Somatosensory reflex seizures in a patient with hypertrophic cranial pachymeningitis

Somatosensory-evoked reflex epilepsy is characterized by seizures in response to specific stimuli. It is highly uncommon for somatosensory-evoked focal seizures to be caused by movement or a change in posture. Reflex epilepsy induced by both somatosensory and proprioceptive stimulations has not been previously reported. In this study, we present a case of reflex epilepsy evoked by somatosensory and proprioceptive stimulation in a patient with hypertrophic cranial pachymeningitis. After comparing our patient with other cases of previously reported somatosensory-evoked reflex epilepsy, we determined that our patient had an unusual cause of reflex epilepsy.     

肥厚性硬脑膜炎16例临床分析

目的 探讨肥厚性硬脑膜炎的临床和影像学特点.方法 对16例肥厚性硬脑膜炎患者的病因、临床表现、核磁共振(M RI)及治疗进行回顾性分析.结果 肥厚性硬脑膜炎可继发于感染性疾病或与自身免疫性疾病并存,临床表现以头痛最常见(100％),磁共振成像可见硬脑膜局部或弥漫性肥厚,T1WI呈等或略低信号,T2WI呈明显低信号,增强扫描可见明显强化.结论 肥厚性硬脑膜炎病因复杂,临床表现以头痛和脑神经麻痹为主要症状,磁共振检查结果是临床诊断的重要依据.     

A case of hypertrophic cranial pachymeningitis developed skull lesion]

We report a case of hypertrophic cranial pachymeningitis (HCP) developed skull lesion. A 70-year-old male presented with the symptom of left hemiconvulsion. MRI revealed that the enhanced intraosseous mass infiltrated into the the dura and brain parenchyma under the parasagittal region of the right parietal bone. Histological examination revealed chronic inflammation with lymphoplasmacytic infiltrate and fibrosis of both intraosseous mass and dural invasive lesion. Steroid therapy resulted in improvement of clinical symptoms and enhanced lesion of MRI. Three years later, the patient presented with generalized convulsion and weakness of right upper and lower limbs. MRI revealed dural thickening with gadolinium enhancement in the bilateral parasagittal region and falx. Angiography showed occlusion of the superior sagittal sinus. The cause of relapsing symptoms in this patient may have been related to the occlusion of the superior sagittal sinus, due to HCP. We considered that the incipient intraosseous mass resulted from a response of the marrow by destructive progression of chronic inflammation passed through the fracture crack or the cavity of arachnoid granulation.     

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.     

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.     

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.     

肥厚性硬脑膜炎的临床及影像学特征

目的研究肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)的临床表现和MRI特征。方法对9例HCP患者的临床、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,临床以头痛和脑神经麻痹为主要表现。MRI结果示受累硬脑膜可见于小脑幕、鞍旁海绵窦区、大脑凸面等;增厚的硬脑膜多呈条带状或斑块状;T1WI呈与皮质相等或略低信号,T2WI呈低信号,3例患者靠近脑实质面的硬膜呈明显高信号;增强扫描后增厚的硬脑膜明显强化。1例伴邻近脑实质长T1长T2异常信号灶。复查时,病情改善者硬脑膜变薄,累及范围缩小,强化减轻,伴发的脑内异常信号影缩小或消失。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。     

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.     

A case of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome]

A 48-year-old female was seen because of left orbital pain. The neurological findings were normal at her first visit. She presented temporary double vision during conservative period. Plain CT revealed no mass around the sellar region. Enhanced CT revealed enhanced mass in the left cavernous sinus. MRI revealed low intensity lesion on both T1 and T2 weighted images. Enhanced MRI showed strongly enhanced mass extended from the left cavernous sinus to the dura of sellar floor, the contralateral cavernous sinus, and cerebellar tentorium. Angiography showed stenosis of the left internal cerebral artery. Biopsy from the dura of the tentorium was performed via anterior temporal approach. Abundant collagen fibers with hyalinization were seen in the thickened dura. A final diagnosis of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome was made. Symptoms were free just after the surgery, however, the regrowth of the tentorial lesion was found on MRI after one year. The size of the lesion decreased after administration of steroid.     

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。     

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。     

Chronic hypertrophic cranial pachymeningitis associated with HTLV-I infection.

Two patients presenting with recurrent multiple cranial neuropathy showed diffuse thickening and gadolinium enhancement of the dura mater on brain MRI. Both had anti-HTLV-I antibodies in serum. A quantitative polymerase chain reaction study of the peripheral blood disclosed that the HTLV-I proviral DNA loads increased considerably in one case and moderately in the other. Both showed a spontaneous proliferation of peripheral blood lymphocytes as well as an increase in helper/inducer T cells. Neither had any other underlying infections or autoimmune diseases. Thus it is possible that hypertrophic pachymeningitis developed as a result of multiorgan involvement of HTLV-I infection in these patients.     

肥厚性硬脑膜炎12例临床表现和影像特征

目的　研究肥厚性硬脑膜炎(hypertrophiccranialpachymeningitis, HCP)的临床表现和磁共振成像(MRI)特征。方法　对12例肥厚性硬脑膜炎患者的临床和MRI资料进行回顾性分析。结果　多呈慢性起病,临床上以头痛(11例)和脑神经麻痹(9例)为主要表现,其中外展神经受损者7例,三叉神经4例,视神经、动眼神经、滑车神经、面神经各2例。MRI示受累硬脑膜最多见于鞍旁海绵窦区(7例),小脑幕(6例)次之,中颅窝底、大脑凸面各4例,累及大脑镰3例。同一病例可累及多个部位(弥漫型),本组弥漫型7例,局限型5例。增厚的硬脑膜厚度为0. 5 ~2. 0cm,形状多呈条带状或斑块状; T1WI呈与脑灰质相等或略低信号,T2WI呈低信号, 4例靠近脑实质面的硬膜呈明显高信号;增强后增厚的硬脑膜明显强化。5例伴邻近脑实质长T1 长T2 异常信号灶。复查时,病情改善者硬脑膜变薄,累及范围缩小,强化减轻,伴发的脑内异常信号影缩小或消失。结论　肥厚性硬脑膜炎的MRI表现较具特征性,结合临床资料可以做出较明确诊断。     

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...     

A case of chronic hypertrophic cranial pachymeningitis causing cranial polyneuropathy and unilateral central retinal vein occlusion]

We presented a 70-year-old woman who developed unilateral visual loss due to central retinal vein occlusion caused by chronic hypertrophic cranial pachymeningitis. She had developed right blindness with optic atrophy due to chronic intracranial pachymeningitis one year before admission. In June 1999, she noticed visual loss of the left eye. On admission, neurological examination revealed left visual loss and sensory impairment in the first branch area of bilateral trigeminal nerves. Ophthalmological examination revealed central retinal vein occlusion of the left eye. Brain MRI showed dural thickening with gadolinium enhancement of the cavernous sinus near the left optic nerve and dilatation of the left supraorbital vein. There was no compression of the left optic nerve. We suggest that central retinal vein occlusion may be caused by compression of the supraorbital vein by dural thickening. This is the first case report of central retinal vein occlusion associated with chronic hypertrophic cranial pachymeningitis.     

Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis

Hypertrophic cranial pachymeningitis (HCP) is a rare inflammatory disease of unknown origin in which recurrence is frequently observed in spite of the initial response to steroid therapy. Three patients, 1 man and 2 women, aged 63, 66, and 67 years, with severe intractable headache were evaluated by brain MRI. All patients were initially given prednisolone (60 mg/day, oral) or dexamethasone (20 mg/day, i.v.), and followed by long-term (at least 1 year) azathioprine therapy. All patients were evaluated by follow-up laboratory tests and brain MRI study, and completed the 2-year follow-up period. Symptoms including headache were initially improved with corticosteroid therapy, but patients became steroid-dependent. Azathioprine administration in these steroid-dependent patients permitted the complete cessation of corticosteroid and led to the clinical and radiological recovery. In conclusion, initial high-dose corticosteroid administration followed by long-term azathioprine therapy may be the ideal treatment of HCP at present.     

肥厚性硬脑膜炎8例临床分析

目的总结分析肥厚性硬脑膜炎的临床特点及影像学特征,探讨其可能的发病机制。方法收集宣武医院2007年1月²009年6月肥厚性硬脑膜炎8例临床资料进行分析。结果均慢性起病,头痛6例;多脑神经损害6例,其中动眼神经6例,舌咽、迷走、听神经各3例,视神经、外展神经各2例,舌下神经1例;癫痫发作2例。脑脊液:压力升高6例,白细胞数升高2例,蛋白升高8例,免疫球蛋白升高6例,白细胞数升高2例。血沉升高4例。血免疫球蛋白升高3例。血补体升高2例。头部MRI病变在T1、T2加权像呈等信号或低信号,增强扫描弥漫强化7例,局部强化1例,受累硬脑膜小脑幕5例,大脑镰、额顶部、海绵窦各3例,伴硬脊膜强化2例,继发静脉窦狭窄2例,无颅底强化病例。激素治疗7例疗效良好,其中1例持续高颅压手术治疗好转,1例未治疗好转;继发于乳突炎、中耳炎或鼻窦炎4例,特发性4例。结论肥厚性硬脑膜炎可发生于任何年龄。主要临床表现和头部MRI检查具有特征性,必要时活检确诊。皮质激素治疗效果好,必要时手术减压。     

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.     

特发性肥厚性硬脑膜炎12例临床分析

目的总结特发性肥厚性硬脑膜炎的临床表现和实验室特点,并探讨特发性肥厚性硬脑膜炎与自身免疫反应的相关性。方法回顾分析12例特发性肥厚性硬脑膜炎患者的临床表现、实验室检查、MRI检查和治疗经过,与18例颅内静脉窦血栓形成患者的实验室治疗进行比较。结果 12例患者临床主要表现为头痛、脑神经损害症状和小脑症状;血清学指标,2例(2/12)于正常值范围,4例(4/12)血常规白细胞计数升高,7例(7/12)血清C-反应蛋白、红细胞沉降率和血清淀粉样蛋白A (serum amyloid A,SAA)水平升高,2例(2/12)类风湿因子水平升高,1例(1/12)抗DNA-组蛋白复合物抗体,1例(1/12)抗核抗体阳性、1例(1/12)可疑阳性,1例抗心磷脂抗体(anti-cardiolipin antibody,ACA)可疑阳性,1例[1/12髓过氧化物酶(myeloperoxidase,MPO)]阳性;脑脊液指标,6例(6/12)压力升高; 4例(4/12)白细胞数升高,10例蛋白定量增高,2例蛋白定量正常,8例(8/12) Pandy试验阳性,1例(1/12)可疑阳性,11例(11/12) IgA升高、11例(11/12) IgM升高、8例(8/12) IgG升高。特发性肥厚性硬脑膜炎患者仅脑脊液蛋白定量(t=4. 78,P=0. 00)以及IgA(t=5. 12,P=0. 00)、IgM(t=2. 16,P=0. 04)和IgG(t=4. 29,P=0. 00)水平高于颅内静脉窦血栓形成患者; Pearson相关分析显示,特发性肥厚性硬脑膜炎患者脑脊液蛋白定量与脑脊液IgA(r=0. 690,P=0. 01)和IgG(r=0. 702,P=0. 01)水平均呈正相关,而与IgM(r=0. 483,P=0. 11)不相关。激素和免疫抑制剂治疗有效。结论特发性肥厚性硬脑膜炎可能系自身免疫反应所致,免疫球蛋白水平升高考虑以鞘内合成和炎症反应为主要原因。