Kallmann合征的MRI表现.pptVIP

MRI findings in Kallmann syndrome H. ZAGHOUANI BEN ALAYA, Z. ACHOUR, M.BHOURI,? M. LIMEME, S. MAJDOUB, H. AMARA, D. BAKIR, CH. KRAIEM HN27 INTRODUCTION: Kallmann syndrome is an inherited disorder characterized by hypogonadotrophic hypogonadism and anosmia or hyposmia. KS is due to abnormal migration of gonado-tropin-releasing hormone (GnRH) as well as olfactory neurons from olfactory placode to the forebrain and hypothalamus during fetal life. Structural olfactory tract abnormalities are well seen on MRI PURPOSE: the aim of this work is to describe the MR appearance of the olfactory bulbs and tracts in patients with Kallmann syndrome, MIETHODS: High-resolution MR scans were performed in two patients with Kallmann syndrome. Coronal T2 weighted contiguous sections were obtained through the olfactory bulbs and tracti. RESULTS: Cases: A 15-year-old female and 20-year-old male adressed to MRI for hypogonadotrophic hypogonadism ((Serum LH 1.5 IU/L, Serum FSH 1.5 IU/L, Testosterone 20 ng/dl) and hyposmia. Coronal T2-weighted MR images through the anterior fossa shows: aplasia of both olfactory bulb and absence of the left olfactory sulcus (case1). Bilateral aplasia of olfactory bulb with normal olfactory tract (case2) No other cranial abnormality found. CASE 1: Coronal T2-weighted MR image through the anterior fossa. olfactory bulbs are absent and the left olfactory sulcus is hypoplastic. CASE 2: coronal T2 images through the frontal lobes demonstrate abnormal anatomy with absence of the olfactory bulbs. Olfactory sulcus,the gyrus rectus and medial orbital gyrus are normal.? DISCUSSION: Kallmann syndrome is an inherited disorder characterized by hypogonadotrophic hypogonadism and anosmia or hyposmia. The inheritance may be X-linked, Autosomal recessive or Autosomal dominant with variable penetrance. The reported incidence is 1 in 10,000 men and 1 in 50,000 women. Kallmann syndrome is an anomaly of neuronal migration. Cells that diffe