肥厚性硬膜炎的临床和影像学特点

目的 探讨肥厚性硬膜炎(HP)的临床及影像学特征.方法 回顾性分析10例HP患者的临床资料.结果 10例患者以亚急性或慢性起病,8例患者继发于感染等自身免疫性疾病.以头痛(8例)或复视(2例)为首发症状.主要临床表现为头痛9例;脑神经麻痹7例;癫痫、共济失调、双下肢无力伴针刺觉减退各1例.MRI示硬膜异常肥厚,呈长或等T1、短T2信号;MRI增强示增厚的硬脑膜明显强化,呈条带状或结节状,不深入脑沟脑回.9例予以皮质类固醇激素治疗,头痛症状均缓解,遗留周围性面瘫4例,视物及听力障碍各1例.结论 HP多为继发性,以头痛及脑神经麻痹为主要症状,MRI平扫及强化可见条带状或斑块状硬膜肥厚,可累及硬脑膜和/或硬脊膜,糖皮质激素治疗有效.     

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。     

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。     

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。     

肥厚性硬脑膜炎的临床、影像学及病理学特征(附1例报告)

目的 探讨肥厚性硬脑膜炎患者的临床、影像学及病理学特征.方法 回顾性分析1例此病患者的临床资料.结果 本例患者临床表现为头痛、双眼视力下降、眼球运动障碍、面神经瘫痪及声音嘶哑等;头部MRI示硬脑膜异常强化灶,以大脑镰、小脑幕等处为主;硬脑膜病理检查可见有慢性炎症改变,有多量浆细胞浸润,纤维组织浸润、增生、玻璃样变性,肉芽肿形成.皮质类固醇激素治疗后疗效显著.结论 肥厚性硬脑膜炎以头痛和多脑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;病理学检查有硬脑膜慢性炎症改变,是确诊的主要依据.     

特发性肥厚性硬脑膜炎临床、实验室检查及影像学特点分析

目的分析6例特发性肥厚性硬脑膜炎(IHP)患者的临床特点及辅助检查特征。以提高对特发性肥厚性硬脑膜炎的认识。方法回顾性分析并总结2014年1月至2017年11月在河南省人民医院神经内科就诊的6例临床诊断为特发性肥厚性硬脑膜炎(IHP)患者的临床、实验室检查结果及影像学特点。结果 6例患者主要临床表现为头痛、颅神经受累、癫痫、精神行为异常和共济失调。实验室检查血沉(ESR)、C反应蛋白(CRP)及脑脊液细胞和蛋白等炎性指标增高。磁共振影像表现为对称或不对称的硬脑膜T1加权等和(或)低信号,T2加权低信号,增强有强化。所有患者均给予激素冲击治疗,预后良好。结论 IHP以头痛及颅神经受累为主要表现,实验室检查炎性指标的升高及影像学硬脑膜增厚强化均有助于临床医师诊断及识别本病。     

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。     

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。     

肥厚性硬脑膜炎的临床、影像学及病理学特征

目的 探讨肥厚性硬脑膜炎(HCP)患者的临床、影像学及病理学特征.方法 对本院1例HCP患者的临床资料及文献报道的77例HCP患者的资料进行回顾性分析.结果 78例HCP患者临床表现均可见慢性头痛,多组脑神经损害;其次为精神异常(10.3%),共济失调(9.0%),癫疒间发作(6.4%),偶见偏瘫及闭经泌乳;74例(94.9%)患者以头痛为首发症状,早期易被误诊为蛛网膜下腔出血、低颅压性头痛及静脉窦血栓形成等.MRI可见大脑镰和(或)小脑幕等处硬脑膜局部或弥漫性肥厚,增强扫描可见强化;病理学表现为硬脑膜纤维组织明显增生,伴炎性细胞浸润;皮质类固醇治疗有效.结论 HCP以头痛及多组脑神经受损为主,临床表现多样,影像学可见大脑镰和小脑幕等处硬脑膜肥厚,MRI检查对诊断有重要意义.     

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和影像学特征

目的 通过对6例特发性肥厚性硬脑膜炎临床资料的分析及文献回顾,总结该病的诊断及治疗方法,以期降低其误诊误治率. 方法 结合文献对2005至2006年我科收治的6例该病患者的临床特点、影像学表现及随访结果进行分析. 结果 特发性肥厚性硬脑膜炎均存在不同程度的免疫功能异常.头痛几乎是首发、必发的症状,大多为隐匿起病,反复发生或迁延不愈.患者可出现神经系统受损的各种表现.本病的头颅MRI可见小脑幕和(或)大脑镰等处的硬脑膜局部或弥漫性增厚,增强扫描可见强化.皮质类固醇治疗有效. 结论 特发性肥厚性硬脑膜炎可能是一种自身免疫性疾病.本病临床表现多样,应注意与其它疾病鉴别.应重视头颅MRI的诊断意义.     

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.     

Idiopathic hypertrophic cranial pachymeningitis.

Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory process of unknown origin that can cause neurological deficits owing to thickening of the dura. Patients with this condition commonly present with cranial neuropathy accompanied by localized headache. The clinical features, neuroimaging findings, histopathological features and treatment outcomes for three patients with this condition are reported here. The first patient presented with subacute dull headache in the left temporal area followed by left abducens nerve palsy. The second patient suffered from a cranial nerve IX-XII lesion accompanied by an occipital headache and the third patient presented with left optic neuropathy and mild headache in the frontal area. In all patients, MRI of the brain revealed prominent dural thickening, and histopathological study of the dura revealed chronic inflammatory cell infiltration. Combined therapy with corticosteroid and immunosuppressive drugs was effective, resulting in almost complete resolution of the symptoms and signs, except for visual impairment in one patient.     

Clinical usefulness of thallium-201 single-photon emission CT in the patient with hypertrophic cranial pachymeningitis]

We report a 54-year-old female with rheumatoid factor-positive hypertrophic cranial pachymeningitis. At age of 51 years she developed headache, hearing loss, right vagal nerve palsy, and right accessory nerve palsy. MRI revealed thickening and gadolinium-enhancement of the cranial dura mater. The initial symptoms significantly improved with corticosteroid therapy. Two years later, she presented with severe headache and neck pain. Although gadolinium-enhanced MR images failed to show any change compared with those before recurrence, 201Tl single-photon emission CT (SPECT) showed a remarkable accumulation of thallium-201 in the dura mater. Furthermore, the abnormal uptake of thallium-201 returned to normal after treatment with corticosteroid. 201T1-SPECT was a useful tool for the evaluation of disease activity in the patient with hypertrophic pachymeningitis.     

Idiopathic hypertrophic pachymeningitis

Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. It is a fibrosing inflammatory process that involves the dura mater. Herein are described 14 patients with idiopathic hypertrophic pachymeningitis; their clinical, laboratory and radiological findings, as well as their treatment, are analyzed. Neuropathological findings of six cases including two autopsied cases are also presented. The main clinical features were headache and cranial nerve palsies. Many patients had mild to moderate elevation of C‐reactive protein, and three patients had perinuclear antineutrophil cytoplasmic antibody. The CSF in most cases showed inflammatory changes. Neuroimaging studies revealed diffuse or localized thickening of the dura, and MRI findings were key to diagnosis of this disorder. The clinical course was chronic. All patients were treated with corticosteroid and improved intially, but half of them experienced relapses. Two patients received surgical intervention. Pathological examination in two autopsied cases revealed diffuse thickening of the dura, especially in the posterior part of the falx cerebri and the tentorium cerebelli. Microscopic examination of the dura showed dense fibrosis with inflammatory cell infiltration composed mainly of lymphocytes. The cell infiltration was marked at the surface of the dura mater. One case had necrotizing vasculitis of the small arteries located in the dura and the cerebral surface. There were no giant cells, caseation necrosis, or epitheloid granuloma. Four patients underwent biopsy of the dura, and the pathological study showed non‐specific inflammatory changes. The relationship of idiopathic hypertrophic pachymeningitis with connective tissue disease or vasculitis syndrome is discussed.     

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。     

Two cases of cranial pachymeningitis presenting only severe continuous headache or convulsion]

In cranial pachymeningitis, headache, cranial nerve palsies, and ataxia were the most common clinical manifestations. We reported two rare cases presenting only headache or convulsion as a main symptom. A 66-year-old man was suffering from severe continuous headache for years with no other clinical symptoms. Laboratory data revealed positive RA test and elevated RAPA. With oral steroid therapy, his headache was subsided. A 39-year-old woman visited us with convulsive attacks as only symptom. Biopsy of the thickened dura revealed granulated epithelioid cells without central necrosis. With antituberculosis therapy, hypertrophy of dura, as well as the symptom, was disappeared. It is difficult to diagnose pachymeningitis when patients manifest only headache or convulsion. We suggest to carry out Gd-enhanced MR scan in these cases.     

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.