3神经科讲课神经系统血管炎

1、神经系统血管炎神经系统血管炎 中南大学湘雅医院神经内科中南大学湘雅医院神经内科 李静李静 病理分类病理分类 n淋巴细胞性血管炎淋巴细胞性血管炎 n肉芽肿性血管炎肉芽肿性血管炎 n混合型血管炎混合型血管炎 n大血管：指主动脉及其最大大血管：指主动脉及其最大 的分支的分支(至肢体、头、颈部至肢体、头、颈部) n中血管：指主要的内脏动脉中血管：指主要的内脏动脉 (肾、肝、冠状、肠系膜动脉肾、肝、冠状、肠系膜动脉) n小血管：指小静脉小血管：指小静脉,毛细血管毛细血管, 小动脉及主质内与小动脉连小动脉及主质内与小动脉连 接的远端动脉细支接的远端动脉细支 血管炎分类（血管炎分类（按受累血管大小） 199

2、4, Chapel Hill Congress 系统系统性血管炎的分性血管炎的分类类 主要受累的血主要受累的血 管管 大血管大血管巨巨细胞动脉细胞动脉炎炎与与RARA有有关联关联的主的主动脉动脉炎炎 感染（感染（eg. eg. 梅毒梅毒) ) 中血管中血管经典经典的的结节性结节性多多动动脉炎脉炎 川崎病川崎病 感染（感染（eg. HBV)eg. HBV) 小血管和中小血管和中动动 脉脉 韦韦格纳格纳肉芽肉芽肿肿 Churg-Strauss Churg-Strauss 綜合症綜合症 显微镜显微镜下多血管炎下多血管炎 继发于继发于RA .发现双侧大脑中动脉闭塞发现双侧大脑中动脉闭塞; .治疗治疗3

3、月后月后,随访见左侧额叶、颞叶、基底节区软化灶随访见左侧额叶、颞叶、基底节区软化灶 Case 30-1996 An 81-Year-Old Man with Progressive Disorientation, Repeated Falls, and a Fluctuating Mental State N Engl J Med 1996; 335:952-959, Sep 26, 1996. Case 30-1996 An 81-Year-Old Man with Progressive Disorientation, Repeated Falls, and a Fluctuating M

4、ental State N Engl J Med 1996; 335:952-959, Sep 26, 1996. 图图2男男,于运动后出现全身无力于运动后出现全身无力,呈瘫痪状态呈瘫痪状态,休息后缓解休息后缓解,病理诊断为血管病理诊断为血管 炎炎 .增强扫描发现右侧小脑半球片状强化病灶增强扫描发现右侧小脑半球片状强化病灶; .检查发现大脑后动脉狭窄检查发现大脑后动脉狭窄,粗细不均匀粗细不均匀; .治疗后治疗后4个月复查个月复查,右小脑病灶消失右小脑病灶消失 Diffusion-Weighted Imaging of Cerebritis American Journal of Neurora

5、diology 24:1110-1113, June-July 2003 Spinal Cord Involvement in Primary Angiitis of the Central Nervous System: A Report of Two Cases American Journal of Neuroradiology 22:577-582 (3 2001) nHemorrhage in CNS vasculitis. nA, Axial SE MR image 示示 T1 高信号高信号, 提示左侧壳核提示左侧壳核(large straight arrow), 左基左基 底节底

6、节 (curved arrow)和右侧是外囊和右侧是外囊 (small arrows)出血出血. nB, The corresponding T2-weighted SE image 示双侧基底节特别是右侧尾状核示双侧基底节特别是右侧尾状核 头部、头部、 (large arrow),外囊外囊 (small arrows), 和内囊后支和内囊后支 (arrowhead)高信号高信号. 及及 左侧顶叶高集中左侧顶叶高集中 nC, 右颈总右颈总A动脉造影示动脉造影示M1段狭窄段狭窄 (double arrow) MCA 远端信号减弱远端信号减弱 (single arrow). A, CT scan

7、shows SAH (arrows). B, SE MR image (3000/100) corresponding to the CT scan in A fails to reveal any parenchymal abnormality; however, decreased T2 signal is present in the region corresponding to subarachnoid blood on the CT scan (arrows). C, Coronal SE MR image (600/20) shows high signal intensity

8、material that may be caused by hemorrhage over the left parietal lobe (arrow). D, Angiogram shows multiple foci of dilatation and stenosis, predominantly in the left ACA distribution (arrowheads). N Engl J Med 1993; 329:117-124, Jul 8 n图图1、小血管壁增厚，纤维素样变性，、小血管壁增厚，纤维素样变性， 大量淋巴细胞为主的炎性细胞浸润，大量淋巴细胞为主的炎性细胞浸

9、润， 炎细胞侵及小血管壁，并在小血管周炎细胞侵及小血管壁，并在小血管周 围呈围呈“套袖套袖”样浸润。（样浸润。（HE染色，染色， 20） n图图2、个别血管周围可见含铁血黄素沉、个别血管周围可见含铁血黄素沉 积。（积。（HE染色，染色，40） n图图3、病变部位脑组织结构疏松，髓鞘、病变部位脑组织结构疏松，髓鞘 变性。（变性。（FASTBLUE染色，染色，20） n病理诊断：符合中枢神经系统血管炎病理诊断：符合中枢神经系统血管炎 Lymphocytic Vasculitis Mimicking Aggressive Multifocal Cerebral Neoplasm: MR Imagin

10、g and MR Spectroscopic Appearance AJNR Am. J. Neuroradiol., Mar 2005; 26: 642 - 645 AJNR Am. J. Neuroradiol., Mar 2005; 26: 642 - 645 AJNR Am. J. Neuroradiol., Mar 2005; 26: 642 - 645 American Journal of Neuroradiology 22:1283-1290 (8 2001) nA, T2：示双侧大脑白质内弥漫性高信号，脑沟增宽示脑萎缩：示双侧大脑白质内弥漫性高信号，脑沟增宽示脑萎缩 nB,

11、增强示双侧大脑白质多灶性点状、线状强化，左枕叶结节状强化增强示双侧大脑白质多灶性点状、线状强化，左枕叶结节状强化 (curved arrows). nFigure 1. Sagittal T2- Weighted MRI Scan Showing a Diffuse Hyperintense Signal within the Pons and Midbrain, with Superior Extension into the Thalamus. nThe brain stem is slightly enlarged, and there is dilatation of the late

12、ral and third ventricles. Case 33-1995 Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old man treated for demyelinating disease N Engl J Med 1995; 333:1135-1143, Oct 26, 1995. nFigure 2. Axial T1-Weighted MRI Scan Showing Scattered Punctate Foci

13、 with Enhancement after the Administration of Gadolinium, Predominantly throughout the White Matter. Case 33-1995 Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old man treated for demyelinating disease N Engl J Med 1995; 333:1135-1143, Oct 26,

14、1995. Axial Nonenhanced CT Scan, Showing an Intraparenchymal Hemorrhage in the Left Frontoparietal Region, with Surrounding Edema, a Shift of the Midline, and Subfalcial Herniation. Case 33-1995 Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old

15、 man treated for demyelinating disease N Engl J Med 1995; 333:1135-1143, Oct 26, 1995. Axial T2-Weighted MRI Scan Showing Extensive, Confluent Hyperintense Signal Predominantly throughout the White Matter. nNumerous small, relatively low-signal nodules are scattered throughout the abnormal white mat

16、ter. Case 33-1995 Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old man treated for demyelinating disease N Engl J Med 1995; 333:1135-1143, Oct 26, 1995. Axial T1- Weighted MRI Scan Revealing Extensive Enhancement of the Nodules after the Admin

17、istration of Gadolinium. Case 33-1995 Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old man treated for demyelinating disease N Engl J Med 1995; 333:1135-1143, Oct 26, 1995. nFigure. (A) Gadolinium-enhanced, T1-weighted MRI of the brain demonst

18、rating unihemispheric disease. (B) Cerebral biopsy. Leptomeningeal vessels with transmural and perivascular chronic inflammatory cell infiltrate composed of mainly small lymphocytes with occasional multinucleate giant cells; well-defined granulomas and vessel necrosis were lacking (original magnific

19、ation 240, hematoxylin and eosin). (C) Immunohistochemistry shows numerous transmural chronic inflammatory cells in small-caliber vessel walls of the cortex, indicative of an active vasculitic process (original magnification 320, hematoxylin and eosin). Isolated angiitis of the central nervous syste

20、m: A case presented with atypical psychiatric symptoms In the second attack of the patients illness, foci of increased signal intensity on the T2-weighted images. Isolated angiitis of the central nervous system: A case presented with atypical psychiatric symptoms After steroid therapy, the generaliz

21、ed decrease in size of the lesions. Progress in Neuro-Psychopharmacology 349:170-180, Jul 10 Magnetic resonance angiography in a patient with Crohns disease associated cerebral vasculitis Clinical Neurology and Neurosurgery, Volume 106, Issue 2, March 2004, Pages 110-113 CNS Vasculitis in Autoimmune

22、 Disease: MR Imaging Findings and Correlation with Angiography American Journal of Neuroradiology 20:75-85 (1 1999) n病人病人36岁，男性，起病急骤，主要表现为左侧肢体偏瘫，发病第岁，男性，起病急骤，主要表现为左侧肢体偏瘫，发病第5天行天行 MRI检查，检查，T2加权自旋回波序列加权自旋回波序列MRI示病灶为上至右侧侧脑室旁，下示病灶为上至右侧侧脑室旁，下 至桥脑、脑桥臂、中脑的广泛病灶，累及右侧丘脑、内囊以及尾状核至桥脑、脑桥臂、中脑的广泛病灶，累及右侧丘脑、内囊以及尾状核 B

23、rain of a Patient with VaricellaZoster Viral Encephalitis, or Small-Vessel Vasculopathy. nShown are multifocal areas of infarction: a superficial wedge- shaped lesion (long solid arrow), deep ovoid lesions in white matter (open arrows), and smaller lesions at junctions of gray and white matter (shor

24、t solid arrows). MRIMRI示双侧基底节区对称性长示双侧基底节区对称性长T2T2信号信号 Laboratory evaluation of headache and vasculitis 建议下列表现可以作为建议下列表现可以作为 的诊断标准的诊断标准: 鉴别诊断鉴别诊断(childhood) nTable 2. Mimics of central nervous system vasculitis in childhood From: Benseler: Curr Opin Rheumatol, Volume 16(1).January 2004.43-50 显性遗传性脑血管

25、病伴随皮层下显性遗传性脑血管病伴随皮层下 痴呆和白质脑病痴呆和白质脑病 影像特征影像特征 动脉硬化性脑梗塞或动脉硬化性脑梗塞或Binswanger综合综合 征征 多发性硬化多发性硬化 部分中枢神经系统血管炎患者的临床表现类似多部分中枢神经系统血管炎患者的临床表现类似多 发硬化，出现自发缓解及复发，伴有视神经病变发硬化，出现自发缓解及复发，伴有视神经病变 和脑干病变，脑脊液寡克隆区带阳性。和脑干病变，脑脊液寡克隆区带阳性。 n但惊厥、严重头痛及脑病等常出现在中枢神经系但惊厥、严重头痛及脑病等常出现在中枢神经系 统血管炎，很少出现在多发性硬化中。统血管炎，很少出现在多发性硬化中。 n多发性脑梗塞伴

26、随弥漫性的白质损害的影象学改多发性脑梗塞伴随弥漫性的白质损害的影象学改 变出现在血管炎，不出现在多发性硬化。变出现在血管炎，不出现在多发性硬化。 治疗治疗 血管炎周围神经病分类血管炎周围神经病分类 2005 50-70% 1966年，年，Brain等等111报道一例报道一例40岁男性患岁男性患 者，主要表现为难以解释的复发性、可自者，主要表现为难以解释的复发性、可自 行缓解的卒中样发作及昏迷，此异常表现行缓解的卒中样发作及昏迷，此异常表现 仅见于桥本氏甲状腺炎仅见于桥本氏甲状腺炎( Hashimoto s Thyroditis , HT )。其后，许多文献报道了。其后，许多文献报道了 多种中枢

27、神经系统功能异常与多种中枢神经系统功能异常与HT的关系，的关系， 并将这类疾病命名为桥本氏脑病并将这类疾病命名为桥本氏脑病 (Hashimoto s encephalopathy , HE)。 定义定义 病因及发病机制病因及发病机制 因免疫炎性反应累及血脑屏障，使血脑屏障受因免疫炎性反应累及血脑屏障，使血脑屏障受 损而致脑内多发性局灶性水肿或弥漫性脑水肿可累损而致脑内多发性局灶性水肿或弥漫性脑水肿可累 及脑干及皮层，从而出现局灶性神经功能缺失或昏及脑干及皮层，从而出现局灶性神经功能缺失或昏 迷等临床症状，并可随皮层激素的应用而迅速缓解。迷等临床症状，并可随皮层激素的应用而迅速缓解。 血管源性水

28、肿学说血管源性水肿学说较好地解释了较好地解释了HE的的 临床过程，但具体的机制，如免疫炎性反应临床过程，但具体的机制，如免疫炎性反应 是如何介导的、与是如何介导的、与 HT的发病机制的关系等的发病机制的关系等 尚需进一步研究阐明。尚需进一步研究阐明。 上述二种形式均可出现痛性发作、肌阵挛、上述二种形式均可出现痛性发作、肌阵挛、 震颤及木僵等。前者起病较急，而后者起病相震颤及木僵等。前者起病较急，而后者起病相 对缓慢。对缓慢。 实验室检查实验室检查 血清抗甲状腺过氧化物酶抗体（血清抗甲状腺过氧化物酶抗体（ anti TPO） 和和/或抗甲状腺球蛋白抗体（或抗甲状腺球蛋白抗体（ anti -Tg）

29、明显增）明显增 高，可较正常高高，可较正常高30倍以上倍以上;抗促甲状腺激素受体抗促甲状腺激素受体 抗体可轻度升高；抗抗体可轻度升高；抗MBP抗体可为阳性；他免抗体可为阳性；他免 疫学检查及常规实验室检查多无异常发现。甲疫学检查及常规实验室检查多无异常发现。甲 状腺功能多正常。状腺功能多正常。 脑脊液检查脑脊液检查 脑电图脑电图 影像学检查影像学检查 The initial images show diffuse confluen thyperintense signal in the subcortical andperiventricular deep white matter. The basal ganglia and thalami are uninvolved. The images 2 years later show compl