肥厚性硬脑膜炎1例报道及文献复习

目的探讨肥厚性硬脑膜炎的临床表现、影像学特征及治疗。方法报道本院1例患者的临床资料并复习相关文献。结果本例患者表现为多组颅神经麻痹,头部MRI示局部硬脑膜异常增生并强化,激素治疗后好转,激素减量后病情再次复发,加用硫唑嘌呤后病情得以控制。结论肥厚性硬脑膜炎以慢性头痛、多组颅神经麻痹及小脑性共济失调为主要临床表现;头颅MRI可见特征性的硬脑膜肥厚并强化改变;激素治疗的基础上加免疫抑制剂可防止病情复发。     

经病理确诊的肥厚性硬脑膜炎一例

1病例报告患者男,74岁,主因"间断性头痛2年,加重20 d"入院。2年前出现间断头痛,以双颞部和顶部为主,呈搏动性,无恶心、呕吐,无肢体活动障碍,未经诊治。近20 d头痛加重,伴耳鸣,止痛药无效,就诊当地医院行头颅MRI示脑膜、小脑幕、大脑镰广泛均匀增厚、异常强化(图1)。为进一步诊治转至北京友谊医院。患者双耳混合性耳聋1年,双眼白内障人工晶体植入术后5年,双眼青光眼病史4年,双目失明5个月,既往有原发性高血压病史2年。起病     

特发性肥厚性硬脑膜炎

<正> 肥厚性硬脑膜炎属罕见疾病。以硬脑膜弥漫性增厚为特征性病变,其病因多种多样,涉及多个学科。少数病例无明确病因,称为特发性肥厚性硬脑膜炎。国内仅有数例报道少数可引起低颅压,本文就我们近年来诊断的4例报告如下。     

肥厚性硬脑膜炎1例报道及讨论

目的探讨肥厚性硬脑膜炎的临床、影像学、病理学特征及治疗。方法分析1例此病患者的临床资料。结果本例患者临床表现为慢性头痛、多颅神经麻痹等;头部MRI示局部硬脑膜异常强化灶;脑膜病理检查显示炎症改变,有多量浆细胞浸润,纤维组织增生。皮质类固醇激素治疗后疗效显著。结论肥厚性硬脑膜炎多以慢性头痛和多颅神经受累为主要临床表现;MRI可见特征性的硬脑膜强化改变;病理学检查是确诊的主要依据。     

肥厚性硬脑膜炎1例

肥厚性硬脑膜炎（hypertrophic cranial pachymeningitis,HCP）属罕见疾病,以局灶性或弥漫性硬脑膜肥厚为病理学特点,临床主要表现为慢性头痛,常伴有多组脑神经损害、局灶性脑损害。现将我科诊治的1例此类病例报道如下。     

肥厚性硬脑膜炎2例报道及文献复习

肥厚性硬脑膜炎(HCP)是一种少见的中枢神经系统慢性无菌性炎性疾病。以头痛为主要临床表现,多伴有颅神经损害。本文复习近5年的有关的病例报道,并结合本人经治2例患者。提示肥厚性硬脑膜炎是一种容易误诊,临床和影像表现特殊,激素为其主要治疗手段的疾病。     

肥厚性硬脑膜炎

肥厚性硬脑膜炎是以颅内硬脑膜弥漫性增厚和纤维性炎症过程为特征的一种少见疾病,分为特发性和继发性,主要表现为头痛、多颅神经麻痹、偏瘫及癫痫发作等。头颅MRI可见硬脑膜局部或弥漫性增厚,增强扫描可见强化。皮质类固醇激素和免疫抑制剂治疗有效,如出现脑实质或神经受压症状,多需手术治疗。本文复习文献,对HP近年研究进行综述。     

特发性肥厚性硬脑膜炎临床及病理特点(附3例报道)

目的探讨特发性肥厚性硬脑膜炎(IHCP)的临床、影像学、病理表现及治疗。方法回顾性分析3例经影像诊断(其中2例经病理活检证实)的IHCP患者的临床资料,总结其临床表现、实验室检查、影像学表现和治疗特点。结果 3例患者均有慢性偏头痛样头痛及脑神经麻痹表现,2例病程有复发和缓解。2例红细胞沉降率加快、C反应蛋白增高,其中1例类风湿因子升高,另1例抗核抗体阳性。脑脊液蛋白3例均升高。头MRI强化均可见硬脑膜增厚,部位与脑神经麻痹相关。糖皮质激素单用或联合环磷酰胺治疗有效。结论 MRI强化对IHCP诊断有特异性,脑膜活检可确诊该病。糖皮质激素联合免疫抑制剂对IHCP复发治疗有效。     

耳源性肥厚性硬脑膜炎1例

肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis, HCP)是以颅内硬脑膜肥厚为特征的一种慢性无菌性炎性疾病,临床上罕见,临床表现复杂多样,易误诊,应予重视.现报道1例如下.     

特殊的肥厚性硬脑膜炎2例报告并文献复习

目的分析肥厚性硬脑膜炎(HCP)的临床特点,提高对该病的认识。方法对2例特殊的HCP患者的临床资料结合文献进行回顾性分析。结果 2例HCP患者均以头痛起病,其中1例初诊为静脉窦血栓形成,经治疗未见好转;另1例逐渐出现面神经麻痹和硬膜下积液,综合分析临床特点、影像学之后,最终确诊为HCP。经激素治疗后取得良好疗效。结论 HCP患者可发生静脉窦血栓形成和硬膜下积液。HCP少见,临床缺乏特征性,易被漏诊、误诊,应引起临床医生重视。     

肥厚性硬膜炎8例分析及文献复习

目的探讨肥厚性硬膜炎的发病机制、临床特点、脑脊液和影像学改变及治疗。方法回顾性分析8例肥厚性硬膜炎患者的临床资料并复习文献。结果 8例患者发病年龄为43～78岁,6例患者均有头痛,2例患者以头晕为主要症状,6例患者存在脑神经受累表现,其中舌咽神经、迷走神经受累4例。血生化检查红细胞沉降率增快5例。腰穿脑脊液检查示压力增高4例,蛋白、细胞数增高;8例患者均未查到结核杆菌、隐球菌、瘤细胞。头MRI及增强扫描示广泛硬脑膜增厚强化。经糖皮质激素治疗后头痛缓解,脑神经受累症状部分好转。结论肥厚性硬膜炎病因复杂,临床多表现为头痛,脑神经易受累,头颅MRI增强发现典型的影像学改变有利于临床诊断,糖皮质激素治疗有效。     

肥厚性硬脑膜炎的临床、病理和影像学特点 (附1例报告)

目的 研究肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)的临床表现、病理和MRI特征.方法 对1例HCP患者的临床、病理和MRI检查资料进行回顾性分析并总结其特点.结果 HCP多慢性起病,临床以头痛和多颅神经麻痹为主要表现.MRI可见受累硬脑膜T1相呈等或略低信号,T2相呈高信号,增强扫描后增厚的硬脑膜明显均匀强化,硬脑膜病理检查可见纤维组织内炎症细胞浸润.激素治疗有效,MRI复查硬脑膜变薄,累及范围缩小,强化减轻.结论 HCP以头痛、多脑神经受累为主要临床表现.MRI扫描可见特征性的硬脑膜强化改变.病理学检查是确诊依据.     

肥厚性硬膜炎的临床与影像学特点分析

目的总结分析肥厚性硬膜炎的临床特点及影像学特征。方法回顾性分析2005年11月~2012年8月来收治的肥厚性硬膜炎10例临床资料。结果 10例患者中7例为HCP(hypertrophic cranial pachymeningtitis,肥厚性硬脑膜炎),1例为HSP(hypertrophic spinal pachymeningtitis,肥厚性硬脊膜炎),2例为HP(hypertrophic pachymeningitis,肥厚性硬膜炎)。男性7例、女性3例,年龄40~78岁,均为慢性起病,1例伴有系统性血管炎,1例患者20 y前曾行额窦骨瘤清除术,2例伴有双侧乳突、中耳炎,1例伴双侧乳突炎及上颌窦炎。7例HCP与2例HP患者均有头痛,其中2例伴有发热、1例患者出现抽搐、7例伴有脑神经受累、1例患者有共济失调;该例HSP患者症状为双下肢麻木无力,二便急,性功能下降。对9例患者行腰穿检查,其中蛋白增高者6例,细胞数增高者4例。对7例HCP与2例HP患者行颅脑与颈髓MRI平扫+增强检查,均见硬脑膜强化,小脑幕、大脑镰、颅底、颞部、鞍上池、小脑蚓等部位均可受累,其中以大脑镰、小脑幕强化最为明显,2例并存颈椎管内硬脊膜增厚强化,1例HSP行腰椎增强MRI,可见L5-S1硬脊膜增厚强化。对7例HCP与2例HP患者给予激素治疗1 w后头痛与脑神经麻痹症状明显好转,1例HSP患者接受激素治疗后脊髓受压症状及尿便障碍稍有好转。结论 HCP与HP患者主要表现为头痛及脑神经麻痹,HSP患者主要表现为神经根痛及脊髓受压症状。头部及脊髓MRI检查具有特征性,激素治疗临床效果满意。     

Hypertrophic pachymeningitis: case report

Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.     

Idiopathic hypertrophic cranial pachymeningitis mimicking multiple meningiomas: case report and review of the literature

A case of idiopathic hypertrophic cranial pachymeningitis with an unusual and misleading manifestation is reported. Computed tomography scan, angiographic and magnetic resonance imaging findings were suggestive of multiple meningeal neoplasms and a correct diagnosis was made only after meningeal biopsy. This 44-year-old patient had a previous history of an ill-defined systemic disorder associating episcleritis, erythroderma nodosa and multiple peripheral arthritis. We review previous reports of idiopathic cranial pachymeningitis with emphasis on radiological investigation techniques, histopathology and possible dysimmune mechanisms of pathogenesis. Received: 28 January 1997 / Accepted: 19 March 1997     

肥厚性硬脑膜炎的临床表现及影像学特征

目的探讨肥厚性硬脑膜炎的临床表现及影像学特征。方法通过4例肥厚性硬脑膜炎的病例报告及相关文献资料的临床表现及影像学特征来进行总结和讨论。结果肥厚性硬脑膜炎主要表现为头痛、脑神经麻痹,MRI表现为硬脑膜增厚呈线条状或斑块状,增强扫描后肥厚的硬脑膜强化。结论肥厚性硬脑膜炎可表现为多种临床过程,MRI表现较具特征性,结合临床表现有利于肥厚性硬脑膜炎的早期诊断。     

脑桥出血后继发肥大性下橄榄核变性临床和影像学特点及文献回顾

目的 探讨脑桥出血后继发肥大性下橄榄核变性（hypertrophic inferior olivary degeneration,HOD）的临床及影像学特点。 方法 回顾分析2009年12月～2013年3月北京航空总医院神经内科和北京天坛医院神经内科住院的3例HOD患者的临床及影像资料,3例患者均为男性,表现为肢体不自主多动、腭肌阵挛、走路不稳等锥体外系症状,均完成颅脑计算机断层扫描（computed tomography,CT）或磁共振成像（magnetic resonance imaging,MRI）检查。 结果 3例患者既往均有脑桥出血史,其中患者1和2为高血压性脑桥出血,患者3为外伤后脑桥出血,临床症状距脑桥出血的时间分别为8个月、12个月和14个月。3例均以锥体外系症状为主要表现,患者1表现为面下颌、躯干、四肢不自主扭转抖动;患者2仅有言语不清及腭肌阵挛;患者3表现为头晕、走路不稳,查体偏侧肢体共济失调。患者1和2出血部位位于脑桥双侧被盖部,患者3位于脑桥左侧被盖部,3例病变部位均为双侧下橄榄核腹外侧孤立局限性病灶。患者1口服氯硝西泮联合丙戊酸钠1周、患者2单纯口服氯硝西泮1周后症状缓解,患者3应用激素治疗2周后症状有所好转,但体征改变不显著。 结论 HOD可由脑桥出血继发,脑桥出血治疗病情稳定后若出现锥体外系症状需注意继发HOD的可能。氯硝西泮和丙戊酸钠对症状控制可能有效,激素疗效不确切。     

Aggressive intracranial and extra‐cranial epithelioid hemangioendothelioma: A case report and review of the literature

Epithelioid hemangioendothelioma is a rare tumor of intermediate malignancy. Twelve intracranial cases have been well documented. We report an intra‐ and extra‐cranial single mass epithelioid hemangioendothelioma. We describe the case of a 27‐year‐old male who presented a left temporal and retroauricular pain and an intra and extra‐cranial mass extending to the left infratemporal fossa. The neoplasm was isointense with gray matter in both T1 and T2‐weighted images, showing marked nodular gadolinium enhancement. About 90% of the tumor was surgically removed. The neoplasm was fibrous, well defined and arose from the left temporal artery branches. Recovery was uneventful. The histological diagnosis was epithelioid hemangioendothelioma. Enhanced magnetic resonance scan was repeated at 3 and 6 months after surgery, showing an increasing mass volume extending to the surrounding tissues, including intracranially, infratemporal fossa and left orbit. The patient died 8 months after the initial diagnosis. Epithelioid hemangioendotheliomas can be very aggressive tumors, presenting initially at an intra‐ and extra‐cranial location, due to its fast growth. From our review of the literature, they seem to have an equal gender distribution (M : F = 7 : 6) and tend to affect people under the age of 30 (10/13). We report a unique and extremely aggressive tumor. The rarity of reports and the continuous spectrum of differentiation, ranging from borderline to highly malignant are obstacles to identifying initial therapeutic protocols and the adjunctive therapy after surgery.     

Idiopathic hypertrophic pachymeningitis - case report and literature review

Idiopathic hypertrophic pachymeningitis (IHPM) is a rare pathological state, with still unclear aetiopathogenesis. We present a case of a 63-year-old woman with cranial variety of that disease. The manifestations of the disease included headaches, paresis of VI, IX, X nerves and cerebellar ataxia. The disease was diagnosed with magnetic resonance imaging (MRI) and histopathological assessment of the pachymeninx biopsy specimen. The MRI revealed significant thickening of the cranial base pachymeninx, compressing the pons and medulla oblongata. MRI examinations could be misinterpreted as extensive meningioma of the skull base. Dura mater biopsy revealed however inflammation with abundant lymphocytic infiltrations. Clinical improvement was obtained after the application of corticosteroids. We noted the subsidence of all symptoms of the disease, as well as radiological improvement, manifested through substantial regression of the described changes in the pachymeninx. The patient has been presented in the context of 65 cases of idiopathic hypertrophic pachymeningitis, described in the literature of English-speaking countries in the last five years. Recently, the importance of the autoimmunogenic background of IHPM has been underlined. In that respect IHPM has become an interdisciplinary problem. Its diagnosis and treatment requires not only radiologists, neurologists, pathomorphologists and neurosurgeons, but also specialists in internal medicine, including immunologists, allergologists and rheumatologists as well - in other words, physicians that rarely take part in the processes of diagnosing and treating intracranial pathologies.     

MPO-ANCA相关性肥厚性硬脑膜炎合并肉芽肿性血管炎临床分析

目的 探讨髓过氧化物酶抗中性粒细胞胞质抗体(MPO-ANCA)相关性肥厚性硬脑膜炎(HCP)合并肉芽肿性血管炎(GPA)的临床特点、治疗及预后.方法 回顾性分析2016-06—2020-06郑州大学第一附属医院神经内科住院治疗的MPO-ANCA相关性HCP合并GPA患者的临床资料.结果 5例患者均为亚急性或慢性起病,病...