No any testes tissue and no any testosterone response to hCG stimulation test were observed. These findings suggest that hypogonadism in the presented patient with XLAG syndrome is primary hypogonadism due to gonadal agenesis or dysgenesis. The basis of the ambiguous genitalia in XLAG syndrome i s not well known.įourth child born to healthy c onsanguineous parents was acce pted to hospital be cause of non-febrile seizures at two m onths of life.
On physical examination, microcephaly, some dysmorphic face features and ambi guous genitalia were determined.Ĭ ranial MRI of the patient showed lissencephaly, agenes is of the corpus callosum and enlarged ventricles.
