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1、关于颅脑先天畸形第一页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:08.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0505.htmTitle:PowerPoint Presentation Size:6165 Last Modified:Thu,15 Jun 2006 02:07:48 GMT学习要点本节应了解、熟悉和掌握的知识点了解颅脑先天发育异常的分类 熟悉常见颅脑发育异常的影像学表现 神经皮肤综
2、合征的影像学表现第二页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:08.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0506.htmTitle:PowerPoint Presentation Size:5554 Last Modified:Thu,15 Jun 2006 02:07:48 GMT学习难点本节学习中的难点脑先天性发育异常的分类和影像学表现 第三页，讲稿共四十三页哦WebZIP 7.0.3.
3、1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0519.htmTitle:PowerPoint Presentation Size:3628 Last Modified:Thu,15 Jun 2006 02:07:58 GMT颅脑先天性畸形分类第四页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09
4、.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0508.htmTitle:PowerPoint Presentation Size:2851 Last Modified:Thu,15 Jun 2006 02:07:48 GMT一、头颅先天性畸形第五页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2
5、-9.files/slide0509.htmTitle:PowerPoint Presentation Size:4811 Last Modified:Thu,15 Jun 2006 02:07:48 GMT狭颅症（craniostenosis）颅缝提早骨化、闭合，致头颅畸形，包括舟状 头畸形、短头畸形、尖头畸形、偏头畸形、小 头畸形等第六页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc
6、/2-9.files/slide0510.htmTitle:PowerPoint Presentation Size:5317 Last Modified:Thu,15 Jun 2006 02:07:50 GMT矢状缝及顶颞缝提早闭合，头颅左右径生长受限，前后径生长显著 舟状头变形舟状头变形第七页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0514.ht
7、mTitle:PowerPoint Presentation Size:5582 Last Modified:Thu,15 Jun 2006 02:07:56 GMT颅底陷入颅底陷入枕骨大孔周围骨质，枕骨基底部、髁部、鳞部向颅腔内陷入扁平颅底前、中、后颅窝失去正常的阶梯状关系第八页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0515.htmTitle:P
8、owerPoint Presentation Size:9979 Last Modified:Thu,15 Jun 2006 02:07:56 GMT测量标准Chamberlain：硬腭后缘与枕骨大孔后缘连线，齿状突高于此线3mm为异常McGregor：硬腭后缘与枕骨最低点连线，齿状突高于此线6mm为异常第九页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide
9、0518.htmTitle:PowerPoint Presentation Size:2855 Last Modified:Thu,15 Jun 2006 02:07:58 GMT二、脑先天性发育异常第十页，讲稿共四十三页哦先天性脑积水先天性脑积水：又称积水性无脑畸形病因：病因不明，可能与胚胎期颈内动脉发育 不良、母体感染、放射损伤有关临床与病理：头颅迅速增大、面部比例失调，智力低下，多在一岁内死亡第十一页，讲稿共四十三页哦影像学表现X线平片：头颅呈球形增大，囟门大、颅缝宽、颅壁薄，颅面骨比例失调CT表现：额、顶、颞叶脑实质几乎消失，部分枕叶、基底神经核、丘脑保存，小脑、脑干、四脑室一般正常M
10、RI表现：幕上为水样信号，大脑镰、基底节、小脑及脑干结构正常第十二页，讲稿共四十三页哦先天性脑积水第十三页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0533.htmTitle:PowerPoint Presentation Size:6666 Last Modified:Thu,15 Jun 2006 02:08:08 GMT 又称 丹-瓦氏畸形 Da
11、ndy-Walker malformation 小脑半球及蚓部发育不良、四脑室中孔和侧孔闭 锁致四脑室扩张和梗阻性脑积水四脑室中、侧孔先天性闭塞四脑室中、侧孔先天性闭塞第十四页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0535.htmTitle:PowerPoint Presentation Size:6644 Last Modified:Thu,15
12、 Jun 2006 02:08:10 GMT后颅凹扩大；巨大囊肿；小脑下蚓缺如，上蚓移位；小脑半球发育不良；脑干受压，幕上脑积水鉴别：蛛网膜囊肿、大枕大池影像学表现影像学表现第十五页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0536.htmTitle:PowerPoint Presentation Size:4915 Last Modified:Thu
13、,15 Jun 2006 02:08:10 GMTDandy-Walker畸形(图)第十六页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0538.htmTitle:PowerPoint Presentation Size:8529 Last Modified:Thu,15 Jun 2006 02:08:12 GMT 概述：前脑无裂畸形、无脑回、多小脑回、
14、灰质异 位、脑裂畸形及脑沟回移位等，胚胎26月 病因不明：血管性，感染性，化学性脑裂、脑沟和脑回发育畸形脑裂、脑沟和脑回发育畸形第十七页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0539.htmTitle:PowerPoint Presentation Size:9369 Last Modified:Thu,15 Jun 2006 02:08:12 G
15、MT无脑回畸形（无脑回畸形（agyriaagyria）无脑回畸形：多见顶枕叶CT、MR表现:脑表面光滑，脑回、沟缺如 脑室扩大,侧裂池、蛛网膜 下腔增宽第十八页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0541.htmTitle:PowerPoint Presentation Size:4412 Last Modified:Thu,15 Jun 200
16、6 02:08:12 GMT无脑回畸形（图）第十九页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0551.htmTitle:PowerPoint Presentation Size:9206 Last Modified:Thu,15 Jun 2006 02:08:20 GMT 病理：融合型、非融合型 临床：难治性癫痫，运动障碍，智力低下，发 育迟缓 影像
17、学表现：中央前、后回附近表面延伸至室管膜下 裂隙（P-E缝）周围皮质不规则增厚脑裂畸形脑裂畸形（schizencephalyschizencephaly）第二十页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0555.htmTitle:PowerPoint Presentation Size:4677 Last Modified:Thu,15 Jun 20
18、06 02:08:22 GMT开放型脑裂畸形(图)第二十一页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0558.htmTitle:PowerPoint Presentation Size:9096 Last Modified:Thu,15 Jun 2006 02:08:24 GMT 病因：不清，可能与染色体异常及基因突变有关 病理：无脑叶、半脑叶、单脑
19、叶型 影像学表现：无脑叶 单脑室，少量脑组织，无中线结构并发 颅面畸形 半脑叶 三脑室中线结构缺如，或有较小大脑镰，纵裂浅 单脑叶 透明隔缺如，半球间裂浅前脑无裂畸形前脑无裂畸形（holoprosencephalyholoprosencephalyholoprosencephaly）第二十二页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0560.htmT
20、itle:PowerPoint Presentation Size:4410 Last Modified:Thu,15 Jun 2006 02:08:26 GMT前脑无裂畸形（图）第二十三页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0561.htmTitle:PowerPoint Presentation Size:5290 Last Modified
21、:Thu,15 Jun 2006 02:08:26 GMT前脑无裂畸形（图）第二十四页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0562.htmTitle:PowerPoint Presentation Size:3840 Last Modified:Thu,15 Jun 2006 02:08:28 GMT前脑无裂畸形（图）第二十五页，讲稿共四十三页哦
22、WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0521.htmTitle:PowerPoint Presentation Size:10453 Last Modified:Thu,15 Jun 2006 02:07:58 GMT脑膜膨出和脑膜脑膨出颅内结构经颅骨缺损疝出颅外病理 显性颅裂畸形型 脑膜膨出型 脑膜脑膨出 型 脑室膨出型 囊性脑膜脑膨出临床颅盖组 膨出软组织肿块
23、颅底组 眶、鼻、咽肿块第二十六页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0522.htmTitle:PowerPoint Presentation Size:5654 Last Modified:Thu,15 Jun 2006 02:08:00 GMT影像学表现CT：颅骨缺损，脑脊液密度膨出疝囊；软组织密 度疝囊MR：脑脊液信号膨出物、脑实质信号膨出
24、物，颅 内脑室牵拉、变形、扩张第二十七页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0526.htmTitle:PowerPoint Presentation Size:4407 Last Modified:Thu,15 Jun 2006 02:08:04 GMT枕部脑膜脑膨出（图）第二十八页，讲稿共四十三页哦WebZIP 7.0.3.1030 offl
25、ine browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0527.htmTitle:PowerPoint Presentation Size:9793 Last Modified:Thu,15 Jun 2006 02:08:04 GMT 病因：先天性，后天性 病理：胚胎早期损伤严重，致完全缺如；胎晚期损 伤轻，致部分缺如 临床：无症状，轻者视觉、交叉触觉障碍，重者智 力低下、癫痫胼胝体发育异常胼胝体发育异常（dysgenesi
26、s of the corpus callosum dysgenesis of the corpus callosum dysgenesis of the corpus callosum）第二十九页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0528.htmTitle:PowerPoint Presentation Size:9292 Last Modif
27、ied:Thu,15 Jun 2006 02:08:06 GMT部分缺如：压部常见，压部带状结构消失，体变薄、中断完全缺如：侧脑室扩大、分离，前角小、后角大，三脑室上升，介侧脑室间，顶紧邻纵裂 池影像学表现影像学表现第三十页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:09.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0529.htmTitle:PowerPoint Presentation Size:4
28、678 Last Modified:Thu,15 Jun 2006 02:08:06 GMT胼胝体完全性缺如(图）第三十一页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0569.htmTitle:PowerPoint Presentation Size:9096 Last Modified:Thu,15 Jun 2006 02:08:34 GMT 脑脊液
29、在脑外异常的局限性积聚 病因：先天性 先天发育 后天性 外伤、感染、手术等 病理：蛛网膜内囊肿，多不与蛛网膜下腔部交通 蛛网膜下囊肿，多与蛛网膜下腔部相通 相邻颅骨及脑组织呈受压改变 临床：部分无症状，部分有轻瘫、癫痫发作等蛛网膜囊肿蛛网膜囊肿（arachnoid cystarachnoid cyst）第三十二页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slid
30、e0570.htmTitle:PowerPoint Presentation Size:8311 Last Modified:Thu,15 Jun 2006 02:08:34 GMT 影像表现：CT、MRI 边缘光滑、脑脊液密度/信号区，无强化，占位轻，颅骨变薄 位 置 外侧裂池、大脑凸面、枕大池多见 鉴别诊断：胆脂瘤，大枕大池，脑穿通畸形囊肿影像学标表现及鉴别诊断影像学标表现及鉴别诊断第三十三页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.5
31、3.133/jpk/contents/g/yxx/doc/2-9.files/slide0574.htmTitle:PowerPoint Presentation Size:5420 Last Modified:Thu,15 Jun 2006 02:08:38 GMT蛛网膜囊肿(图)第三十四页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0578.htmT
32、itle:PowerPoint Presentation Size:3580 Last Modified:Thu,15 Jun 2006 02:08:42 GMT三、神经皮肤综合征（neurocutaneous syndromes）第三十五页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0580.htmTitle:PowerPoint Presentati
33、on Size:6509 Last Modified:Thu,15 Jun 2006 02:08:42 GMT一组遗传性斑痣性错构瘤病分两型：神经纤维瘤病型（NF-l）神经纤维瘤病型（NF-2）神经纤维瘤病神经纤维瘤病（neurofibromatosisneurofibromatosis）第三十六页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0584.h
34、tmTitle:PowerPoint Presentation Size:9235 Last Modified:Thu,15 Jun 2006 02:08:46 GMT NF-1型 NF-2型 CNS病变 颅内神经肿瘤胶质瘤 单、多发脑脊膜瘤丛状神经纤维瘤 脊神经多发神经鞘瘤其它 髓内室管膜瘤、星形细胞瘤 影像学表现影像学表现第三十七页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-
35、9.files/slide0589.htmTitle:PowerPoint Presentation Size:4676 Last Modified:Thu,15 Jun 2006 02:08:48 GMT神经纤维瘤病-II型（图）第三十八页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0590.htmTitle:PowerPoint Presentati
36、on Size:9739 Last Modified:Thu,15 Jun 2006 02:08:50 GMT 病因：常染色体显性遗传，外显率高 临床：皮脂腺瘤、癫痫、智力低下结节性硬化结节性硬化（tuberous sclerosistuberous sclerosis）第三十九页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0591.htmTitle:P
37、owerPoint Presentation Size:6109 Last Modified:Thu,15 Jun 2006 02:08:50 GMT灰、白质、室管膜下，异常结节信号、密度区、可钙化室间孔室管膜下巨细胞星形细胞瘤影像学表现影像学表现第四十页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0592.htmTitle:PowerPoint Pre
38、sentation Size:5156 Last Modified:Thu,15 Jun 2006 02:08:50 GMT结节性硬化（图）第四十一页，讲稿共四十三页哦WebZIP 7.0.3.1030 offline browser(Unregistered)on 09/13/07 10:15:10.Address:http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0597.htmTitle:PowerPoint Presentation Size:6149 Last Modified:Thu,15 Jun 2006 02:08:56 GMT顶枕叶脑回样、轨道状钙化；脑萎缩；脉络丛、引流静脉明显且强化影像学表现影像学表现第四十二页，讲稿共四十三页哦感谢大家观看第四十三页，讲稿共四十三页哦