THE ASSOCIATION OF CONGENITAL SPASTIC QUADRIPLEGIA AND ANDROGENIC PRECOCITY IN FOUR PATIENTS

Abstract
LESIONS in and around the posterior portion of the hypothalamus have been reported in association with precocious puberty. Pelizzi (1) described a syndrome which he called “macrogenitosomia precoce.” The findings included tumors of the pineal gland, precocious sexual development and adiposity or general overgrowth, together with symptoms of hydrocephalus. Haldeman (2) in 1927 listed 113 cases of pineal tumor, of which 16 showed macrogenitosomia precox, all males between 3 and 16 years of age. Horrax and Bailey (3) and Henyer et al. (4) reported similar cases and concluded that the cause of the precocious puberty in this syndrome was unknown and that pineal involvement was not necessary. Vickers and Tidswell (5) described precocious puberty in association with a hypothalamic tumor. Horrax and Bailey (6) give the typical neurologic localizing signs of pineal tumors as: i) involvement of the corpora quadrigemina, i.e., oculomotor palsies, partial or complete deafness; ii) spasticity, usually bilateral; and iii) evidence of implication of the cerebellum or cerebellar tracts.

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