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Similar Publications

[Callosogenital dysplasia].

Presse Med

March 1989

Hôpitaux de Toulon.

A nosological entity, calloso-genital dysplasia, is described from a case of primary amenorrhoea with coloboma and total agenesis of the corpus callosum. Deficiency of the thalamic gonadotropic hormone secretion was elicited, together with normal or moderately elevated prolactinaemia, the significance of which is discussed. Thyrotropic, somatotropic and corticotropic functions were normal.

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MR tomography is the only imaging method that can demonstrate atrophy of the olfactory lobe of the brain in olfacto-genital dysplasia (Kallmann's syndrome). The MRT findings in five patients with Kallmann's syndrome are described. The MRT criterion for the presence of Kallmann's syndrome appears to be an interruption or total absence of the olfactory sulcus.

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The clinical syndrome of OGD in the female is displayed as hypogonadism and most commonly type I hyposmia ("anosmia"). The main pathologic findings are absence of the olfactory bulbs and tracts, hypoplasia of the hypothalamus, a normal pituitary gland, and normal appearing, although unstimulated, ovaries. The syndrome of OGD may be considered as an attenuated form of holoprosencephaly.

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