本文選題：原發(fā)性中樞神經(jīng)系統(tǒng)血管炎 + 瘤樣脫髓鞘病變��； 參考：《大連醫(yī)科大學(xué)》2017年碩士論文

【摘要】：背景原發(fā)性中樞神經(jīng)系統(tǒng)血管炎(primary angiitis of the central nervous system,PACNS)是一種累及中樞神經(jīng)系統(tǒng)中小血管和軟腦膜微血管管壁的病因不明的炎癥性病變。臨床及輔助檢查多缺乏特異性表現(xiàn),尤其是影像上常為占位樣表現(xiàn),易誤診為瘤樣脫髓鞘病變(tumefactivedemyelinating lesions,TDLs)。TDLs 是一種特殊類型的中樞神經(jīng)系統(tǒng)炎性脫髓鞘病,常累及皮質(zhì)及皮質(zhì)下,病灶多不規(guī)則,又常以頭痛為首發(fā)癥狀,也易與PACNS相混淆。特別是二者病理組織學(xué)改變受取材影響也有一些相似特點(diǎn),有時病理診斷也很難清楚界定。因此,對于兩者臨床、影像、病理究竟有何異同,國內(nèi)外這些方面的對比研究較少,值得進(jìn)一步研究。目的對比PACNS與TDLs的臨床、影像及病理特點(diǎn),為臨床鑒別提供重要參考依據(jù)。提高對PACNS及TDLs的診斷及鑒別診斷能力,以減少誤診與漏診。方法回顧性分析就診于海軍總醫(yī)院住院的經(jīng)病理證實(shí)的PACNS19例、TDLs17例,具有完整的臨床、影像、病理資料。結(jié)果1.PACNS與TDLs的一般人口學(xué)資料及發(fā)病特點(diǎn):①PACNS發(fā)病平均年齡32.0±13.7歲,TDLs發(fā)病平均年齡39.3±12.8歲,兩組無統(tǒng)計(jì)學(xué)差異。②PACNS男性13例、女性6例,TDLs男性7例、女性10例,男女發(fā)病無統(tǒng)計(jì)學(xué)差異。③PACNS平均就診病程42.2±70.7W,TDLs平均就診病程4.0±2.7W,顯然PACNS病程較TDLs就診病程長,兩者存在統(tǒng)計(jì)學(xué)差異(P=0.033)。④PACNS以慢性起病多見(P=0.001),TDLs則以亞急性起病常見(P=0.010),具有統(tǒng)計(jì)學(xué)差異。2.PACNS與TDLs的首發(fā)癥狀及主要臨床表現(xiàn)對比:(1)PACNS常見首發(fā)癥狀頭痛(5例)、頭暈(4例)、癲癇(3例),而TDLs為頭暈(4例)、頭痛(3例)、肢體無力(3例),兩組各項(xiàng)首發(fā)癥狀均無統(tǒng)計(jì)學(xué)差異。(2)PACNS主要臨床表現(xiàn)為頭痛52.6%(10/19)、肢體無力47.3%(9/19)、癲癇31.5%(6/19)、認(rèn)知功能下降21.1%(4/19)、頭暈21.1%(4/19)、感覺障礙21.1%(4/19)、視力下降15.8%(3/19)、飲水嗆咳10.5%(2/19)、失語5.2%(1/19)、意識障礙5.2%(1/19)、中樞性面舌癱5.2%(1/19)、眼球突出5.2%(1/19)。TDLs的主要臨床表現(xiàn)為肢體無力29.4%(5/17)、頭暈23.5%(4/17)、中樞性面舌癱17.6%(3/17)、頭痛17.6%(3/17)、認(rèn)知功能下降17.6%(3/17)、感覺障礙 17.6%(3/17)、視力下降 17.6%(3/17)、癲癇 5.9%(1/17)、失語5.9%(1/17)、飲水嗆咳5.9%(1/17)。頭痛組存在統(tǒng)計(jì)學(xué)差異(P=0.041),余臨床主要表現(xiàn)無統(tǒng)計(jì)學(xué)差異。3.PACNS與TDLs影像學(xué)特點(diǎn)對比:(1)病灶累及皮層或皮層下的PACNS患者63.2%、TDLs29.4%,兩組存在統(tǒng)計(jì)學(xué)差異(P=0.041)。病灶累及腦干的PACNS患者為15.8%、TDLs52.9%,兩組有統(tǒng)計(jì)學(xué)差異(P=0.033)。累及大腦腦葉結(jié)構(gòu)中,累及顳葉的PACNS8例(42.1%)、TDLs1例(5.9%),兩組有統(tǒng)計(jì)學(xué)差異(P=0.020);累及頂葉的PACNS 9例(47.4%)、TDLs 2例(11.8%),有統(tǒng)計(jì)學(xué)差異(P=0.017);累及枕葉的PACNS 5例(26.3%),TDLs 0例,存在統(tǒng)計(jì)學(xué)差異(P=0.017)。兩組在其它受累部位側(cè)腦室旁、丘腦、基底節(jié)區(qū)、半卵圓中心、胼胝體、小腦的對比無統(tǒng)計(jì)學(xué)差異。(2)PACNS平均病灶數(shù)量2±1個,TDLs 5±4個,無統(tǒng)計(jì)學(xué)差異;PACNS多發(fā)病變16例、單發(fā)病變3例,TDLs多發(fā)病變11例、單發(fā)病變6例,無統(tǒng)計(jì)學(xué)差異。PACNS單純幕上15例、單純幕下2例、混合2例;TDLs單純幕上9例、單純幕下6例、混合2例。(3)12例PACNS患者病灶中心可見短T1,相應(yīng)部位呈短T2改變,TDLs無一例呈短T1、短T2信號改變,具有統(tǒng)計(jì)學(xué)差異(P=0.000);所有PACNS的T1WI、T2WI病灶均為不均勻病灶,所有TDLs均呈均勻病灶,存在統(tǒng)計(jì)學(xué)差異(P=0.000);兩組T2FLAIR均為高信號。(4)68.4%PACNS的DWI呈病灶中心低信號、周邊高信號,TDLs0例,有統(tǒng)計(jì)學(xué)差異(P=0.000);15.8%呈不均勻高信號、10.5%為病灶低信號內(nèi)存在點(diǎn)狀高信號、5.2%呈低信號。17例TDLs的DWI均呈均勻高或稍高信號,而PACNS無1例,具有統(tǒng)計(jì)學(xué)差異(P=0.000)。(5)19例PACNS患者中15例呈現(xiàn)不規(guī)則、不連續(xù)、不均勻強(qiáng)化,其中有的可表現(xiàn)為不規(guī)則腸腔樣強(qiáng)化、不規(guī)則不均一彌漫樣強(qiáng)化、不規(guī)則不連續(xù)壁薄厚不均勻強(qiáng)化;1例結(jié)節(jié)狀強(qiáng)化,1例斑片狀強(qiáng)化,1例腦膜樣強(qiáng)化,1例粟粒樣強(qiáng)化。TDLs強(qiáng)化方式是多樣的,梳齒征強(qiáng)化4例、片狀強(qiáng)化4例、C形強(qiáng)化3例、環(huán)形強(qiáng)化2例、線狀強(qiáng)化2例、點(diǎn)狀強(qiáng)化1例、斑片狀強(qiáng)化1例。(6)水腫程度:PACNS無水腫3例,輕度8例,中度7例,重度1例;TDLs無水腫2例,輕度10例,中度5例,重度0例。占位效應(yīng):PACNS無占位效應(yīng)6例,輕度7例,中度4例,明顯占位效應(yīng)2例;TDLs無占位效應(yīng)4例,輕度9例,中度3例,明顯占位效應(yīng)1例。上述均無統(tǒng)計(jì)學(xué)差異。(7)3例PACNS患者行SWI檢查,均有陳舊出血之低信號改變。3例TDLs行SWI檢查,未見出血信號改變。3例PACNS與10例TDLs行MRS檢查均提示Ch0/Cr、Cho/NAA以及脂質(zhì)、乳酸峰均上升,無特異性。4.PACNS與TDLs病理對比:PACNS肉芽腫型1例(5.2%)、TDLs 0例,無統(tǒng)計(jì)差別;PACNS淋巴細(xì)胞浸潤型13例(68.4%)、TDLs 6例(35.3%),無統(tǒng)計(jì)差異;PACNS纖維素壞死型5例(26.3%),TDLs 0例,存在統(tǒng)計(jì)學(xué)差異(P=0.047);PACNS組血管壁炎性浸潤13例(68.4%),TDLs1例(5.8%),有統(tǒng)計(jì)學(xué)差別(P=0.002)。PACNS髓鞘脫失2例(10.5%),TDLs 17例(100%),有統(tǒng)計(jì)學(xué)差異(P=0.000)。非特異性炎癥反應(yīng)、組織壞死、軸索染色、免疫組化(CD3、CD20、CD68、LCA、GFAP)均無統(tǒng)計(jì)學(xué)差異。結(jié)論1.PACNS起病方式以慢性起病多見,TDLs以亞急性起病常見;PACNS就診病程比TDLs長。2.PACNS頭痛較TDLs多見。3.PACNS與TDLs影像學(xué)有顯著區(qū)分:(1)PACNS好發(fā)于皮層及皮層下,且易累及顳頂枕腦葉結(jié)構(gòu),TDLs易累及腦干;(2)PACNS病灶中心呈短T1、短T2信號為其顯著特點(diǎn),TDLs無一例短T1、短T2信號改變;(3)PACNS的DWI以病灶中心低信號、周邊高信號為主,TDLs則呈均勻高信號;(4)PACNS病灶不均勻,TDLs病灶相對較均勻;(5)PACNS以不規(guī)則、不連續(xù)強(qiáng)化為其主要特點(diǎn),TDLs以相對連續(xù)強(qiáng)化(梳齒征、片狀、C形、環(huán)形強(qiáng)化等)常見;(6)PACNS與TDLs均以輕、中度水腫及輕度占位效應(yīng)為主。4.PACNS與TDLs病理學(xué)存在區(qū)分:(l)PACNS病理分型有肉芽腫型、淋巴細(xì)胞浸潤型、纖維素壞死型,TDLs僅可見淋巴細(xì)胞浸潤型;(2)PACNS以血管壁炎性細(xì)胞浸潤為其主要病理特征,相對而言TDLs以血管周淋巴細(xì)胞浸潤為其主要特點(diǎn);(3)極少PACNS存在髓鞘脫失,TDLs則主要表現(xiàn)為髓鞘脫失。
[Abstract]:Background primary central nervous system vasculitis (primary angiitis of the central nervous system, PACNS) is an unidentified inflammatory disease involving the middle and small vessels of the central nervous system and the microvascular wall of the pia CNS. Clinical and auxiliary examinations are often lack of specific manifestations, especially in images, which are often misdiagnosed. Tumefactivedemyelinating lesions (TDLs).TDLs is a special type of inflammatory demyelinating disease of the central nervous system, often involved in the cortex and subcortical, the focus is often irregular, and often with headache as the first symptom, it is also easy to confuse with PACNS. In particular, the two cases of histopathological changes are also influenced by some materials. Similar characteristics, sometimes pathological diagnosis is difficult to define clearly. Therefore, what is the difference between the clinical, image, and pathology of the two, there are few comparative studies at home and abroad, and it is worth further study. Objective to compare the clinical, imaging and pathological features of PACNS and TDLs, to provide important reference for clinical identification and to improve the diagnosis of PACNS and TDLs. And differential diagnosis ability to reduce misdiagnosis and missed diagnosis. Method retrospective analysis of PACNS19 cases confirmed by pathology in Navy General Hospital, TDLs17 cases, with complete clinical, imaging, pathological data. Results the general demographic data and pathogenesis of 1.PACNS and TDLs: (1) the average age of PACNS is 32 + 13.7 years, and the average of TDLs is on the average. Age 39.3 + 12.8 years old, there was no statistical difference between two groups. (2) PACNS male 13 cases, female 6 cases, TDLs male 7 cases, female 10 cases, there was no statistical difference between men and women. (3) the average course of medical treatment was 42.2 + 70.7W, TDLs average course was 4 + 2.7W, obviously the course of PACNS was longer than that of TDLs, and there was a statistical difference between the two (P=0.033). (4) PACNS with chronic The onset was common (P=0.001), and TDLs was common in subacute onset (P=0.010). There were statistical differences between the first symptoms of.2.PACNS and TDLs and their main clinical manifestations: (1) common symptoms of headache (5 cases), dizziness (4 cases), epilepsy (3 cases), TDLs for dizziness (4 cases), headache (3 cases), limb weakness (3 cases), and no statistics of the first symptoms of two groups. (2) the main clinical manifestations of PACNS were headache 52.6% (10/19), limb weakness 47.3% (9/19), epilepsy 31.5% (6/19), cognitive decline 21.1% (4/19), dizziness 21.1% (4/19), sensory disturbance 21.1% (4/19), visual impairment 15.8% (3/19), choking 10.5% (2/19) in drinking water, 5.2% (1/19), 5.2% (1/19), central facial tongues 5.2% (1/19), and eyeball process. The main clinical manifestations of 5.2% (1/19).TDLs were limb weakness (5/17), dizziness 23.5% (4/17), central facial palsy 17.6% (3/17), headache 17.6% (3/17), cognitive decline 17.6% (3/17), sensory impairment 17.6% (3/17), visual impairment 17.6% (3/17), epilepsy 5.9% (1/17), 5.9% (1/17) aphasia and 5.9% (1/17) in drinking water. There was statistical difference in headache group. P=0.041, there was no statistical difference between.3.PACNS and TDLs: (1) there were 63.2%, TDLs29.4%, and two groups of PACNS patients with cortical or subcortical lesions (P=0.041). The PACNS patients involving the brain stem were 15.8%, TDLs52.9%, and two groups were statistically different (P=0.033). PACNS8 (42.1%), TDLs1 (5.9%) involving the temporal lobe (42.1%), two groups with statistical difference (P=0.020), 9 cases of PACNS (47.4%) involving the parietal lobe, 2 TDLs (11.8%), PACNS 5 (26.3%) involving occipital lobe, 0 cases of TDLs (P=0.017). The two group was in the side ventricle of the other affected parts, the thalamus, and basal ganglia. There was no statistical difference between the center of the oval circle, the corpus callosum and the cerebellum. (2) the average number of PACNS lesions was 2 + 1, TDLs 5 + 4, without statistical difference; there were 16 cases of PACNS multiple lesions, 3 cases of single lesion, 11 cases of multiple lesions, 6 cases of single lesion, 15 cases on.PACNS single screen, 2 cases simple under the episodes, 9 cases on the simple act of TDLs, alone, 9 cases on the simple act of TDLs, alone, alone, alone on the act of simple act of 9 cases, alone, alone, alone on the TDLs screen. There were 6 cases under pure screen, and 2 cases were mixed. (3) the focus center of 12 PACNS patients showed short T1, the corresponding site showed a short T2 change, no TDLs was short T1, the short T2 signal was changed with statistical difference (P=0.000); all PACNS's T1WI, T2WI focus were uneven lesions, all TDLs were uniform, and there were statistical differences (P=0.000) in all two groups. Signal. (4) the DWI of 68.4%PACNS showed low signal of focus center, peripheral high signal, TDLs0 case, with statistical difference (P=0.000); 15.8% was uneven high signal, 10.5% was low signal memory in point like high signal, 5.2% low signal.17 case TDLs DWI showed uniform high or slightly high signal, and PACNS No 1 cases (P=0.000). (5) 19 PA (5) 19 cases PA. 15 of the patients with CNS were irregular, discontinuous and uneven. Some of them were irregular intestinal cavity enhancement, irregular and heterogeneous diffuse enhancement, irregular and discontinuous wall thickness, 1 cases of nodular enhancement, 1 patchy enhancement, 1 cases of meningoid enhancement, and 1 miliary enhancement.TDLs strengthening methods. 4 cases of tooth sign enhancement, 4 cases of flaky enhancement, 3 cases of C shape strengthening, 2 cases of ring strengthening, 2 cases of linear strengthening, 1 cases of dot strengthening, 1 cases of patchy enhancement. (6) edema without 3 cases, 8 mild cases, moderate 7, severe 1 cases, TDLs without edema 2 cases, mild 10 cases, moderate cases and severe cases. There were 2 cases of significant occupying effect, 4 cases of TDLs no occupying effect, 9 mild cases, 3 moderate cases and 1 cases of significant occupying effect. (7) 3 cases of PACNS patients underwent SWI examination, all of which had low signal changes of old bleeding and SWI examination in TDLs, no bleeding signal changes.3 PACNS and 10 TDLs cases MRS examination were both Ch0/Cr, Cho/NAA, and MRS. Lipid, lactic acid peak increased, no specific.4.PACNS and TDLs pathological comparison: PACNS granulomatous 1 cases (5.2%), TDLs 0 cases, no statistical difference, PACNS lymphocyte infiltration 13 cases (68.4%), TDLs 6 cases (35.3%), no statistical difference, PACNS cellulose necrotic type 5 cases (26.3%), TDLs 0 cases, there were statistical differences (P=0.047); PACNS group vascular wall inflammatory infiltration 13 Cases (68.4%), TDLs1 (5.8%), statistically significant (P=0.002).PACNS myelin loss in 2 cases (10.5%), TDLs 17 cases (100%), there were statistically significant differences (P=0.000). Non specific inflammatory response, tissue necrosis, axonal staining, immunohistochemical (CD3, CD20, CD68, LCA, GFAP) have no statistical difference. Conclusion 1.PACNS onset method is more common with chronic onset, TDLs to subacute Sexual onset is common; the course of PACNS is more than TDLs long.2.PACNS headache more than TDLs,.3.PACNS and TDLs imaging can be distinguished: (1) PACNS is well distributed in the cortex and subcortex, and is prone to the temporal occipital lobe structure, TDLs is easy to involve brain stem; (2) the center of PACNS focus is short T1, short T2 signal is its significant characteristics, TDLs has no short, short signal change; (3) The DWI of PACNS was characterized by low signal of focus center, high peripheral signal and uniform high signal in TDLs; (4) the focus of PACNS is uneven and TDLs focus is relatively uniform; (5) PACNS is irregular and discontinuous intensification is the main characteristic, TDLs is common in relative continuous enhancement (comb sign, slice shape, C shape, ring strengthening, etc.); (6) PACNS and TDLs are mild and moderate edema. The.4.PACNS and the mild occupying effect were mainly differentiated between.4.PACNS and TDLs pathology: (L) the pathological types of PACNS were granuloma type, lymphocyte infiltration type, cellulose necrotic type, and TDLs only infiltrating type of lymphocyte; (2) PACNS was the main pathological characteristic of vascular wall inflammatory cell infiltration, and relative TDLs was mainly pericyte infiltration of blood vessels. Characteristics: (3) few PACNS had myelin demyelination; TDLs was mainly myelin depigmentation.
【學(xué)位授予單位】：大連醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R743

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