Hypergonadotropic Hypogonadism in Oligophrenia

Abstract

Eight patients representing 5 different, probably hereditary neurological syndromes with oligophrenia and hypogonadism as the common features were examined clinically and endocrinologically. Two sisters suffered from polyneuropathy, 1 male from ataxia, 1 male from spastic tetraplegia, 2 sisters and a brother from myopathy and 1 male patient from epilepsy and polyneuropathy. The latter patient was diagnosed as having an acute intermittent porphyria. All the patients had degenerative neurological disorders. The karyotypes were normal. The patients all had signs of hypogonadism. Four male patients had marked testicular atrophy but otherwise normal external genitalia. The testosterone levels in the blood were normal or slightly decreased. Three of the females had their menarche at a normal age but a very early menopause; the 4th female never menstruated. The 4 females had normal breasts and body hair. All patients had high basal luteinizing hormone (LH) and follicle-stimulating hormone levels and the response to i.v. LH-releasing hormone was exaggerated. The prolactin values were normal. None of the examined patients had any signs of thyroid or adrenal insufficiency and the sella turcica was normal. A possible etiology to their hypergonadotropic hypogonadism is discussed.