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 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.     

Idiopathic hypertrophic cranial pachymeningitis mimicking multiple meningiomas: case report and review of the literature

A case of idiopathic hypertrophic cranial pachymeningitis with an unusual and misleading manifestation is reported. Computed tomography scan, angiographic and magnetic resonance imaging findings were suggestive of multiple meningeal neoplasms and a correct diagnosis was made only after meningeal biopsy. This 44-year-old patient had a previous history of an ill-defined systemic disorder associating episcleritis, erythroderma nodosa and multiple peripheral arthritis. We review previous reports of idiopathic cranial pachymeningitis with emphasis on radiological investigation techniques, histopathology and possible dysimmune mechanisms of pathogenesis. Received: 28 January 1997 / Accepted: 19 March 1997     

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 pachymeningitis: case report

Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.     

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。     

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。     

Hypertrophic pachymeningitis with anti-neutrophil cytoplasmic antibody (p-ANCA), and diabetes insipidus

We treated a patient with idiopathic cranial hypertrophic pachymeningitis and elevated serum titer of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) reactive against myeloperoxidase. This 67-year-old man showed multiple cranial nerve-palsies, central diabetes insipidus (DI), and an intrasellar mass. DI and intrasellar mass had been present for 3 years, and DI had been well controlled by intranasal desmopressin. His nerve-palsies were most likely caused by thickened dura matter detected by the brain MRI. Granuloma may develop in the sella, and MRI findings in our patient are compatible to it. Corticosteroid and oral cyclophosphamide therapy improved his neurological symptoms and serum p-ANCA level with showing good correlation. DI improved temporally for 2 months. Few other cases of hypertrophic pachymeningitis with elevated p-ANCA have been reported, however the etiology is unknown. As p-ANCA antibodies have been detected in many of vasculitides, microvasculitis may be involved in some cases of idiopathic hypertrophic pachymeningitis.     

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.     

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 Spinal Pachymeningitis with an Osteolytic Lesion

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic, progressive, inflammatory disorder characterized by marked fibrosis of the spinal dura mater with unknown etiology. According to the location of the lesion, it might induce neurologic deficits by compression of spinal cord and nerve root. A 58-year old female with a 3-year history of progressive weakness in both lower extremities was referred to our institute. Spinal computed tomography (CT) scan showed an osteolytic lesion involving base of the C6 spinous process with adjacent epidural mass. Magnetic resonance imaging (MRI) revealed an epidural mass involving dorsal aspect of cervical spinal canal from C5 to C7 level, with low signal intensity on T1 and T2 weighted images and non-enhancement on T1 weighted-enhanced images. We decided to undertake surgical exploration. At the operation field, there was yellow colored, thickened fibrous tissue over the dura mater. The lesion was removed totally, and decompression of spinal cord was achieved. Symptoms improved partially after the operation. Histopathologically, fibrotic pachymeninges with scanty inflammatory cells was revealed, which was compatible with diagnosis of idiopathic hypertrophic pachymeningitis. Six months after operation, motor power grade of both lower extremities was normal on physical examination. However, the patient still complained of mild weakness in the right lower extremity. Although the nature of IHSP is generally indolent, decompressive surgery should be considered for the patient with definite or progressive neurologic symptoms in order to prevent further deterioration. In addition, IHSP can present as an osteolytic lesion. Differential diagnosis with neoplastic disease, including giant cell tumor, is important.     

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.     

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.     

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。     

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.     

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.     

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.     

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.     

Intracranial hypertrophic pachymeningitis with high perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) occurred in a patient on hemodialysis]

We present a 66-year-old woman undergoing hemodialysis who developed intracranial hypertrophic pachymeningitis. Neurological examinations revealed a loss of bilateral visual acuity with optic atrophy, headache, and markedly restricted bilateral extraocular movement. MRI examinations demonstrated homogenous hypertrophic dural enhancement compatible with hypertrophic cranial pachymeningitis, and biopsied dural specimen revealed chronic inflammatory changes with proliferation of dense collagen fibers. There was no direct evidence of vasculitis and specific infections including tuberculosis and troponema pallidum. Most of the inflammatory infiltrates were demonstrated to be T lymphocytes. Intriguingly, p-ANCA was found to be highly elevated at x 220 and decreased to x 110 after steroid treatment. Neurological manifestations and radiological findings also improved in accordance with the lowering of p-ANCA. Although a few reports have described similar conditions such as chronic renal failure accompanying hemodialysis and pachymeningitis, and though vasculitis was not depicted histologically in this patient, we considered that immunological mechanisms probably provoked the patient's glomerulonephritis and pachymeningitis. Additionally, positive reaction against hepatitis c virus might have influenced the immunological system leading to the occurrence of the pachymeningitis.     

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.