原发性中枢神经系统血管炎与瘤样脱髓鞘病变临床、影像、病理对比研究

发布时间：2018-06-02 21:25

本文选题：原发性中枢神经系统血管炎 + 瘤样脱髓鞘病变　；参考：《大连医科大学》2017年硕士论文

【摘要】：背景原发性中枢神经系统血管炎(primary angiitis of the central nervous system,PACNS)是一种累及中枢神经系统中小血管和软脑膜微血管管壁的病因不明的炎症性病变。临床及辅助检查多缺乏特异性表现,尤其是影像上常为占位样表现,易误诊为瘤样脱髓鞘病变(tumefactivedemyelinating lesions,TDLs)。TDLs 是一种特殊类型的中枢神经系统炎性脱髓鞘病,常累及皮质及皮质下,病灶多不规则,又常以头痛为首发症状,也易与PACNS相混淆。特别是二者病理组织学改变受取材影响也有一些相似特点,有时病理诊断也很难清楚界定。因此,对于两者临床、影像、病理究竟有何异同,国内外这些方面的对比研究较少,值得进一步研究。目的对比PACNS与TDLs的临床、影像及病理特点,为临床鉴别提供重要参考依据。提高对PACNS及TDLs的诊断及鉴别诊断能力,以减少误诊与漏诊。方法回顾性分析就诊于海军总医院住院的经病理证实的PACNS19例、TDLs17例,具有完整的临床、影像、病理资料。结果1.PACNS与TDLs的一般人口学资料及发病特点:①PACNS发病平均年龄32.0±13.7岁,TDLs发病平均年龄39.3±12.8岁,两组无统计学差异。②PACNS男性13例、女性6例,TDLs男性7例、女性10例,男女发病无统计学差异。③PACNS平均就诊病程42.2±70.7W,TDLs平均就诊病程4.0±2.7W,显然PACNS病程较TDLs就诊病程长,两者存在统计学差异(P=0.033)。④PACNS以慢性起病多见(P=0.001),TDLs则以亚急性起病常见(P=0.010),具有统计学差异。2.PACNS与TDLs的首发症状及主要临床表现对比:(1)PACNS常见首发症状头痛(5例)、头晕(4例)、癫痫(3例),而TDLs为头晕(4例)、头痛(3例)、肢体无力(3例),两组各项首发症状均无统计学差异。(2)PACNS主要临床表现为头痛52.6%(10/19)、肢体无力47.3%(9/19)、癫痫31.5%(6/19)、认知功能下降21.1%(4/19)、头晕21.1%(4/19)、感觉障碍21.1%(4/19)、视力下降15.8%(3/19)、饮水呛咳10.5%(2/19)、失语5.2%(1/19)、意识障碍5.2%(1/19)、中枢性面舌瘫5.2%(1/19)、眼球突出5.2%(1/19)。TDLs的主要临床表现为肢体无力29.4%(5/17)、头晕23.5%(4/17)、中枢性面舌瘫17.6%(3/17)、头痛17.6%(3/17)、认知功能下降17.6%(3/17)、感觉障碍 17.6%(3/17)、视力下降 17.6%(3/17)、癫痫 5.9%(1/17)、失语5.9%(1/17)、饮水呛咳5.9%(1/17)。头痛组存在统计学差异(P=0.041),余临床主要表现无统计学差异。3.PACNS与TDLs影像学特点对比:(1)病灶累及皮层或皮层下的PACNS患者63.2%、TDLs29.4%,两组存在统计学差异(P=0.041)。病灶累及脑干的PACNS患者为15.8%、TDLs52.9%,两组有统计学差异(P=0.033)。累及大脑脑叶结构中,累及颞叶的PACNS8例(42.1%)、TDLs1例(5.9%),两组有统计学差异(P=0.020);累及顶叶的PACNS 9例(47.4%)、TDLs 2例(11.8%),有统计学差异(P=0.017);累及枕叶的PACNS 5例(26.3%),TDLs 0例,存在统计学差异(P=0.017)。两组在其它受累部位侧脑室旁、丘脑、基底节区、半卵圆中心、胼胝体、小脑的对比无统计学差异。(2)PACNS平均病灶数量2±1个,TDLs 5±4个,无统计学差异;PACNS多发病变16例、单发病变3例,TDLs多发病变11例、单发病变6例,无统计学差异。PACNS单纯幕上15例、单纯幕下2例、混合2例;TDLs单纯幕上9例、单纯幕下6例、混合2例。(3)12例PACNS患者病灶中心可见短T1,相应部位呈短T2改变,TDLs无一例呈短T1、短T2信号改变,具有统计学差异(P=0.000);所有PACNS的T1WI、T2WI病灶均为不均匀病灶,所有TDLs均呈均匀病灶,存在统计学差异(P=0.000);两组T2FLAIR均为高信号。(4)68.4%PACNS的DWI呈病灶中心低信号、周边高信号,TDLs0例,有统计学差异(P=0.000);15.8%呈不均匀高信号、10.5%为病灶低信号内存在点状高信号、5.2%呈低信号。17例TDLs的DWI均呈均匀高或稍高信号,而PACNS无1例,具有统计学差异(P=0.000)。(5)19例PACNS患者中15例呈现不规则、不连续、不均匀强化,其中有的可表现为不规则肠腔样强化、不规则不均一弥漫样强化、不规则不连续壁薄厚不均匀强化;1例结节状强化,1例斑片状强化,1例脑膜样强化,1例粟粒样强化。TDLs强化方式是多样的,梳齿征强化4例、片状强化4例、C形强化3例、环形强化2例、线状强化2例、点状强化1例、斑片状强化1例。(6)水肿程度:PACNS无水肿3例,轻度8例,中度7例,重度1例;TDLs无水肿2例,轻度10例,中度5例,重度0例。占位效应:PACNS无占位效应6例,轻度7例,中度4例,明显占位效应2例;TDLs无占位效应4例,轻度9例,中度3例,明显占位效应1例。上述均无统计学差异。(7)3例PACNS患者行SWI检查,均有陈旧出血之低信号改变。3例TDLs行SWI检查,未见出血信号改变。3例PACNS与10例TDLs行MRS检查均提示Ch0/Cr、Cho/NAA以及脂质、乳酸峰均上升,无特异性。4.PACNS与TDLs病理对比:PACNS肉芽肿型1例(5.2%)、TDLs 0例,无统计差别;PACNS淋巴细胞浸润型13例(68.4%)、TDLs 6例(35.3%),无统计差异;PACNS纤维素坏死型5例(26.3%),TDLs 0例,存在统计学差异(P=0.047);PACNS组血管壁炎性浸润13例(68.4%),TDLs1例(5.8%),有统计学差别(P=0.002)。PACNS髓鞘脱失2例(10.5%),TDLs 17例(100%),有统计学差异(P=0.000)。非特异性炎症反应、组织坏死、轴索染色、免疫组化(CD3、CD20、CD68、LCA、GFAP)均无统计学差异。结论1.PACNS起病方式以慢性起病多见,TDLs以亚急性起病常见;PACNS就诊病程比TDLs长。2.PACNS头痛较TDLs多见。3.PACNS与TDLs影像学有显著区分:(1)PACNS好发于皮层及皮层下,且易累及颞顶枕脑叶结构,TDLs易累及脑干;(2)PACNS病灶中心呈短T1、短T2信号为其显著特点,TDLs无一例短T1、短T2信号改变;(3)PACNS的DWI以病灶中心低信号、周边高信号为主,TDLs则呈均匀高信号;(4)PACNS病灶不均匀,TDLs病灶相对较均匀;(5)PACNS以不规则、不连续强化为其主要特点,TDLs以相对连续强化(梳齿征、片状、C形、环形强化等)常见;(6)PACNS与TDLs均以轻、中度水肿及轻度占位效应为主。4.PACNS与TDLs病理学存在区分:(l)PACNS病理分型有肉芽肿型、淋巴细胞浸润型、纤维素坏死型,TDLs仅可见淋巴细胞浸润型;(2)PACNS以血管壁炎性细胞浸润为其主要病理特征,相对而言TDLs以血管周淋巴细胞浸润为其主要特点;(3)极少PACNS存在髓鞘脱失,TDLs则主要表现为髓鞘脱失。
[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.
【学位授予单位】：大连医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R743

【参考文献】