[Cerebral gigantism. Review of the literature apropos of one case].
The authors report a case of cerebral gigantism and review 83 cases found in the world literature. The diagnosis depends on the association of several factors, none of which alone are specific but which, on the whole, form a fairly characteristic picture. Large size at birth, leading only rarely to gigantism at adult age. Facial dysmorphia of acromegaloid type. Ecephalopathy with mental deficiency without any neuroradiological abnormality. Finally, endocrine investigations and somatotropic function were normal. No etiology has been found up to now. The most satisfactory theory is that of a hypothalamopituitary disturbance. Treatment may simple avoid excess height at adult age and is based on sex hormone therapy to accelerate bony maturation.
D000172:Acromegaly / Q000209:etiology; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000365:Age Determination by Skeleton; D001927:Brain Diseases / Q000209:etiology; D003394:Craniofacial Dysostosis / Q000209:etiology; D003878:Dermatoglyphics; D004967:Estrogens / Q000627:therapeutic use; D005877:Gigantism / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D007621:Karyotyping; D008297:Male; D010901:Pituitary Function Tests; D012737:Sex Factors
D000305:Adrenal Cortex Hormones; D004967:Estrogens
Gigantisme cérébral. Revue de la littérature à propos d'un cas