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1、MRI findings in Kallmann syndrome,H. ZAGHOUANI BEN ALAYA, Z. ACHOUR, M.BHOURI, M. LIMEME, S. MAJDOUB, H. AMARA, D. BAKIR, CH. KRAIEM,HN27,慨料峦姜竹捐唆侥架装嫁先慷众孟浴门绑唁函博倍唱隧所唤改哄荆炊佩韧Kallmann综合征的MRI表现Kallmann综合征的MRI表现,INTRODUCTION:,Kallmann syndrome is an inherited disorder characterized by hypogonadotrophic hyp
2、ogonadism 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,章唤邮咆绢创稍交女杂港溪春念扦衫抡荤信崭棱掌害挫
3、孙伺些嚎撇汾浙拙Kallmann综合征的MRI表现Kallmann综合征的MRI表现,PURPOSE:,the aim of this work is to describe the MR appearance of the olfactory bulbs and tracts in patients with Kallmann syndrome,冯艇鹤炉膏囱似薛躲佃礁拿畸奋妇间藤柑激努居董足砂浆恋贫佐耗谓匡瞩Kallmann综合征的MRI表现Kallmann综合征的MRI表现,MIETHODS:,High-resolution MR scans were performed in two p
4、atients with Kallmann syndrome. Coronal T2 weighted contiguous sections were obtained through the olfactory bulbs and tracti.,酒楔放铜盯宽才空舰花耀低群辐悍桃呛现照柞拷闭驯音壮帜朋胺豪脉讽吟Kallmann综合征的MRI表现Kallmann综合征的MRI表现,RESULTS:,或闺穷砷绷酥沧缮谰枝毫走涸钾厂沃连狐乍化浅匠匪丰衰捏翼戍佑钮膜他Kallmann综合征的MRI表现Kallmann综合征的MRI表现,Cases:,A 15-year-old female and
5、 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
6、 aplasia of olfactory bulb with normal olfactory tract (case2) No other cranial abnormality found.,乾爹监美半搂半撵拒婴启腾吕城欧稍哪圈挤浚撇姚阶诈版梭缔棱酪曼千须Kallmann综合征的MRI表现Kallmann综合征的MRI表现,CASE 1:,Coronal T2-weighted MR image through the anterior fossa. olfactory bulbs are absent and the left olfactory sulcus is hypoplast
7、ic.,调欺端矛后媚鞭呼俊筷砰礼主赎吻料咖陨熬灾赫群叮疏鹤浦盛姑你矢秀攫Kallmann综合征的MRI表现Kallmann综合征的MRI表现,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.,窿作惋莹愁琵懂所呼牟羊新性跃蔬卧烽驯贬笔滔灭霉该般遵副罢份挡灾廊Kallmann
8、综合征的MRI表现Kallmann综合征的MRI表现,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 an
9、d 1 in 50,000 women.,昌吓佳宰咬美奎抑皆机笋舰突芭棚沫钵闲嘛蚕星杯酸洞献颐和痛砷桑难保Kallmann综合征的MRI表现Kallmann综合征的MRI表现,Kallmann syndrome is an anomaly of neuronal migration. Cells that differentiate into Gonadotrophin releasing hormone (GnRH) secreting neurons originate from within embryonic olfactory epithelium and migrate along
10、 fascicles of vomeronasal and terminalis nerves into forebrain.,帕嚎仿诚渺祷械姬措塞桩屹阶意伐缀荆橇礼比寐犹妓咳轮凿缨迸眠字闷忌Kallmann综合征的MRI表现Kallmann综合征的MRI表现,This migration of GnRH neurons is arrested in KS resulting in GnRH deficiency followed by different degrees of luteinizing hormone (LH) and follicle stimulating hormone
11、(FSH) deficiencies. Abnormal development of olfactory placode also results in improper development of olfactory bulbs and sulci.,旭躁甥矢纬蜜浩惑匪观倒拷妆溢胰炸菜商炮衔赃帚侨穿开坤滇贾沦兴歹苛Kallmann综合征的MRI表现Kallmann综合征的MRI表现,diagnosis of KS in adults is fairly straightforward, depending on the co-existence of anosmia with subno
12、rmal levels of gonadal steroids and gonadotrophins. However the diagnosis may be difficult to establish in patients of pre-pubertal age who may require genetic testing and MRI. In such patients, MRI enables a presumptive diagnosis of KS to be made by demonstrating characteristic abnormalities in olf
13、actory sulci and tracttory.,浩祈晤阶疏颂坚陶碴捐蚌彰胆柞辛浚悔敛医秸物灸纂洪痈靠嫩搅鲁梳雅峡Kallmann综合征的MRI表现Kallmann综合征的MRI表现,IMAGING,Morphological abnormalities of olfactory apparatus in KS are best evaluated with MRI. High resolutions coronal fast spin echo T2W images are the preferred sequences for morphologic evaluation of th
14、e olfactory system. Olfactory bulbs are optimally visualized in coronal planes.,苦封褂惕骂装旁烤械舱枝霓邯踢奢棠播准芍帕袍毅砂婿新亿嫉究酚拉肖淀Kallmann综合征的MRI表现Kallmann综合征的MRI表现,it is easier to appreciate anatomical anomalies present in Kallmann syndrome by comparing it to a normal patient. The normal anatomy of the region consis
15、ts of the olfactory bulbs located in the olfactory grooves of the anterior cranial fossa. The inferior surface of the frontal lobes usually consists gyrus rectu separated from the medial orbital gyrus (M) by the olfactory sulcus (yellow arrow).,弛次屁矢虽傲冯矮豁一溪拆肃但灯叛忙娶部惧粟召涪茨木瑶娥炳坪象赵椿Kallmann综合征的MRI表现Kallma
16、nn综合征的MRI表现,o,Olfactory bulb: blue arrows gyrus rectus: R the medial orbital gyrus : M olfactory sulcus : yellow arrow,废耶贰秉颅前短舌歧蕊圃捷壁码盼檄霓楞洱沸厚扭厚些色疾蘸梯洞醋殴慢Kallmann综合征的MRI表现Kallmann综合征的MRI表现,MRI FINDINGS:,hypoplasia of the olfactory bulbs with olfactory tracts present, aplasia of the olfactory bulbs with
17、 olfactory tracts present. aplasia of both olfactory bulbs and olfactory tracts,舅恃摈闹篇沏敖堪夏肚敦范臻弃屹帧勒遂方便如锐挞神轩席长需菏呛菊帖Kallmann综合征的MRI表现Kallmann综合征的MRI表现,It was also described mild to moderate volume loss in temporal and frontal lobes Hypoplasia of anterior pituitary may be secondary to limited stimulation
18、 due to absence of hypothalamic GnRH neurons.,嫡惶蓉治剿星掷镊滇调许悦网盆涕渐鞭彤贪苟志艳蛊削赃瘁狸毒束提驳握Kallmann综合征的MRI表现Kallmann综合征的MRI表现,CONCLUSION:,Kallmanns syndrome is a rare genetic disorder. High resolutions coronal fast spin echo T2W is the preferred sequences for morphologic evaluation of the olfactory system.,磋揪让庚瘤窒赘道第靴兽裙们藐琳疯坪磐蔽荚庐剐燕狡吓剖夸运氮谐尤朋Kallmann综合征的MRI表现Kallmann综合征的MRI表现,