Hypogonadotropic hypogonadism in patients with multiple congenital defects.

Several syndromes have been reviewed in which hypogonadotropism is associated with multiple somatic and neurologic anomalies. A review of the literature indicates that these conditions demonstrate considerable clinical heterogeneity. In the several hypogonadotropic syndromes described to date autosomal and X-linked transmission have been implicated in the inheritance of the hypogonadism. The associated neurologic and somatic anomalies may segregate independent of the hypogonadism and in some instances may have a separate mode of transmission.