肥厚性硬脑膜炎七例分析

肥厚性硬脑膜炎是一种临床少见的疾病，现将我们诊治的7例总结报告如下。     

肥厚性硬脑膜炎3例诊断分析及文献复习

肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)是一种少见的中枢神经系统慢性无菌性炎性疾病,其特点是硬脑膜的纤维性增生,引起神经系统进行性损害.其病因复杂,临床表现多种多样,容易误诊、漏诊.现报道我院收治的3例患者,结合文献分析如下……     

肥厚性硬脑膜炎一例

探讨肥厚性硬脑膜炎(HCP)的临床、影像学特征及治疗。分析1例此病患者的临床资料。本例患者临床表现为慢性头痛伴低热等;头部MRI示局部硬脑膜异常强化灶。皮质类固醇激素治疗后疗效显著。HCP多以慢性头痛为主要临床表现;MRI可见特征性的硬脑膜强化改变。     

特发性肥厚性硬脑膜炎

肥厚性硬脑膜炎（hypertrophic pachymeningitis，HP）是一种少见的中枢神经系统慢性无菌性炎性疾病，其特点是硬脑膜和（或）硬脊膜的纤维性增生，引起神经系统进行性损害，临床常见慢性头痛和多颅神经麻痹。对一些病因不明确的增生性硬脑膜炎，称之为特发性肥厚性硬脑膜炎（idiopathic hypertrophic pachymeningitis，IHP），可以引起整个颅内硬脑膜弥散性或局灶性损害，通常影响到大脑镰、小脑幕、鞍旁和海绵窦。临床表现和放射影像学与颅底脑膜瘤相似，大脑表面的局灶性硬脑膜增生少见，诊断上需要排除其他已知的可以引起硬脑膜增生性的疾病。     

影像呈结节状占位样的特发性肥厚性硬脑膜炎临床分析

 目的 探讨影像呈结节状占位样的特发性肥厚性硬脑膜炎(IHCP)的临床、影像及病理特点。方法 对4例影像呈结节状的IHCP患者的临床资料进行回顾性分析。结果 4例均为男性,平均发病年龄40.25(33～50)岁,病程长(4个月～12年),病情反复发作。主要表现为慢性头痛、多组脑神经麻痹及癫痫发作。头颅影像示局部硬脑膜增厚,呈团块或结节状,与脑膜瘤等肿瘤相似。2例组织病理学检查示硬脑膜结缔组织增生、散在中性粒细胞及浆细胞浸润。甲泼尼龙或联合免疫抑制剂治疗有效。结论 结节状占位样IHCP多以慢性头痛起病,糖皮质激素或联合免疫抑制剂治疗有效,病情易复发。头颅影像可见硬脑膜肥厚,呈团块或结节状占位,易与脑肿瘤、炎性肉芽肿相混淆。
        

肥厚性硬脑膜炎一例

患者男，33岁，因顽固性持续头痛，阵发性加剧3年，伴恶心、呕吐加重半年，于2003年7月18日入院。2000年5月出现不明诱因头痛，疼痛始发于左上颌窦处，继之双眼胀痛，并向两颞侧放射，最后扩散至整个头部。头痛剧烈持续约1～2h，每日2～3次，须服止痛药方能止痛。2002年8月出现左眼睑下垂、视物成双影，1周后出现左眼外展障碍，诊     

疑诊为颅内静脉窦血栓形成的肥厚性硬脑膜炎一例

<正>患者男,65岁,主因“发作性头痛4年余”于2016年3月7日收入首都医科大学宣武医院。患者于2011年11月劳累后出现头痛,主要表现为右侧颞部轻度间断性胀痛,偶可缓解。2014年6月患者出现双侧颞部间断性胀痛,程度较前加重且伴视物不清,于外院就诊并行头部MR静脉成像(MRV)及全脑DSA,示右侧横窦、乙状窦未显影(图1,2),腰椎穿刺压力为220 mm H2O,其余检查未见异常,考虑“颅内静     

肥厚性硬脑膜炎-肺部结节-肌痛-皮下结节

<正>病历摘要患者,男,38岁,因"头痛18个月,四肢游走性肌痛、胸痛6个月"于2004年10月入院。患者2003年5月无诱因出现左枕部搏动性疼痛,多在下午和夜间发作,每周2～3次,持续30～6     

变应性肉芽肿性血管炎心脏受累临床特点分析

目的:分析变应性肉芽肿性血管炎心脏受累的临床特点.方法:回顾性分析北京协和医院1990～2006年确诊的心脏受累的变应性肉芽肿性血管炎12例患者,对其临床特点进行分析.结果:心脏受累的表现主要有心肌受累、心肌梗死、心包积液、心律失常和心功能衰竭等.激素加免疫抑制剂的疗效满意,除1例未使用激素加免疫抑制剂治疗的患者猝死外.其余患者均好转.结论:变应性肉芽肿性血管炎累及心脏的表现多种多样;仅针对心脏治疗效果差,应积极治疗原发病.     

韦格纳肉芽肿病100例临床分析

目的 总结分析100例韦格纳肉芽肿病(WG)患者的临床持点方法回顾性分析北京协和医院1998年1月至2009年12月确诊的100例WG患者的临床资料结果100例患者的男女比例1.04∶1,发病年龄4～72岁,平均(39±17)岁.上呼吸道(86%)、肺脏(82%)、肾脏(70%)和眼(53%)受累常见,神经系统(12%)及心脏(11%)等相对少见.胞质型抗中性粒细胞胞质抗体(cANCA)阳性率为77%,ANCA阴性率为8%.影像学以肺部多发结节/肿块影伴或不伴空洞病灶(59%,48/81)及鼻窦炎(57%,44/77)最常见.具有诊断意义的病理为坏死性肉芽肿和(或)小血管炎,鼻黏膜/肿中物及肺活检分别占78%及75%,肾活检以局灶节段坏死性肾小球肾炎(59%)最有诊断意义.WG亚组以全身型(49%)最常见,其次为局限型(22%)、早期全身型(15%)、重症肾脏病型(9%)及难治型(5%).激素联合环磷酰胺治疗,诱导缓解期总缓解率为78%,其中早期全身型最高(73%),病死率为4%.随访1～145个月,16例(22%)合并感染,9例(12%)合并慢性肾功能不全,8例(11%)并发深静脉血栓5例(8%)死亡,其中2例死于感染结论WG临床表现复杂多样,综合ANCA、影像学和组织活检利于早期诊断,早期治疗,减少并发症的发生,改善预后.     

Hypertrophic cranial pachymeningitis in a patient with aplastic anemia

 We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T₁- and T₂-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/μl) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out. Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI. On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T₁-weighted MRI images and was also enhanced by gadolinium. Cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia. Received: April 21, 1998 / Accepted: August 28, 1998     

Two cases of hypertrophic pachymeningitis associated with myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA)-positive pulmonary silicosis in tunnel workers

Two cases of hypertrophic pachymeningitis (HP) associated with pulmonary silicosis in tunnel workers are described. In both cases the myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA) was positive. Two patients with pulmonary silicosis developed headache and neurological disturbance, and a diagnosis of HP was made. In both cases the serum CRP level and the MPO-ANCA titer were elevated. Corticosteroid therapy produced a rapid improvement in all the clinical and laboratory parameters. Although an association has been noted between exposure to silica dust and ANCA-associated vasculitis, particularly glomerulonephritis, central nervous system involvement is rare. However, there have been some recent reports of HP cases that were positive for ANCA, and the association between HP and vasculitis has been discussed in the medical literature. HP may be one feature of multiorgan involvement in ANCA-associated disease, and the association between silica dust exposure and HP should be considered, as with other forms of ANCA-associated vasculitis.Abbreviations BUN Blood urea nitrogen - CSF Cerebrospinal fluid - HP Hypertrophic pachymeningitis - MPA Microscopic polyangiitis - MPO-ANCA Myeloperoxidase antineutrophil cytoplasmic autoantibody - RF Rheumatoid factor - SLE Systemic lupus erythematosus - WG Wegeners granulomatosis     

A case of idiopathic hypertrophic cranial pachymeningitis manifested only by positive rheumatoid factor and abnormal findings of the anterior falx

In this report, we present a rare case of a 52-year-old man with a unique form of hypertrophic pachymeningitis involving the anterior part of the falx and who was positive for rheumatoid factor. The clinical symptom was only headache, without any cranial nerve palsies or ataxia. Diagnosis was made by gallium scintigraphy and magnet resonance imaging but was not confirmed by dural biopsy. Treatment with corticosteroid alone was extremely effective for him, while in most cases hypertrophic pachymeningitis recurs or progresses despite the treatment.     

45例原发性肥厚型心肌病临床资料分析

对45例原发性肥厚型心肌病（HCM）的临床、心电图、超声心动图资料和其中16树冠状动脉及左室造影资料进行分析，结果显示患者年龄、病程和临床表现差异较大，ECG检查主要表现为左侧胸前导联T波倒置（71％），S－T段下移（71％），左室肥厚或高电压（44．4％）。超声心动图检查示以室间隔非对称性肥厚为主（93．3％），心脏收缩功能正常甚至超常（左心室射血分数56％～86％平均70．5％±10．5％），而有舒张功能的损害（44．4％病例超声心动图二尖瓣口流速曲线峰值A：E＞1）。7例心尖HCM主要表现为心绞痛，ECG表现类似其他非心尖HCM，但其左室肥厚或高电压发生率更高（P＜0.01），T波倒置更深（P＜0．05），超声心动图必要时结合心导管左室造影可明确诊断。1例兼有心肌肥厚和心脏扩大者，心肌收缩功能减退，临床经过凶险。     

13例嗜酸性肉芽肿性多血管炎临床特点观察

目的 探讨嗜酸性肉芽肿性多血管炎(EGPA)的临床表现、病理及影像学特征,以提高临床诊治水平.方法 回顾性分析2006年1月-2021年4月于解放军总医院确诊的13例EGPA患者的临床资料,包括基本信息、临床表现、受累器官情况、血液学及影像学检查、病理检查和治疗、随访情况.结果 13例EGPA患者中男7例,女6例;年龄...     

32例AIDS伴发中枢神经系统感染病人临床分析

目的分析艾滋病(AIDS)住院病人中,中枢神经系统感染的发病率、疾病谱及临床特点。方法对2009-2011年,在北京佑安医院住院治疗的AIDS伴发中枢神经系统感染病人的临床资料进行回顾总结,对其临床表现、诊治、转归等进行分析。结果 502例AIDS住院病人中,伴发中枢神经系统感染者32例,发生率6.4%。主要症状包括头痛23例(71.9%)、发热23例(71.9%)、呕吐20例(62.5%)、脑膜刺激征12例(37.5%)、肢体活动障碍9例(28.1%)、意识障碍7例(21.9%)等。这些病人的CD4细胞中位数为31/mm3,其中,≤100/mm3者27例,占总数的84.4%。诊断包括:隐球菌脑膜脑炎13例(40.6%),弓形体脑病10例(31.3%),结核性脑膜炎5例(15.6%),真菌性脑炎3例(9.4%),巨细胞病毒(CMV)脑炎1例(3.1%)。最终,32例病人中有22例(68.8%)治愈出院,5例(15.6%)死亡,5例(15.6%)未愈出院。结论 AIDS病人中枢神经系统感染以发热、头痛、呕吐、脑膜刺激征阳性等症状体征比较多见,主要发生于CD4细胞较低的病人,且病死率高,在临床中应引起重视。