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 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.     

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.     

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.     

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。     

An old man presenting with fluctuating bilateral multiple cranial nerve palsies and positive test for perinuclear antineutrophil cytoplasmic antibody]

A 70-year-old man admitted to a local hospital because of facial muscle weakness, tinnitus and facial pain in left side, was then given corticosteroid with a tentative diagnosis of Bell's palsy and his symptoms gradually improved. Since these symptoms recurred six months later, he was referred to our neurological service. As his brain CT revealed diffuse thickening and enhancement of the dura mater, he was thought to have hypertrophic pachymeningitis (HP). Intravenous antibiotics were started for aspiration pneumonia and his neurological symptoms gradually improved. HP caused by bacterial infection was thus likely and antibiotics, rifampicin and metronidazole, were administered orally as an outpatient. However, one month later, these symptoms were worsened with headache and double vision. He was then rehospitalized. MR imagings of the head with gadolinium disclosed diffuse meningeal thickening and enhancement, especially of the left-sided cerebellar tentorium. Erythrocyte sedimentation rate and C-reactive protein were moderately elevated. Serum angiotensin converting enzyme was within normal range. The test for cytoplasmic antineutrohil cytoplasmic antibody (ANCA) in the serum was negative, however, that for perinuclear ANCA was positive at a titer of 99 EU. Lumbar puncture showed normal findings and negative culture results for bacteria, fungi or mycobacteria. Dural biopsy specimens showed non-specific granulomatous inflammation of the dura with epithelioid histiocytes and Langerhans type multinuclear giant cells with caseous necrosis, however, with no presence of fungi or tubercle bacilli. After the oral administration of cyclophosphamide (100 mg, daily) and prednisolone (40 mg, daily), his neurological symptoms and laboratory findings have been gradually improved and he is well one year after discharge. This case together with previous reports suggests that ANCA positive HP without evidence of other organ involvements may belong to the limited form Wegener's granulomatosis. In the literatures of idiopathic HP, the treatment effect with corticosteroid alone is initially favorable, but transient. On the other hand, using the combined therapy of cyclophosphamide and prednisolone, the remission has been achieved in more than 90% of patients with WG. These data suggest that P-ANCA positive HP should be treated with a combination of corticosteroid and cyclophosphamide.     

Multiple cranial nerve involvement associated with tentorial pachymeningitis of granulomatous type

Otitis media in a 43 year-old male patient was followed by signs of chronic meningitis with persistent headaches, moderate pleiomorphic lymphocytosis of CSF without hypoglycorrhachia and unilateral impairment of cranial nerves XI to II. These signs were accompanied by widespread inflammation with general deterioration of the patient's condition. After a time the tentorium of cerebellum showed thickening and significant contrast uptake in conjunction with necrotizing granulomatous inflammation, indicating tentorial pachymeningitis. Swift and complete remission was obtained with a corticoid-cyclophosphamide combination. After reviewing the other possible causes of chronic meningitis, the authors proposed a diagnosis of Wegener's disease of auricular origin in view of the compatibility of all the data. A few cases of cranial pachymeningitis of indeterminate origin are described in the literature. Only this patient was treated with a corticoid-cyclophosphamide combination. The remarkable efficacy of this double therapy confirms the diagnostic hypothesis of Wegener's granulomatosis.     

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.     

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.     

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.     

Idiopathic Hypertrophic Cranial Pachymeningitis Misdiagnosed as Acute Subtentorial Hematoma

A case of idiopathic hypertrophic cranial pachymeningitis (IHCP) misdiagnosed as an acute subdural hematoma is reported. A 37-year-old male patient presented with headache following head trauma 2 weeks earlier. Computerized tomography showed a diffuse high-density lesion along the left tentorium and falx cerebri. Initial chest X-rays revealed a small mass in the right upper lobe with right lower pleural thickening, which suggested lung cancer, such as an adenoma or mediastinal metastasis. During conservative treatment under the diagnosis of a subdural hematoma, left cranial nerve palsies were developed (3rd and 6th), followed by scleritis and uveitis involving both eyes. Magnetic resonance imaging (MRI) revealed an unusual tentorium-falx enhancement on gadolinium-enhanced T1-weighted images. Non-specific chronic inflammation of the pachymeninges was noticed on histopathologic examination following an open biopsy. Systemic steroid treatment was initiated, resulting in dramatic improvement of symptoms. A follow-up brain MRI showed total resolution of the lesion 2 months after steroid treatment. IHCP should be included in the differential diagnosis of subtentorial-enhancing lesions.     

Severe dysphagia in lower cranial nerve involvement as the initial symptom of Wegener's granulomatosis

We observed a 42-year-old woman presenting with severe dysphagia secondary to paralysis of the lower cranial nerves and right phrenic nerve involvement, followed by respiratory failure. An EMG confirmed bilateral denervation of the 9th, 10th, 11th and 12th cranial nerves and right phrenic nerve. Videolaryngoscopy showed bilateral vocal fold immobility. Anemia, elevated ESR, microhematuria and C-ANCA (PR-3) antibodies were detected. Brain MRI and CSF were normal. A chest CT showed bilateral, irregular pulmonary lesions. An 18F-FDG total body scan showed diffuse hypermetabolic regions in both pulmonary bases, in the mediastinic region and in the rhinopharynx, raising the suspicion of a neoplastic process. A transthoracic biopsy disclosed nodular granulomatous aggregates with multinucleated giant cells, supporting the diagnosis of Wegener's granulomatosis. Immunosuppressive therapy achieved complete clinical resolution and cleared the pulmonary lesions. To the best of our knowledge this is the first report of Wegener's granulomatosis presenting with neurogenic dysphagia due to lower cranial nerve palsy.     

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.     

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.     

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.     

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、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 masquerading as Tolosa-Hunt syndrome.

Idiopathic hypertrophic cranial pachymeningitis is a rare condition. A case of idiopathic hypertrophic cranial pachymeningitis presenting as Tolosa-Hunt syndrome is being reported. The importance of neuroimaging in patients with suspected Tolosa-Hunt syndrome is discussed. Tolosa-Hunt syndrome might represent a focal manifestation of Idiopathic hypertrophic cranial pachymeningitis. Future studies are necessary to further clarify the relationship between these two conditions.     

A clinical study of pachymeningitis]

We report five patients with cranial and spinal pachymeningitis not associated infections or tumors. The underlying causes of pachymeningitis in four patients were Sj?gren syndrome, Wegener's granulomatosis, microscopic polyangitis, and dermatomyositis, respectively. In one patient, the cause was unknown. Cerebrospinal fluid analysis showed an increase in cells in only one patient and elevated protein levels in three patients. Blood test revealed an inflammatory disease except in one patient. The anti-nuclear antibody was present in four of five patients examined, and P-ANCA was present in two out of four patients. The prognosis was good except in one patient of Wegener's granulomatosis. We reviewed 48 previously reported cases of pachymeningitis were not associated infections or tumors. The lesions of pachymeningitis were more localized in the cranial dura than the spinal dura, and many cases had immunological abnormalities.