基底尖动脉综合症课件

1、基底动脉尖综合症 向Louis R Caplan致敬！病例分享 与尸解报告尸解报告：死因为左侧小脑上动脉和左侧大脑后动脉供血区的栓塞。包括了中脑动眼神经核，导水管附近的灰质，红核，大脑脚，左侧小脑上部 ，左侧丘脑腹外侧核。尸解报告：中脑右侧动眼神经核，右侧中部纵向的神经纤维束和 右侧第4颅神经区域，右侧红核背侧部分。梗死病灶部分跨过中线至左侧中脑的背部，但左侧第3颅神经区域和脑干的腹侧豁免。尸解报告：腹侧的基底动脉阻塞致丘脑和中脑梗死，以及双侧大脑后动脉供血区域梗死，左侧更广泛。Case 4 尸解报告： 左侧中脑的梗死灶，主要累及左侧小脑上脚，大脑脚，黑质，红核和导水管附近灰质。“基底动脉尖”

2、综合症的由来前身：Segarra “the syndrome of the mesencephalic artery” (1970)Louis R Caplan,M.D. Neurology1980, 30:72-79What is“Top of the basilar ”syndrome?责任血管：Posterior communicating and posterior cerebral arterial tributaries of the basilar artery病灶部位：midbrain, thalamus ,portion of temporal and occipital l

3、obes，cerebellum相应症状：array of visual,oculomotor and behavioral abnormalities, usually without prominent motor dysfunctionPart 1: rostral brainstem infarctionDisorders of ocular movement1.垂直注视障碍 无论是主动或是头眼反射诱发 中脑被盖部2 .单或双眼汇聚障碍 内收位； convergence retraction nystagmus(自发或注视活动的上行物体时)3.“Pseudosixth” （Fisher

4、假性外展神经麻痹） 双侧并伴有双眼明显的内聚Part 1: rostral brainstem infarctionDisorders of ocular movement4. 单或双上睑抬升及回缩（Collier sign）5.歪斜分离（指垂直面）“midbrain skew”中脑导水管周边灰质 多伴有瞳孔及动眼神经症状6.瞳孔改变 双侧交感神经受损 ：瞳孔小 对光反应幅度小（鉴别：脑桥瞳孔 小 但反应灵敏） EW核：大且中间位固定的瞳孔瞳孔异位症 （corectopia iridis） 虹膜 交替性Part 1: rostral brainstem infarctionBehavioral

5、 abnormalities:1.Somnolence（嗜睡） a patient with bilateral third nerve palsies in sleep-like state for 3 years,尸解发现基底动脉尖阻塞2.Peduncular hallucinosis（大脑脚幻觉）生动且逼真，但病人多数能意识到其不真实病灶指中脑，而非仅限于大脑脚多数出现在晚上Part 1: rostral brainstem infarctionBehavioral abnormalities:3.异常的回答 （“unusual reports”）定向力丧失，多有睡眠障碍在某些刺激下容易

6、出现，如看到图片一般不模拟现实检查者的观察或问话常被融入患者的回答一部分患者做梦多，难以分清梦境与现实Part 2：posterior cerebral artery territory hemisphere infarction单侧病灶 ：Visual defects:偏盲1.能意识到视野有缺损 鉴别：顶叶病变往往有视觉忽略2. 保留视运动性眼球震颤 占位病变消失，血管病变存在；3. 偏盲视野内偶尔可见物体！ 鉴别：距状沟附近 VS 视觉通路上大脑中动脉供血区域 Part 2：posterior cerebral artery territory hemisphere infarction单

7、侧病灶 ：Visual defects:4.在偏盲视野内上下象限受累程度不同5.在偏盲视野边缘可见闪光 5/156.视觉迟滞 三种形式：a train of individuals repeated to hemianopic field; looking toward hemianopic field but image of previously in front of him;seen after being movedPart 2：posterior cerebral artery territory hemisphere infarction单侧病灶：Behavioral defec

8、ts左侧枕叶命名性失语失读但不失写一过性Korsakoff样健忘综合症视觉失认右侧枕叶Charcot-Wilbrand综合症表现为视觉影像回忆能力丧失，无法产生视觉梦境及人面失认（视觉失认综合症目前认为优势侧角回）Part 2：posterior cerebral artery territory hemisphere infarction双侧病灶Visual defects: Cortical blindnessThe Balint syndrome（双侧顶枕病变综合症） Asimultagnosia（刺激失认）无法一览无余 optical apraxia(视觉失用)眼手无法协调 Aprax

9、ia of gaze（凝视不能）不能随心所“看” Metamorphosia（视觉变形）Behavorial abnormalities Memory defects（记忆力减退） Agitatied delirium（激越型谵妄）sensory defects以本体感觉异常症状更明显Motor defects少见，轻度面瘫除外发病机制Embolic mechanism(intra-arterial or cardiac)Atherosclerosis is usually most severe at the origin of the vertebral artery in the nec

10、k,in the intracranial portion of the vertebral artery and at the proximal end of the basilar artery.Neuroradiology. 1987;29(4):354-9.Top of the basilar syndrome: clinico-radiological evaluation.Sato M, Tanaka S, Kohama A.AbstractSixteen patients having infarction caused by circulatory disturbance at

11、 top of the basilar artery, that is to say, the top of the basilar syndrome, were studied, the diagnosis having been made by computerized tomography. Infarcts were widely distributed in each patient between the thalamus, midbrain, pons, cerebellum, and occipital lobe. Both thalami were involved in 7

12、 cases. When the thalamus was involved bilaterally, the low density areas were symmetrical in size and localization. Angiography revealed that stenosis or occlusion lay within a circle 2 cm in diameter surrounding the five-forked junction at the top of the basilar artery in 84.6%. Recanalization of

13、the occluded artery occurred in 61.5%, suggesting that embolism played an important role in appearance of this syndrome.J Stroke Cerebrovasc Dis. 2012 Nov;21(8):909.e7-8. doi:.Transient total mesencephalic locked-in syndrome after bilateral ptosis due to basilar artery thrombosis.AbstractLocked-in s

14、yndrome (LIS) usually occurs as a result of pontine lesions and has been classified into various categories on the basis of neurologic conditions, of which transient total mesencephalic LIS is extremely rare. A 53-year-old man presented with bilateral ptosis followed by a total locked-in state. In t

15、he clinical course, the patient successfully recovered with only left slight hemiparesis（轻偏瘫） and skew deviation remaining. Magnetic resonance imaging revealed multiple ischemic lesions caused by thrombosis at the top of basilar artery, including the bilateral cerebral peduncles, tegmentum（被盖） of the midbrain, and the right cerebellar hemisphere. Antecedent bilateral pt