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A case of hypertrophic cranial pachymeningitis was reported. A 58-year-old female presented the symptoms of headache and vomiting. At the age of 27, she had suffered from tuberculosis. Neurological examination on admission revealed bilateral papilledema, bilateral hearing disturbance, right hypoglossal nerve palsy, ataxic gait, and bilateral intentional tremor. CT scan showed dilatation of the lateral and third ventricles, and compression of the fourth ventricle with marked enhancement of cerebellar tentorium. A ventriculoperitoneal shunt was installed bringing about improvement in bilateral papilledema, ataxic gait, and bilateral intentional tremor. One month later, ataxic gait and bilateral intentional tremor recurred, and monoparesis of the left upper extremity developed. MRI demonstrated hypertrophic dura mater in the posterior fossa and compressed cervical spinal cord. Decompressive surgery was performed bringing about remarkable clinical improvement. The pathological specimen showed thickening of the dura mater with concentric layers of dense fibrous tissue infiltrated with plasma cells. A diagnosis of hypertrophic cranial pachymeningitis was established. Three years later, the clinical features were found unchanged, but contrast enhancement of cerebellar tentorium had progressed markedly. Hypertrophic pachymeningitis is a uncommon disease. But it should be noted that intracranial involvement is very rare. The etiology, symptomatology, neuroradiology, and treatment are discussed and the literature is reviewed.
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PMID:[A case of hypertrophic cranial pachymeningitis]. 203 16

Here we present a 53-year-old woman with rheumatoid pachymeningitis. The subject had rheumatoid arthritis (RA) for 15 years. In April, 1996 she began to experience intermittent headaches. In September, her headaches became severe and continuous. In October, she suddenly developed ptosis of the left eye and diplopia. She also started to have dysphagia and she found it increasingly difficult to eat. She was admitted to our hospital on November 1, 1996. Neurological examinations revealed palsies of the left IIIrd, IVth, and VIth, and bilateral IXth, and Xth cranial nerves. Laboratory findings showed leukocytosis, elevated blood sedimentation rate, and positive CRP. Serum RA titer was positive (30x). The cerebrospinal fluid was normal and bacteriological examination was negative. T1-weighted MRI demonstrated hypertrophic cranial dura extending from the falx cerebri to tentorium cerebelli, which was enhanced markedly by Gd-DTPA. The dura adjacent to the cavernous sinus and the clivus were also thickened, which probably caused her cranial polyneuropathies. The dural biopsy showed massive infiltration of the inflammatory cells throughout the dura, proliferation of collagen fibers, and necrotic granuloma with neutrophilic infiltrations. Neither rheumatoid nodules, nor vasculitis were found. Despite the absence of rheumatoid nodules in the dural biopsy, the clinical features, pathologic specimens, and MRI findings of the thickened dura were most consistent with rheumatoid pachymeningitis. Administration of dexamethason ameliorated her headache on the 4th hospital day, and the cranial polyneuropathies completely disappeared on the 35th hospital day. The dural enhancement previously seen on the contrast T1-weighted MRI was diminished. Serum RA titer was also normalized (10x). Rheumatoid pachymeningitis is an extremely rare disease, and only 16 cases were reported in the literatures. Hypertrophic pachymeningitis should be considered as a diagnostic possibility in RA patients who have prolonged headache, and Gd-DTPA MRI is recommended to demonstrate the dural involvement.
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PMID:[A case of rheumatoid pachymeningitis]. 943 Oct

Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower cranial nerves were chiefly involved in two patients whereas optic nerve was the predominantly affected nerve in one. Except for the presence of rheumatoid arthritis in one of the patients, we could not document clinical or biochemical evidence of any predisposing infective, inflammatory or infiltrative condition in the other two. All three patients had characteristic changes on imaging suggestive of thickened and enhancing duramater. Although variable steroid responsiveness was seen in all the three patients, tendency towards steroid dependence was evident. The clinical presentations, causes, radiological features, management options and differential diagnosis of this unique clinical syndrome have been discussed.
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PMID:Hypertrophic pachymeningitis: varied manifestations of a single disease entity. 1196 Jan 42

Hypertrophic pachymeningitis is a rare disorder characterized by meningeal thickness, that can be caused by infection, tumoral infiltration, inflammatory disorders or idiopathic. We report the case of a 40 year-old man that presented with longstanding headache and progressive bilateral visual loss and proptosis. Cranial and orbital magnetic resonance imaging revealed diffuse dural thickness and bilateral extraconal orbital lesion. Extensive investigation did not reveal any systemic condition. Histopathological study after meningeal and orbital biopsy disclosed a chronic inflammatory process compatible respectively with idiopathic hypertrophic pachymeningitis (IHP) and orbital pseudotumor. This case emphasizes that orbital involvement can occur in IHP and that its early identification is of great importance in order to improve the visual prognosis of this condition.
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PMID:[Cranial idiopathic hypertrophic pachymeningitis associated with orbital pseudotumor: case report]. 1625 78

Hypertrophic pachymeningitis is a condition characterized by significant chronic inflammatory thickening of the cranial dura mater, frequently presenting with symptoms such as headache and cranial neuropathy. In this report, we describe a very rare case of hypertrophic pachymeningitis, considered to be attributable to Epstein-Barr virus (EBV), which was diagnosed in a patient who visited our hospital with a complaint of ongoing severe headaches. The diagnosis was based on positive specific serum EBV antibody titers, with VCA-IgM levels of less than 1:10, VCA-IgG levels of 1:160, and EBNA levels of 1:40, as well as on the results of magnetic resonance imaging of the head with contrast media.
Headache 2007 Apr
PMID:A case of hypertrophic pachymeningitis with prolonged headache, attributable to Epstein-Barr virus. 1744 15

Hypertrophic pachymeningitis is a very unusual disease, the main characteristic of which is thickening of the dura mater. We describe a patient who started this illness showing chronic headache and pauciimmune necrotizing extracapillary perinuclear antineutrophil cytoplasmic antibody (P-ANCA) associated glomerulonephritis. The diagnosis was made by brain magnetic resonance image. She received immunosuppressant therapy with prednisonel and cyclophosphamide with clinical improvement.
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PMID:[Hypertrophic pachymeningitis, glomerulonephritis and P-ANCA associated small vessel vasculitis]. 1759

Hypertrophic pachymeningitis (HP) is thought to have an autoimmune etiology but its precise cause and treatment remains to be elucidated. Here, we report the clinical details and therapeutic responses of 3 patients with HP and reviewed 66 previously reported cases in the literature. Among these patients, headache was the most frequent complaint. Cranial nerve involvement was also frequently observed, with the optic nerve being the most frequently impaired followed by the oculomotor, trochlear, and abducens nerves in frequency. Elevated C-reactive protein levels and erythrocyte sedimentation rates were found in approximately 97% of the patients. Steroids were the most commonly prescribed therapy, but no definite protocols for the standard dose and duration in HP have been proposed thus far. The average initial dose of prednisolone (PSL) was 42.7 mg/day, and the average maintenance dose was 12.4 mg/day in the chronic stage. Recurrence occurred in many patients when the dose of PSL was reduced to under 20 mg/day. Therefore, steroids should be tapered extremely slowly.
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PMID:[Hypertrophic pachymeningitis: three adult cases and a review of the literature]. 2159 92

Hypertrophic pachymeningitis is a rare disease, and the fibrosing inflammatory process causes a thickening of the dura mater. A 62-year-old male undergoing corticosteroid therapy for autoimmune pancreatitis presented with headache and right facial numbness. Brain CT and MRI revealed thickened mass lesion around the tentorium. The specimen obtained by biopsy showed a small number of immunostain areas positive for IgG and IgG4. Systemic IgG4 related disease entity is proposed and analyzed from Japan, and pachymeningitis is also included in the examination. Some autoimmune mechanism is related to pachymeningitis, however, it is necessary to consider well if only the IgG4 has responsibility for the disease.
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PMID:[Hypertrophic pachymeningitis associated with autoimmune pancreatitis examined for IgG4 related disease: a case report]. 2179 26

Hypertrophic pachymeningitis is a progressive disease resulting in a diffuse thickening of dura mater due to inflammation, tumor or autoimmune diseases, but most cases are idiopathic. It is seldom reported to be related to sensorineural hearing loss, but it can cause sensorineural hearing loss which can be potentially reversed through treatment. Here, we report the case of a 54-year-old woman who had progressive, bilateral, worse in the left, sensorineural hearing loss and visual disturbance with an accompanying headache over several months. Brain MRI showed diffusely thickened dura mater, highly enhanced after gadolinium administration, which was consistent with pachymeningitis. It was assumed to be related to autoimmune pathogenesis on the basis of elevated serum myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titers. After empirical steroid and cyclophosphamide therapy, auditory impairment improved, especially in the high frequency region of the pure tone audiogram, and significant improvement in the word recognition test. Moreover, a follow-up MRI revealed much decreased enhancement of the dura mater, and the MPO-ANCA titer decreased to within the normal range. In the case of rapidly progressive sensorineural hearing loss or hearing impairment accompanying other cranial neuropathy, pachymeningitis should be taken into consideration, and brain MRI with gadolinium enhancement is the best method of detecting it. Also, to ensure proper treatment, a cautious evaluation including an ANCA work-up should be performed.
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PMID:Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA. 2194 83

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.
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PMID:Idiopathic hypertrophic cranial pachymeningitis: Three biopsy-proven cases including one case with abdominal pseudotumor and review of the literature. 2202 32


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