[Two cases of de Morsier's olfactogenital syndrome].
Two cases of De Morsier's olfactogenital syndrome (complex hypogenitalism and anosmia) are presented. The first patient was of eunuchoid build and presented testicle and penile hypotrophy, depressed adrenal activity and gonad-stimulating pituitary function and distinctly below-normal urinary 17-ketosteroid values. The second subject was gynaecoid, menopausal gonadotrophic hormonal values, short urethra with no radiological signs of the prostate, and gynaecoid upper pelvic basin diameters. The karyotype pattern was normal XY in the first case and XX in the second. It is suggested that embryopathic or genetic changes, probably in the rhinencephalon, led to a direct pituitary nervous short-circuit that impeded normal FSH gonadal stimulation and hence gonadal influence on the phenotype throughout both intra- and extrauterine life, particularly at puberty.
Ghislanzoni
G
G
Clinica Dermatologica dell'Università di Milano.
Bellani
G
G
Lenzi
P
P
D015068:17-Ketosteroids / Q000652:urine; D000368:Aged; D041321:Chromosomes, Human, X; D041322:Chromosomes, Human, Y; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D006728:Hormones / Q000378:metabolism; D006801:Humans; D007006:Hypogonadism / Q000196:embryology / Q000209:etiology* / Q000235:genetics / Q000378:metabolism; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D000857:Olfaction Disorders / Q000196:embryology / Q000209:etiology* / Q000235:genetics / Q000378:metabolism; D009833:Olfactory Pathways / Q000002:abnormalities* / Q000196:embryology; D011467:Prostate / Q000002:abnormalities; D012727:Sex Characteristics; D013577:Syndrome; D013737:Testis / Q000473:pathology*
D015068:17-Ketosteroids; D006728:Hormones; D005640:Follicle Stimulating Hormone