Elsevier

Molecular Brain Research

Volume 6, Issue 4, December 1989, Pages 311-326
Molecular Brain Research

Research report
Luteinizing hormone-releasing hormone (LHRH)-expressing cells do not migrate normally in an inherited hypogonadal (Kallmann) syndrome

https://doi.org/10.1016/0169-328X(89)90076-4Get rights and content

Abstract

Kallmann syndrome inherited hypogonadotropic hypogonadism with anosmia, is associated with an X-chromosome deletion at Xp 22.3. In a Kallmann fetus, we have found an absence of luteinizing hormone-releasing hormone (LHRH)-expressing cells in the brain despite dense clusters of LHRH cells and fibers in the nose. LHRH-containing cells and neurites end in a tangle beneath the forebrain, within the dural layers of the meninges, on the dorsal surface of the cribriform plate of the ethmoid bone. Normal fetal brains, matched for age and sex, had LHRH cells and fibers, as expected, in the hypothalamus and preoptic area. Since LHRH-expressing cells recently were discovered to migrate from the olfactory placode into the brain, it appears that the hypogonadotropism of the Kallmann syndrome can be accounted for by a failure of LHRH cells to migrate into the brain.

References (38)

  • K. Kovacs et al.

    Pituitary changes in Kallmann's syndrome: a histologic, immunocytologic, ultrastructural, and immunoelectron microscopic study

    Fertil. Steril.

    (1982)
  • A. Mroueh et al.

    Olfacto-genital dysplasia

    Am. J. Obst. and Gynecol.

    (1968)
  • M.R. Soules et al.

    Female Kallmann's syndrome: evidence for a hypothalamic luteinizing hormone-releasing hormone deficiency

    Fertil. Steril.

    (1980)
  • A. Ballabio et al.

    X-linked ichthyosis, due to steroid sulphatase deficiency, associated with Kallmann syndrome (hypogonadotropic hypogonadism and anosmia): linkage relationships with Xg and cloned DNA sequences from the distal short arm of the X chromosome

    Hum. Genet.

    (1986)
  • P.E. Belchetz et al.

    Hypophysial responses to continuous and intermittant delivery of hypothalamic gonadotropin releasing hormone

    Science

    (1978)
  • Bick, D., Curry, C.J.R., McGill, J.R., Schorderet, D.F., Bux, R.C. and Moore, C.M., A male infant with ichthyosis,...
  • Bick, D., Snead, M., Yen, P.H., McGill, J., Schorderet, D., Hejtmancik, J.F., Ballabio, A., Campbell, L., Moore, C.,...
  • C. Brookover

    The peripheral distribution of the nervus terminalis in an infant

    J. Comp. Neurol.

    (1917)
  • C. Bugnon et al.

    Cyto-immunological study of the ontogenesis of the hypothalamo-pituitary axis in the human fetus

    J. Steroid Biochem.

    (1976)
  • K.L. Crossin et al.

    Site-restricted expression of cytotactin during development of the chick embryo

    J. Cell Biol.

    (1986)
  • W.F. Crowley et al.

    Simulation of the normal menstrual cycle in Kallmann's syndrome by pulsatile administration of luteinizing hormone-releasing hormone (LHRH)

    J. Clin. Endocrinol and Met.

    (1980)
  • G. De Morsier

    Etudes sur les dysraphies cranio-encephaliques. I. Agenesie des lobes olfactifs (telencepephaloschizis lateral) et des commissures calleuse et anterieure (telencephaloschizis median) La dysplasie olfacto-genitale

    Schweiz. Arch. Neurol. Psychiat.

    (1954)
  • G. De Morsier

    Median cranioencephalic dysraphias and olfactogenital dysplasia

    World Neurol.

    (1962)
  • P.M. Dubois et al.

    Mise en evidence par immunofluorescence de l'activité gonadotrope LH dans l'antehypophyse foetale humaine

  • G.M. Edelman

    Cell adhesion molecules

    Science

    (1988)
  • R. Guillemin

    Peptides in the brain: the new endocrinology of the neuron

    Science

    (1978)
  • M. Hermanussen et al.

    Heterogeneity of Kallmann's syndrome

    Clin. Genet.

    (1985)
  • F.J. Kallmann et al.

    The genetic aspects of primary eunuchoidism

    Am. J. Ment. Defic.

    (1944)
  • D. Klingmüller et al.

    Induction of puberty in a patient with hypogonadrotropic hypogonadism: effect of a sequentially applied hCG and pulsatile GnRH administration

    Horm. Metab. Res.

    (1980)

    • Cited by (0)

    View full text
    Copyright © 1989 Published by Elsevier B.V.