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The examination of the central nervous system (CNS) of an 11-month-old citrullinaemic patient revealed an ulegyria with a dense fibrillary gliosis and large amounts of lipid phagocytes. Alzheimer type 2 glial cells were present in intact cortical areas. While such cells were related to the insufficiently controlled hyperammonaemia, the ulegyria resulted from impaired oxygenation or impaired blood circulation, possibly influenced by the hyperammonaemia and citrullinaemia.

A 41 year old man had recurrent hypothermia with loss of consciousness which eventually became permanent. There was agenesis of the corpus callosum, with no evidence of dysfunction of the hypothalamo pituitary axis. Autopsy confirmed the agenesis, and showed intense fibrillary gliosis restricted to the arcuate nucleus and premammillary area, which may account for the thermoregulatory disturbance. The association of these hypothalamic lesions with congenital absence of the corpus callosum would seem a mere coincidence were it not for the striking clinical similarities between this case and others previously published. It is suggested that agenesis of the corpus callosum should be looked for in cases of unexplained spontaneous hypothermia.

Summary Th is report presents findings from an unusual case of Pick's disease that was characterized by existence of choreo-athetoid movements and frequent epileptic seizures in the clinical course, and, neuropathologi-cally, by the absence of “wedge-shaped” localized atrophy and fibrillary gliosis in the affected region in spite of thirteen years' clinical course. These findings are compared to those of other case of unclassifiable presenile dementia.

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 patients 1,2 and confirmed at autopsy in a fourth 3 —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