基底尖動(dòng)脈綜合癥課件

1、基底動(dòng)脈尖綜合癥 向Louis R Caplan致敬！病例分享 與尸解報(bào)告尸解報(bào)告：死因?yàn)樽髠?cè)小腦上動(dòng)脈和左側(cè)大腦后動(dòng)脈供血區(qū)的栓塞。包括了中腦動(dòng)眼神經(jīng)核，導(dǎo)水管附近的灰質(zhì)，紅核，大腦腳，左側(cè)小腦上部 ，左側(cè)丘腦腹外側(cè)核。尸解報(bào)告：中腦右側(cè)動(dòng)眼神經(jīng)核，右側(cè)中部縱向的神經(jīng)纖維束和 右側(cè)第4顱神經(jīng)區(qū)域，右側(cè)紅核背側(cè)部分。梗死病灶部分跨過中線至左側(cè)中腦的背部，但左側(cè)第3顱神經(jīng)區(qū)域和腦干的腹側(cè)豁免。尸解報(bào)告：腹側(cè)的基底動(dòng)脈阻塞致丘腦和中腦梗死，以及雙側(cè)大腦后動(dòng)脈供血區(qū)域梗死，左側(cè)更廣泛。Case 4 尸解報(bào)告： 左側(cè)中腦的梗死灶，主要累及左側(cè)小腦上腳，大腦腳，黑質(zhì)，紅核和導(dǎo)水管附近灰質(zhì)。“基底動(dòng)脈尖”

2、綜合癥的由來前身：Segarra “the syndrome of the mesencephalic artery” (1970)Louis R Caplan,M.D. Neurology1980, 30:72-79What is“Top of the basilar ”syndrome?責(zé)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相應(yīng)癥狀：array of visual,oculomotor and behavioral abnormalities, usually without prominent motor dysfunctionPart 1: rostral brainstem infarctionDisorders of ocular movement1.垂直注視障礙 無論是主動(dòng)或是頭眼反射誘發(fā) 中腦被蓋部2 .單或雙眼匯聚障礙 內(nèi)收位； convergence retraction nystagmus(自發(fā)或注視活動(dòng)的上行物體時(shí))3.“Pseudosixth” （Fisher

4、假性外展神經(jīng)麻痹） 雙側(cè)并伴有雙眼明顯的內(nèi)聚Part 1: rostral brainstem infarctionDisorders of ocular movement4. 單或雙上瞼抬升及回縮（Collier sign）5.歪斜分離（指垂直面）“midbrain skew”中腦導(dǎo)水管周邊灰質(zhì) 多伴有瞳孔及動(dòng)眼神經(jīng)癥狀6.瞳孔改變 雙側(cè)交感神經(jīng)受損 ：瞳孔小 對光反應(yīng)幅度小（鑒別：腦橋瞳孔 小 但反應(yīng)靈敏） EW核：大且中間位固定的瞳孔瞳孔異位癥 （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,尸解發(fā)現(xiàn)基底動(dòng)脈尖阻塞2.Peduncular hallucinosis（大腦腳幻覺）生動(dòng)且逼真，但病人多數(shù)能意識到其不真實(shí)病灶指中腦，而非僅限于大腦腳多數(shù)出現(xiàn)在晚上Part 1: rostral brainstem infarctionBehavioral abnormalities:3.異常的回答 （“unusual reports”）定向力喪失，多有睡眠障礙在某些刺激下容易

6、出現(xiàn)，如看到圖片一般不模擬現(xiàn)實(shí)檢查者的觀察或問話常被融入患者的回答一部分患者做夢多，難以分清夢境與現(xiàn)實(shí)Part 2：posterior cerebral artery territory hemisphere infarction單側(cè)病灶 ：Visual defects:偏盲1.能意識到視野有缺損 鑒別：頂葉病變往往有視覺忽略2. 保留視運(yùn)動(dòng)性眼球震顫 占位病變消失，血管病變存在；3. 偏盲視野內(nèi)偶爾可見物體！ 鑒別：距狀溝附近 VS 視覺通路上大腦中動(dòng)脈供血區(qū)域 Part 2：posterior cerebral artery territory hemisphere infarction單

7、側(cè)病灶 ：Visual defects:4.在偏盲視野內(nèi)上下象限受累程度不同5.在偏盲視野邊緣可見閃光 5/156.視覺遲滯 三種形式：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單側(cè)病灶：Behavioral defec

8、ts左側(cè)枕葉命名性失語失讀但不失寫一過性Korsakoff樣健忘綜合癥視覺失認(rèn)右側(cè)枕葉Charcot-Wilbrand綜合癥表現(xiàn)為視覺影像回憶能力喪失，無法產(chǎn)生視覺夢境及人面失認(rèn)（視覺失認(rèn)綜合癥目前認(rèn)為優(yōu)勢側(cè)角回）Part 2：posterior cerebral artery territory hemisphere infarction雙側(cè)病灶Visual defects: Cortical blindnessThe Balint syndrome（雙側(cè)頂枕病變綜合癥） Asimultagnosia（刺激失認(rèn)）無法一覽無余 optical apraxia(視覺失用)眼手無法協(xié)調(diào) Aprax

9、ia of gaze（凝視不能）不能隨心所“看” Metamorphosia（視覺變形）Behavorial abnormalities Memory defects（記憶力減退） Agitatied delirium（激越型譫妄）sensory defects以本體感覺異常癥狀更明顯Motor defects少見，輕度面癱除外發(fā)病機(jī)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（輕偏癱） 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（被蓋） of the midbrain, and the right cerebellar hemisphere. Antecedent bilateral pt