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.     

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.     

Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen

Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. The condition is being reported more frequently owing to the use of cranial MRI. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. A variety of autoimmune and infectious diseases can result in this condition, which is labeled as idiopathic in the absence of any definite inciting factor. The diagnosis of hypertrophic cranial pachymeningitis is based on neuroimaging of thickened and enhancing dura mater. It can be defined pathologically on biopsy. A specific treatment is indicated in some cases of secondary hypertrophic cranial pachymeningitis. Mostly, treatment relies on corticosteroids and immunosuppressive agents. This review summarizes the current knowledge on causes, clinical presentation, diagnosis, and treatment of this disorder.     

Hypertrophic cranial pachymeningitis due to Aspergillus flavus.

A 59-year-old woman suffered from occipital headache and bilateral cranial nerve VII, VIII, IX, X, XI and right XII deficit after developing otitis media. Magnetic resonance imaging (MRI) showed a thickening of the dura mater which was enhanced by gadolinium-DTPA (Gd). Aspergillus flavus was identified from the culture of otorrhea. She was treated with miconazole, flucytosin and fluconazole, which resulted in an improvement of the clinical symptoms and a thinning of the Gd-enhanced lesions on MRI. This is the first case of hypertrophic cranial pachymeningitis caused by Asp. flavus infection.     

Two cases of hypertrophic cranial pachymeningitis associated with infection in the external auditory canal and paranasal sinus]

We here present two cases of hypertrophic cranial pachymeningitis exhibiting unique multiple cranial neuropathies, both of which were associated with otic and paranasal infections. Case 1: A 76-year-old woman developed headache after undergoing surgical dilatation of the external auditory canal, with subsequent development of a bacterial infection. Neurological examination reveled only bilateral hearing disturbance. MRI and CT scans demonstrated thickening of the dura mater and inflammatory granulation around the left cerebellar tentorium. Based on a diagnosis of hypertrophic pachymeningitis associated with previous infection, antibiotics were administered, followed by oral prednisolone therapy. This treatment relieved the headache and improved the MRI findings. However, 2 months later, the headache became worse and impaired movement of the soft palate, atrophy of the left side of the tongue, and atrophy of the sternocleidomastoideus muscle were noted. MRI revealed aggravated inflammatory changes around the left cerebellar tentorium and their expansion into the jugular foramen. Occlusive changes in the transverse and sigmoid sinuses were also seen. Case 2: A 78-year-old man developed bilateral visual loss, right frontal headache, and bilateral restriction of eye movement. He had been treated for phemphigus with prednisolone and azathioprine. MRI showed hypertrophic dura mater spreading continuously from the frontal base and ethmoid and frontal sinuses to the falx and right frontal lobe. Since Pseudomonas aeruginosa was cultivated in biopsy specimens from the dura mater, antibiotic agents were administered. The clinical symptoms resolved and MRI findings gradually improved.     

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.     

A case of hypertrophic pachymeningitis, resolved by antimicrobial therapy]

A 65-year-old woman with diabetes mellitus and chronic otitis media developed headache, fever, and hoarseness, all of which did not responded to the oral antibiotics. As stiff neck and lower cranial nerve palsies appeared, bacterial meningitis was suspected. Neurological examination revealed the right hearing disturbance, right recurrent laryngeal nerve palsy, left sternocleidomastoid muscle atrophy and bilateral tongue atrophy. The CSF examination revealed mild pleocytosis and elevated protein, but no bacterial organism was cultured from the CSF. CT scans showed bilateral mastoiditis, and the right mastoid process and a posterior part of the petrous bone were eroded, indicating the exposed bony structures to the posterior fossa. MRI scans demonstrated the thickening of the dura mater of the posterior fossa and the right cerebellar tentorium. This is a rare example of bacterial pachymeningitis of the posterior fossa, the clinical symptoms and MRI findings of which resolved solely by antimicrobial agents without corticosteroid.     

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.     

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。     

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.     

Progress in MR imaging: differential diagnosis of infectious and inflammatory diseases with MR imaging]

MR imaging is useful for diagnosis of infectious and inflammatory diseases. Diffusion weighted images (DWI) and fluid-attenuated inversion recovery (FLAIR) images revealed hyperintense signal changes in the anterior striata and cortices in patients with Creutzfeldt-Jakob disease, which were characteristic. In patients with nonherpetic and non-paraneoplastic acute limbic encephalitis, DWI and FLAIR images showed hyperintense changes in the hippocampi, amygdales, cingulate gyri, and occasionally claustrum, but not in the anterior and lateral temporal cortices, which were often involved in herpes simplex encephalitis. In ventriculitis, DWI and FLAIR images showed high signal intensity changes with niveau formation in the lateral ventricle, which indicated the presence of pus in the ventricle. In Japanese encephalitis, T2-weighted images showed high signal intensity changes in the thalami and substantia nigra, which were most often involved by Japanese encephalitis. In idiopathic cranial hypertrophic pachymeningitis, thallium-201 SPECT showed a remarkable accumulation in the abnormal dura matter. Furthermore, the abnormal uptake of thallium-201 returned to normal after treatment with corticosteroid. Thallium-201 SEPCT was useful for the evaluation of disease activity in patients with hypertrophic cranial pachymeningitis.     

P‐ANCA‐positive Wegener’s granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: Case report and review of literature

An autopsy case of hypertrophic pachymeningitis and multiple cranial neuropathies is reported. A 53‐year‐old woman with paraplegia and various neurological signs which developed over a 2 year period was diagnosed as having an epidural mass with thickened dura mater extending from the lower cervical to the thoracic spinal cord. In addition, bilateral episcleritis, blephaloptosis, and blindness of the right eye with various cranial nerve deficits were found to be caused by the mass lesions involving the paranasal sinuses, orbit, and the cavernous sinus. Perinuclear antineutrophil cytoplasmic antibody (p‐ANCA) was positive, but cytoplasmic antineutrophil cytoplasmic antibody (c‐ANCA) was negative by enzyme‐linked immunosorbent assay. The partially removed epidural mass with hypertrophied dura mater and biopsy of the paranasal lesions showed chronic granulomatous inflammation with vasculitis. The remaining lesions resolved with steroid therapy with remarkable neurological improvement. The positive p‐ANCA test, paranasal involvement, the report of a similar histopathological case and a review of the literature on granulomatous pachymeningitis suggest the presence of p‐ANCA‐positive Wegener’s granulomatosis with central nervous system involvement characterized by hypertrophic pachymeningitis and/or multiple cranial neuropathies.     

Hypertrophic pachymeningitis in an immunocompetent adult with positive Aspergillus DNA in the cerebrospinal fluid

A 42-year-old immunocompetent man presented with subacute onset unilateral headache and associated lower cranial nerve palsies. Cranial magnetic resonance imaging showed enhancing thickened tentorium cerebelli and subtentorial dura mater. Cerebrospinal fluid examination revealed lymphocytic pleocytosis and positive polymerase chain reaction assay of Aspergillus DNA. While on voriconazole treatment a progressive increase was noted in subtentorial pachymeningeal hypertrophy, which was excised because of critical compression of the medulla. The excision material showed extensive fibrosis, cellular infiltrates and no organisms. With combination therapy with anti-fungal agents and corticosteroids, pachymeningitis showed regression. We hypothesised that intact immune status and less burden of Aspergillus infection in this patient may have resulted in a chronic progressive hypertrophic pachymeningitis.     

Serial Neuroimaging in Tolosa‐Hunt Syndrome with Acute Bilateral Complete Ophthalmoplegia

Tolosa‐Hunt syndrome (THS) is a very rare, relapsing, and remitting painful ophthalmoplegia caused by nonspecific granulomatous inflammation in the cavernous sinus. To our knowledge, bilateral complete, simultaneous palsies of all 3 cranial nerves associated with extraocular movement have not been reported. We describe the first such patient with bilateral THS that responded quickly to corticosteroid therapy. A 54‐year‐old man presented with a periorbital and frontal headache with acute bilateral severe blepharoptosis and fixed eyes, which dramatically responded to corticosteroid therapy. He had diabetes mellitus type II. Brain MRI showed granulomatous inflammation in both cavernous sinuses and thickening of the surrounding dura mater of the cranial base, suggesting the coexistence of focal hypertrophic cranial pachymeningitis. Our experience indicates that steroid therapy with strict control of blood sugar should be considered in patients with THS complicated by diabetes. MRI is a valuable tool for serially monitoring the response of lesions to treatment in THS.     

Idiopathic hypertrophic pachymeningitis: Clinical,laboratory and neuroradiologic features in China

Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. Clinical, laboratory, neuroradiologic and therapeutic data from 12 patients with idiopathic hypertrophic pachymeningitis (IHP) from our department were retrospectively studied. There were four men and eight women with a mean age of 49 ± 15.3 years, and more than half of the patients (58%) were aged 40–60 years. Headache was the most common symptom, occurring in 92% of patients. Headache improved markedly and rapidly after glucocorticoid treatment. Optic nerve involvement was noted in seven patients (58%). C-reactive protein levels increased in 80% and the erythrocyte sedimentation rate increased in 71% of patients. Three patients were positive for autoantibodies, including antinuclear antibodies (ANA), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), anti-cardiolipin antibodies (ACA) and rheumatoid factor (RF). Cerebrospinal fluid showed inflammatory changes, and protein levels were low to moderately elevated. MRI revealed a thickened dura in all patients, and five patients (42%) were diagnosed with sinus stenosis/occlusion. IHP is a chronic inflammatory disorder of the dura with three groups of symptoms, namely headache, cranial nerve palsy and symptoms due to sinus stenosis/occlusion. However, IHP has different features in China in that it predominantly affects women and the age of onset is younger. Sinus stenosis/occlusion is relatively common in IHP patients in China.     

Secondary hypertrophic pachymeningitis in antiphospholipid syndrome

This report describes a rare case of hypertrophic pachymeningitis secondary to antiphospholipid syndrome in a 37-year-old woman. The patient had a history of antiphospholipid syndrome and developed hypertrophic pachymeningitis subsequently. Pathological examination of the dura mater showed not only fibrous thickening, the typical finding of hypertrophic pachymeningitis, but also thrombosis in the dural microvessels and T cell infiltration without B cell or plasma cell infiltration, suggesting cell-mediated immunity. The dural thickening spontaneously improved and did not deteriorate during corticosteroid therapy. The histological findings and spontaneous remission observed in this case might be characteristic of secondary hypertrophic pachymeningitis with antiphospholipid syndrome. Further investigations are necessary to elucidate the pathophysiology of this novel type of hypertrophic pachymeningitis.     

A case of Garcin's syndrome caused by pachymeningitis secondary to otitis media, responsive to antibiotic therapy]

A 55-year-old woman noticed progressive hearing loss, earache and tinnitus in the left side in December 1988, followed by dysesthesia of the left face and hypogeusia. Those symptoms did not respond to the otological treatment of otitis media. In addition, the left eyelid ptosis, double vision and dysphagia appeared in May 1989. On admission, cranial nerves from III to XII were affected exclusively in the left side. The cell counts and the protein levels in the CSF were elevated. The MRI of the head showed hypertrophic dura mater at the left base of the skull covering the temporal lobe. No bone destruction was found in CT. The biopsy revealed the thickened dura mater with microabscess containing Langhans giant cells and lymphocytes. A diagnosis of pachymeningitis was made. After the therapy with antibiotics and then with prednisolone for several months, cranial nerve disturbances disappeared except hearing loss with decrease in cell counts and protein levels of the CSF. The follow-up MRI after one year showed decrease in thickness of dura mater. The MRI, particularly using enhancement with Gd-DTPA, was useful to make a diagnosis of hypertrophic pachymeningitis. It is suggested that the administration of antibiotics should be considered in the treatment of the pachymeningitis even if the causative agents were not identified.     

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。