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Agenesis of the corpus callosum associated with relapsing hypothermia: a clinico pathological report

par Noel, Pierre Raoul ;Hubert, J.P.;Ectors, Michel Paul
Référence Brain, 96, 2, page (359-368)
Publication Publié, 1973

Article révisé par les pairs

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Résumé :

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.