手术后继发性肥厚性硬脑膜炎2例

目的探讨继发性肥厚性硬脑膜炎(SHCP)的诊治方法。方法回顾性分析2例手术后SHCP病人的临床资料,总结其临床表现、脑脊液检查指标、影像学表现和治疗特点。结果 2例均表现为偏头痛样头痛,合并脑神经麻痹1例。结合MRI和其他实验室辅助检查诊断为SHCP 1例,经病理检查确诊为SHCP 1例。抗生素疗效不佳,经甲泼尼龙静脉滴注后症状缓解。结论手术后继发SHCP临床少见,头颅MRI有助于诊断,脑膜活检可确诊该病。抗生素疗效不佳,糖皮质激素治疗有效,手术对于延缓脑神经损伤可能有一定作用。     

特发性肥厚性硬脑膜炎的临床、病理特点及治疗体会

目的探讨特发性肥厚性硬脑膜炎（IHCP）的临床、病理特点并介绍治疗体会。方法收集4例IHCP患者的临床、影像和病理资料,总结其临床特点及治疗方法。结果IHCP主要临床表现为慢性头痛、多脑神经麻痹以及癫痫发作,头颅MRI增强检查可见局部脑膜增厚,脑膜病理检查显示肉芽组织样增生。甲基泼尼松龙联合免疫抑制剂治疗IHCP效果好。结论IHCP多以慢性头痛起病,头颅影像可见硬膜肥厚,糖皮质激素加免疫抑制剂治疗有效。     

经病理确诊的特发性肥厚性硬脑膜炎1例报道并文献复习

目的 探讨特发性肥厚性硬脑膜炎的临床表现、影像学特征、诊断及治疗。方法 报道本院1例经病理确诊的特发性肥厚性硬脑膜炎患者的临床资料并复习相关文献。结果 本例患者表现为慢性反复头痛,头颅磁共振增强扫描示左侧小脑幕异常增生并强化,病理活检提示大量慢性炎症细胞浸润,经过激素冲击治疗头痛缓解,后续小剂量激素联合免疫抑制剂甲氨蝶呤口服,临床预后良好。结论 特发性肥厚性硬脑膜炎病因复杂,多以慢性头痛、多组脑神经麻痹及小脑性共济失调为主要临床表现; 头颅MRI可见特征性硬脑膜肥厚及强化表现; 临床需与多种颅内疾病相鉴别,病理活检可确诊; 激素治疗基础上联合免疫抑制剂可防止病情复发。     

脑神经胶质瘤病的诊断和治疗

目的 探讨脑神经胶质瘤病的临床特征及其诊断和治疗。方法 回顾性分析9例经开颅手术或定向活检证实为脑神经胶质瘤病的临床表现。影像学资料及治疗效果。结果 9例均有颅高压表现。CT扫描共发现23个病灶。其中病灶累及3个脑叶5例。累及2个脑叶4例，CT平扫病灶呈高密度5个。等密度5例。低密度13例。大多数病灶强化不明显，瘤周轻中席子工水肿，4例行MRI检查发现胼胝体肥大2例。9例均获得半年以上随访。结论 CT扫描易误诊为转移瘤，MRI扫描对脑神经胶质瘤病的诊断有一定的价值。确诊依赖于病理检查。手术切除程度是决定预后的重要因素。术后分割放疗是控制肿瘤生长及延迟复发的重要辅助治疗手段。     

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9例的临床表现及影像学特征分析

目的分析肥厚性硬脑膜炎患者的临床表现、影像学特征、治疗及预后,以提高对该病的认识。方法收集我院2012年2月至2015年7月收治的肥厚性硬脑膜炎患者9例,进行回顾性分析。结果 9例患者均慢性起病,平均病程10.3 m(1 m~3 y),8例以慢性头痛为首发症状,1例以精神异常为首发症状,6例合并脑神经受损。实验室检查示9例非特异性炎性指标升高,6例脑脊液检查压力升高,7例蛋白升高。头部磁共振成像(MRI)以小脑幕、大脑镰等处硬脑膜局限性或弥漫性肥厚为主,T1加权像表现为等信号,T2加权像表现为等信号或低信号,增强明显强化,呈线性或结节性异常强化的特征性表现,治疗后复查可见硬脑膜增厚变薄或消失,强化减轻。9例均经糖皮质激素治疗,6例明显好转,3例无效加用免疫抑制剂后好转。结论肥厚性硬脑膜炎常表现为慢性头痛及脑神经受损,MRI增强可见特征性表现,糖皮质激素治疗可明显缓解症状,难治者可加用免疫抑制剂治疗。     

原发性中枢神经系统血管炎2例报道并文献复习

目的报道2例原发性中枢神经系统血管炎(PACNS)患者的临床、影像学和病理特点。方法回顾分析2例经病理活检确诊的PACNS患者,总结其临床表现、影像学和病理特点。结果 2例患者均以局灶性神经系统症状起病,以右侧肢体无力伴语言障碍为主要表现,数月内持续进展。2例患者头颅MRI均提示颅内多发水肿病灶伴渗血,增强后有不规则强化,动态MRI随访见病灶均呈持续进展。2例患者血清免疫指标以及血管炎指标均阴性,经脑组织活检病理排除了肿瘤、真菌、结核和病毒感染,证实为PACNS。结论 PACNS以中枢神经系统局限性受损为主要特点,症状常持续性进展,影像学检查有临床诊断意义,但脑组织活检和排除其他疾病是诊断本病的关键。     

立体定向活检术在肥厚性硬脑膜炎诊断中的应用(附11例报告及文献复习)

目的分析肥厚性硬脑膜炎的临床特点,探讨立体定向活检术在肥厚性硬脑膜炎诊断中的价值。方法收集2015年1月至2019年12月解放军总医院第六医学中心神经外科收治的11例肥厚性硬脑膜炎患者的临床资料,对患者的临床表现、实验室、影像学及病理检查资料进行回顾性分析。结果 11例患者中,女性6例、男性5例,发病年龄为31～66(47.2±9.2)岁。主要临床表现:头痛10例、视力下降7例、眼球运动障碍3例、听力下降3例、癫1例、肢体活动不利1例和声音嘶哑1例。头颅MRI检查均可见不同程度硬脑膜强化,部分病例累及软脑膜。9例行开颅硬脑膜病变切除或活检术,2例行立体定向活检手术。2例病理检查符合IgG4相关性硬脑膜炎表现。结论肥厚性硬脑膜炎临床表现以头痛伴或不伴脑神经麻痹为主要症状,MRI为主要检查手段,活检手术对明确诊断有重要意义。     

肥厚性硬脑膜炎一例

目的提高对本病的认识,以减少误诊。方法报道1例肥厚性硬脑膜炎(HCP)患者的病史、症状、体征、血和脑脊液实验室检验、影像学(CT、MRI、SPET)及硬脑膜病理资料,并对治疗反应和1年随访结果进行总结。结果患者男性,61岁,主因两个半月来头晕、耳鸣、吞咽困难、声音嘶哑伴头痛、抽搐入院。既往有眼球摘除史。查体以左侧为主的多组脑神经受累的体征,脑脊液(CSF)白细胞、蛋白轻度增高,头颅MRI硬脑膜异常增厚和明显强化以及较典型的硬脑膜病理改变,对类固醇激素治疗反应良好,停药后复发特点,支持HCP的诊断。结论临床有本病的症状体征的患者要尽早做强化头颅MRI和硬膜活检,早期诊断和正确的治疗预后较好。     

Bickerstaff's脑干脑炎4例并文献复习

目的分析Bickerstaff脑干脑炎(BBE)的临床特点,以提高临床医生对其认识,提高诊治水平。方法对瑞金北院神经内科收治的4例BBE患者的临床资料进行回顾性分析,并综合相关文献,总结其临床特点、发病机制、治疗方法及预后。结果 4例患者均为急性起病,发病前有上呼吸道感染者2例,疱疹病毒感染1例,鼻窦炎1例。临床主要表现为急性眼肌麻痹(眼内肌麻痹3例,眼外肌麻痹1例),共济失调4例,意识障碍1例,腱反射亢进4例,病理征阳性2例,1例患者同时累及脑神经(面神经、前庭神经及听神经)。4例患者脑脊液检查及头颅MRI检查结果基本正常。2例行血清抗GQ1b抗体检查者均为阴性。4例患者使用糖皮质激素或联合免疫球蛋白治疗预后较好。结论 BBE的确诊主要依据临床表现、血清抗体、影像学及神经电生理作为辅助诊断,激素或激素联合免疫球蛋白治疗有效。     

Idiopathic hypertrophic cranial pachymeningitis

Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. The diagnosis of idiopathic hypertrophic cranial pachymeningitis was based on neuroimaging findings of thickened enhancing dura, exclusion of known causes and histopathologic findings compatible with nonspecific inflammation in the meningeal biopsies. Corticosteroid therapy was effective in all cases in inducing a complete or partial remission of the neurologic symptoms and signs. We describe the clinical, radiological and pathological features of idiopathic hypertrophic cranial pachymeningitis and discuss the relationship of this entity with other inflammatory fibrosclerotic disorders to explain the pathogenesis. A high index of suspicion, prompt confirmation of the diagnosis by meningeal biopsy, and early institution and long-term maintenance of steroid therapy may help to prevent irreversible neurologic sequelae, especially blindness.     

海绵窦海绵状血管瘤的影像特征和显微手术

目的 总结海绵窦海绵状血管瘤的诊断和手术治疗经验,探讨其影像特征和手术要点.方法 对2001年10月至2008年10月收治并手术切除的13例海绵窦海绵状血管瘤进行回顾性分析.病灶最大径3.5～6 0 cm,均经MRI证实和显微手术切除.结果 病变被全切除9例,大部切除3例,活检1例,无手术死亡.海绵窦神经保留情况:8例保留动眼神经;9例保留三叉神经第Ⅱ、Ⅲ支;其余均未能保留.结论 海绵窦海绵状血管瘤可以根据其MRI的特征性影像获得确诊.合适的手术人路、娴熟的显微外科技术是全切肿瘤的必备条件.海绵窦内神经的保留仍然是未能完全解决的问题.

Abstract：

Objective To analyze the imaging character and surgical therapy of cavernous hemangiomas in cavernous sinus ( CSHs) .Method From 2001 to 2008, 13 patients with CSHs were surgically treated in our department.The diameters of CSHs varied from 3.5 cm to 6.0 cm.Results All the patients were operated on with no mortality.Complete tumor removal was achieved in 9 cases, partial tumor removal in 3 cases, and biopsy in 1 case.Cranial nerve Ⅲ was preserved in 8 cases and cranial nerves V2,V3 in 9 cases.In the other cases, cranial nerves in cavernous sinus were sacrificed unavoidably.Conclusions CSHs can be diagnosed preoperatively according to MRI and can be removed successfully by experienced surgeons who are familiar with operative approaches and microsurgical technique.However,great efforts should be made to decrease postoperative cranial nerve deficits.     

Idiopathic hypertrophic cranial pachymeningitis: Three biopsy-proven cases including one case with abdominal pseudotumor and review of the literature

Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Dural biopsy is essential to exclude secondary causes of pachymeningitis. There is paucity of data on biopsied cases of HP. We report three biopsy-proven cases of idiopathic hypertrophic cranial pachymeningitis. All our patients had headaches and multiple cranial neuropathies; ataxia was seen in one patient. One patient had recurrent anterior and posterior cranial neuropathies, while one each had recurrent anterior and posterior cranial neuropathies. Two patients had profound irreversible mono-ocular visual loss. All of them showed prominent pachymeningeal thickening on imaging. Infarcts were seen in one patient, which have rarely been documented. All patients showed biopsy evidence of meningeal thickening and nonspecific chronic inflammation of the dura. The disease may have a remitting and relapsing course, and usually responds to steroids. Clinical improvement was excellent in two patients and modest in one on steroid therapy. All our patients required azathioprine during the course of therapy. Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier.     

Idiopathic hypertrophic cranial pachymeningitis with perifocal brain edema

Idiopathic hypertrophic cranial pachymeningitis (IHCP) consists of meningeal thickening due to chronic inflammation for which no cause such as infection, specific granulomatous disease, or malignancy is demonstrable. We present a case of IHCP with perifocal brain edema suggesting mass lesion with its magnetic resonance imaging (MRI) and pathologic findings. A 36-year-old woman was admitted to our hospital in August 2001 with a complaint of motor weakness in her left leg for 1 month. Magnetic resonance imaging with gadolinium revealed enhancement of the thickened dura mater and perifocal edema in the right frontoparietal region suggesting a mass lesion. Histological examination of the biopsy specimen revealed a dense fibrous cellular tissue. There was no obvious inflammatory infiltrate but in some areas between fibrous bundles one or two lymphocytes were detected. According to our knowledge, our patient is the second report in the literature of IHCP with focal edema causing a mass effect.     

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。     

Uncommon findings in idiopathic hypertrophic cranial pachymeningitis

Abstract.Background: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare, poorly understood, inflammatory disease, usually involving the dura mater of skull base, tentorium, and falx, and presenting with headache, progressive cranial nerve palsies, and cerebellar dysfunction.Patients and Methods: In four patients, the diagnosis of IHCP has been made on the basis of extensive clinical, and radiological investigation, and confirmed by dural biopsy in three patients. The clinical follow-up ranges from 24 to 120 months.Results : At diagnosis, all the patients complained of severe, progressively increasing headache, two had simple or complex partial seizures, but none had cranial nerve palsies. Two patients had electrophysiological evidence of axonal peripheral neuropathy, biopsy-proved in one of them. In all the patients, MRI showed linear or focal thickening of the dura mater of the tentorium and/or of the convexity, sparing the skull base. In one patient, MRI findings resembled chronic subdural hematoma. Dural biopsy demonstrated fibrosis and prominent CD4+ T-cells inflammatory infiltrate. Pachymeningitis was highly responsive to steroid therapy, as was the peripheral neuropathy. In three patients, temporary steroids withdrawal led to dramatic clinical worsening including status epilepticus in one.Conclusions : In the patients here reported, absence of cranial nerve impairment, seizures, MRI findings resembling chronic subdural hematoma, and association with polineuropathy were unusual findings of IHCP. Moreover, the type of inflammatory infiltrate, lacking in previous reported cases, suggests a probable pathogenetic role for cell-mediated immunity of unknown origin.     

Primary malignant meningeal melanomatosis: a clinical, radiological and pathologic case study

A 40 year-old woman with subacute headache and visual impairment was admitted. Neurological examination revealed meningismus, diminished visual acuity, bilateral sixth cranial nerve palsy, and papillary edema. Dermatologic examination was normal. The brain CT scan showed hydrocephalus and hyperdense edging around fissures and sulci. The CSF study showed an increased protein level, with persistently negative microbiologic and cytological studies. Prior to Gd-DPTA injection, the brain T1-WI MRI revealed leptomeningeal hyperintensity. A dark subpial substance became evident at cerebral biopsy. The histopatological diagnosis was diffuse leptomeningeal melanomatosis. This case report highlights the diagnostic value of the brain MRI findings in primary leptomeningeal melanomatosis, a rare pathologic condition diagnosed in most published cases only after necropsy. Meningeal T1-WI hyperintensity prior to contrast injection is not caused by sarcoidosis or meningeal carcinomatosis, lymphomatosis or gliomatosis.     

颅内结核结节的MRI分型与临床研究

目的研究颅内结核结节(ITN)的MRI分型和临床特点。方法回顾分析35例ITN病例的头颅MRI表现和临床特点,全部病例均根据临床表现、PPD试验、胸片、脑脊液检查以及手术病理或药物治疗随访等建立诊断。结果ITN磁共振成像主要表现为脑实质或脑池内大小不等的结节,根据其成像特点可分为肉芽肿型、干酪样型、脑膜炎型及弥漫性粟粒型。临床主要表现为颅内高压、癫、脑膜刺激征、脑神经损害和结核中毒症状等,抗结核药物治疗后26例痊愈或好转,9例改手术治疗。结论MRI对颅内结核结节具有较高的诊断价值,其分型可指导临床治疗。     

Case report of Wegener's granulomatosis presenting with multiple cranial nerve palsy and hypertrophic cranial pachymeningitis]

We report a 41-year-old man whose initial neurological symptoms are atypical of Wegener's granulomatosis. The patient was admitted because he developed left ocular pain, headache, bilateral visual loss and left abducens nerve palsy. He was initially diagnosed with optic neuritis at ophthalmological department and steroid therapy was started. Although steroid therapy led to rapid recovery of visual acuity and eye movement, he was readmitted for seizure. Two weeks later, a second seizure attack occurred, followed by palsy of the left side of cranial nerves II, III, IV, V and VI. Brain MRI showed focal thickening and enhancement of the dura mater over left frontal lobe, leading to a new presumptive diagnosis of idiopathic hypertrophic cranial pachymeningitis. Steroid therapy was resumed and the symptoms improved rapidly. As right hemiparesis developed during the clinical course, another brain MRI was obtained. T2-weighted image showed a high intensity area in the left portion of the pons. 14 months later, recurrent epistaxis suggestive of Wegener's granulomatosis appeared. A subsequent nasopharyngeal mucosa biopsy revealed a necrotizing granulomatous inflammation. A significant elevation of PR-3 ANCA was also noted. A definitive diagnosis of Wegener's granulomatosis was established. The initial presentation of this case was of multiple cranial neuropathies with no superior respiratory tract symptoms, which are typical of early stage Wegener's granulomatosis. In patients with various central nervous system symptoms and MRI evidence of hypertrophic cranial pachymeningitis, a thorough clinical workup of vasculitis syndrome including Wegener's granulomatosis should be considered.