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.     

IgG4-Related Intracranial Hypertrophic Pachymeningitis : A Case Report and Review of the Literature

Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Recently, the possibility that IgG4-related sclerosing disease may underlie some cases of intracranial hypertrophic pachymeningitis has been suggested. We herein report the tenth case of IgG4-related intracranial hypertrophic pachymeningitis and review the previous literature. A 45-year-old male presented with left-sided focal seizures with generalization. Magnetic resonance imaging (MRI) revealed a diffuse thickening and enhancement of the right convexity dura matter and falx with focal nodularity. The surgically resected specimens exhibited the proliferation of fibroblast-like spindle cells and an infiltration of mononuclear cells, including predominantly plasma cells. The ratio of IgG4-positive plasma cells to the overall IgG-positive cells was 45% in the area containing the highest infiltration of plasma cells. On the basis of the above findings, IgG4-related sclerosing disease arising from the dura mater was suspected. IgG4-related sclerosing disease should be added to the pachymeningitis spectrum.     

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。     

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.     

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.     

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.     

硬脑膜磁共振强化的临床分析

目的分析MRI提示硬脑膜强化的常见病因,以便为此类疾病的诊断提供帮助。方法对20例MRI增强扫描示硬脑膜强化患者的病史、体征及辅助检查结果等临床资料进行综合分析。结果20例MRI增强扫描示硬脑膜强化患者中脑膜癌病(MC)9例、肥厚性硬脑膜炎(HP)6例、低颅压综合征(SIH)5例。结论硬脑膜强化可见于中枢神经系统多种疾病,在临床工作中应综合全面分析患者病例资料以明确诊断。     

A case of hypertrophic spinal pachymeningitis associated with MPO-ANCA]

A 71-year-old man was admitted to our hospital, because of subacute progressive back pain and thoracic transverse myelopathy. Magnetic resonance imaging showed thickening with gadolinium enhancement of dura mater at the T1-T6 vertebrate levels. A dura biopsy specimen revealed fibrous thickening of the dura with the inflammatory changes, and diagnosis of hypertrophic spinal pachymeningitis was made. The cerebrospinal fluid (CSF) contained high titer of myeloperoxydase anti-neutrophil cytoplasmic antidody (MPO-ANCA), indicating that the antibody synthesized intrathecally. After treatment with oral prednisolone, 60 mg daily for a month, thickness of the dura and the CSF MPO-ANCA improved dramatically. This is the first report of hypertrophic pachymeningitis associated with MPO-ANCA localized exclusively in the spine. This report suggests that intrathecal MPO-ANCA synthesis is an index of disease activity as well as a diagnostic hallmark. Early corticosteroid therapy is recommended in this disorder.     

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.     

A case of hypertrophic cranial pachcymeningitis in a patient with lymphocytic hypophysitis]

We report a 67-year-old man of lymphocytic hypophysitis complicated by hypertrophic cranial pachcymeningitis. The hypophysitis presenting with central diabetes insipidus caused isolated adrenocorticotropic hormone (ACTH) deficiency after three years and two months since the onset of his illness. Moreover he suffered from left cavernous sinus syndrome after four years. Brain gadolinium (Gd) enhanced MRI, which was obtained in 2001, showed only enhancement of the pituitary stalk. However, brain Gd enhanced MRI, performed four years later, showed not only a remarkable thickening and enhancement of the pituitary stalk but also abnormal enhancement of the lesion in the left cavernous sinus. In addition, it showed diffuse thickening and enhancement of the dura mater. This clinical course suggested that chronic inflammation of the pituitary had spread to the dura mater, a clinical condition differing from parasellar chronic inflammatory disease (PSCID). In addition, because diffuse thickening and enhancement of the dura mater was present, it was likely that lymphocytic hypophysitis was complicated by hypertrophic cranial pachcymeningitis due to autoimmune reactions. We should carefully observe cases of lymphocytic hypophysitis and assess change over time in the dura mater of the whole brain by Gd enhanced MRI.     

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.     

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.     

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.     

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 pachymeningitis of the cavernous sinus mimicking meningioma with complete occlusion of the carotid artery

Idiopathic hypertrophic pachymeningitis (IHPM) is a rare inflammatory entity characterized by the idiopathic thickening of the dura mater. An increasing number of cases have been reported involving the cavernoss sinus. We present the case of a patient with IHPM who was diagnosed of meningioma and showed a complete occlusion of the intracavernous carotid artery. This 42 years old woman was sent to our hospital to be treated radiosurgicaly with the diagnosis of meningioma. The diagnosis was based on MRI findings and on the temporal resistance to corticoid treatment. A complete study of the patient demonstrated the inflammatory nature of the lesion, which improved after long corticosteroid treatment. The differential diagnosis between meningioma and IHPM may be complex because of the similarities in image findings and the temporal resistance to corticosteroids shown by IHPM, thus we emphasize the usefulness of the SPECT to differentiate between inflammation and tumor in this clinical setting. In addition, our patient showed a total occlusion of the intracavernous carotid artery and, as far as we know, this is the first case with such a finding reported in the literature.     

A case of subarachnoid hemorrhage with pituitary apoplexy caused by idiopathic hypertrophic pachymeningitis

Steroid therapy is considered to improve clinical symptoms in hypertrophic pachymeningitis. We present a 70-year-old man with idiopathic hypertrophic pachymeningitis, whose clinical signs progressively worsened despite steroid therapy. He died of subarachnoid hemorrhage (SAH) with pituitary apoplexy 2 months after the admission regardless of improvement of laboratory data and magnetic resonance imaging appearance by one-and-half-month steroid therapy. Autopsy revealed thickened dura mater supporting the diagnosis of hypertrophic pachymeningitis. Brain parenchyma is generally not affected by the disease; however, histological investigation suggested that inflammation of the dura caused damage to superior hypophyseal artery resulting in SAH and apoplexy in the anterior lobe of the pituitary gland. The higher dose and the longer duration of steroid therapy should have achieved in our case although most laboratory data recovered within the normal range. The aggressiveness of hypertrophic pachymeningitis must be evaluated by clinical signs rather than by laboratory data or imaging examinations.     

Intraventricular cytarabine in a case of idiopathic hypertrophic pachymeningitis

Idiopathic hypertrophic chronic pachymeningitis (IHCP) is characterised by inflammatory fibrotic thickening of the dura mater. Long term management is controversial. A 28 year old man with craniospinal IHCP and prominent lymphocytic meningitis is reported. Cerebrospinal fluid and histological examination suggested a CD4+ T cell driven process and B cell stimulation. After surgical, tuberculostatic, and immunosuppressive treatment failed to control the progressive meningeal hypertrophy, causing severe headache and neurological disability, the disease process eventually abated with intraventricular cytarabine treatment.     

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.     

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up

Idiopathic hypertrophic cranial pachymeningitis (IHPM) is a clinicopathological entity characterized by thickening and fibrosis of the dura mater with resultant clinical symptoms. It is generally steroid responsive and has a tendency to remit and relapse. We present here two patients with IHPM with associated dural sinus occlusion and describe their clinicoradiological features and long-term course and outcome.     

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.