Infundibulo-tuberal syndrome: the origins of clinical neuroendocrinology in France.

Abstract

The birth of clinical neuroendocrinology can be dated to the year 1900, when the French neurologist Joseph Babinski (1857-1932) described a particular syndrome of adiposity and sexual infantilism in an adolescent with a craniopharyngioma expanding at the base of the brain. This condition of adipose-genital dystrophy, also known as Babinski-Fröhlich syndrome, represented the first clinical evidence that the brain controlled endocrine functions. Adipose-genital dystrophy forms part of infundibulo-tuberal syndrome, which groups the endocrine, metabolic and behavioral disturbances caused by lesions involving the upper neurohypophysis (median eminence) and the adjacent basal hypothalamus (tuber cinereum). This syndrome was originally described by the French neuropsychiatrists Henri Claude (1869-1946) and Jean Lhermitte (1877-1959) in 1917, also in a patient with a craniopharyngioma. This type of tumor involves specifically the infundibulo-tuberal region of the hypothalamus, providing a clinical model to conceptualize the separation of hypophyseal and hypothalamic functions. The French School of Neurology analyzed and reported the symptoms associated with dysfunction of the basal hypothalamus by craniopharyngiomas and other types of tumors, influencing significantly the development of clinical neuroendocrinology. Experimental lesions performed in the tuber cinereum by the French physiologists Jean Camus (1872-1924) and Gustave Roussy (1874-1948) demonstrated unmistakably the anatomical origin of infundibulo-tuberal syndrome in the basal hypothalamus. This article reviews the original findings on infundibulo-tuberal syndrome reported by the French School of Neurology in the first decades of the twentieth century and the great influence this school had on modern conceptions of hypothalamic control over endocrine functions and behavior.