and vasodilatation ushers in a precipitous drop of temperature that may reach a nadir of 30 C or less. The episode, which can last anywhere between one half to two hours, may recur many times in a day, with or without prolonged remissions of weeks', months', or years' duration. A curious absence of the corpus callosum was demonstrated roentgenologically in three patients1,2 and confirmed at autopsy in a fourth3—the latter also manifesting heavy fibrillary gliosis with neuronal loss in the premammillary region and in the arcuate nuclei of the hypothalamus.
The exact mechanism of this curious hypothermia is poorly understood. It is tempting