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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes a 69-year-old woman who presented with acute monocular visual loss, ipsilateral headache, and elevated sedimentation rate and C-reactive protein. Both temporal artery biopsies were negative. Neuroimaging, dural biopsy, and breast biopsy all confirmed the diagnosis of carcinomatous hypertrophic pachymeningitis associated with metastatic breast carcinoma. After treatment with corticosteroids, her vision improved. Her clinical presentation initially mimicked the symptoms and signs of giant cell arteritis. Acute monocular visual loss without other cranial nerve palsies may be an uncommon presentation of hypertrophic pachymeningitis from metastatic breast carcinoma.
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PMID:Acute monocular visual loss in carcinomatous hypertrophic pachymeningitis mimicking giant cell arteritis. 1648 77

Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with progressive bifrontal headache, bilateral visual loss and transient episode of confusion. Neurological examination revealed bilateral optic atrophy, apathy and no focal neurological deficit. Investigations showed anemia of chronic disease, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. No specific inflammatory diseases or malignancy such as systemic lupus erythematosus, syphilis, hematologic malignancy were found MRI of the brain revealed thickened and enhanced dura mater and leptomeninges at the inferior aspect of bilateral frontal lobes as well as vasogenic edema of the frontal lobes. Cerebrospinal fluid showed mild pleocytosis, high protein level and normal glucose level. Meningeal biopsy revealed nonspecific inflammatory process of the dura and leptomeninges. There was no granuloma formation or evidence of vasculitis. Special stain for tuberculous bacilli, fungus and malignancy were all negative. The diagnosis of "idiopathic hypertrophic pachymeningitis" was made. The patient was treated with oral prednisolone 45 mg/day. Her headache was improved, but the profound vision loss in both eyes remained unchanged after 2 years of follow-up. Prednisolone was tapered within 18 months. Idiopathic hypertrophic cranial pachymeningitis usually involves dura at tentorium cerebelli, cavernous sinus and base of the skull. The extensive involvement at the anterior cranial fossa is extremely rare.
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PMID:Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature. 1651 96

A 54-year-old man was admitted to our hospital because of a headache, dry cough, low grade fever and hearing loss sustained for 6 months. Physical and neurological examinations revealed bilateral conjunctival hyperemia, fine crackles in the lower lungs, cutaneous scars, horizontal gaze evoked nystagmus, bilateral moderate sensorineural deafness and mild hyperreflexia. Hypertrophic intracranial pachymeningitis (HIP) accompanied by episcleritis, pulmonary fibrosis, subcutaneous fibrosis of the trunk and upper limbs, bilateral chronic otitis media and sinusitis of the paranasal cavities were observed. Histopathological investigation of biopsied tissues from the dura matter, lung, skin and nasal mucosa showed marked fibrosis with lymphocyte and plasma cell infiltrations. The diagnosis of multifocal fibrosclerosis (MF) was made; this is a rare syndrome of unknown etiology characterized by fibrosis involving multiple organ systems. Although steroid pulse therapy and cyclophosphamide (CP) pulse therapy was not effective in his illnesses, the combination therapy of corticosteroid and oral CP was dramatically effective. We concluded that HIP can be a manifestation of MF, and additional oral CP should be considered as a treatment for steroid-resistant MF with HIP.
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PMID:[Oral cyclophosphamide therapy for multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis]. 1661 37

We describe a case of hypertrophic cranial pachymeningitis (HCP) associated with Wegener's granulomatosis (WG) in a 60-year-old man presenting with chronic headache and multiple cranial nerve neuropathies. A test for antibodies to the neutrophil cytoplasmic protein myeloperoxidase (MPO-ANCA) was positive in this case. We review the literature on perinuclear (p)-ANCA-related HCP, including our case. This case indicates the link between MPO-ANCA-positive WG and HCP.
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PMID:MPO-ANCA-positive Wegener's granulomatosis presenting with hypertrophic cranial pachymeningitis: case report and review of the literature. 1714 71

In a 67-year-old man with a history of professional asbestos exposure and persistent headaches since one year, a diagnosis of idiopathic pachymeningitis was made. IHCP (idiopathic hypertrophic cranial pachymeningitis) is in literature referred to as one of the presentations of Multifocal Idiopathic Fibrosclerosis or MIF, representing a group of fibro-inflammatory and pathologically similar diseases, including also retroperitoneal fibrosis, mediastinal fibrosis and sclerosing cholangitis. Since recent literature links asbestos exposure to a risk of development of retroperitoneal fibrosis, the present case suggests to focus in future research of IHCP on possible external causes.
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PMID:An external cause for 'idiopathic' pachymeningitis? 1732 46

We report a case of systemic lupus erythematosus (SLE) complicated with hypertrophic pachymeningitis. A 34-year old woman who was diagnosed as SLE in 1985 was admitted to our hospital for a high grade fever and a headache. Laboratory findings showed increased titer of anti-double strand DNA antibody and decreased number of platelets. She complained a severe headache and hearing loss which were worsened by head-up position, resembling the symptoms of intracranial hypotension. MRI findings revealed thickened dura and she was diagnosed as hypertrophic pachymeningitis. Both clinical symptoms and laboratory findings were resolved after methyl-prednisolone pulse therapy followed by a high dose of prednisolone. Although hypertrophic pachymeningitis is a rare complication with SLE, it should be considered in SLE patients with severe headache.
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PMID:[A case report of hypertrophic pachymeningitis associated with systemic lupus erythematosus, showing a headache and hearing loss resembling intracranial hypotension]. 1733 6

Idiopathic hypertrophic pachymeningitis (IHPM) is a rare pathological state, with still unclear aetiopathogenesis. We present a case of a 63-year-old woman with cranial variety of that disease. The manifestations of the disease included headaches, paresis of VI, IX, X nerves and cerebellar ataxia. The disease was diagnosed with magnetic resonance imaging (MRI) and histopathological assessment of the pachymeninx biopsy specimen. The MRI revealed significant thickening of the cranial base pachymeninx, compressing the pons and medulla oblongata. MRI examinations could be misinterpreted as extensive meningioma of the skull base. Dura mater biopsy revealed however inflammation with abundant lymphocytic infiltrations. Clinical improvement was obtained after the application of corticosteroids. We noted the subsidence of all symptoms of the disease, as well as radiological improvement, manifested through substantial regression of the described changes in the pachymeninx. The patient has been presented in the context of 65 cases of idiopathic hypertrophic pachymeningitis, described in the literature of English-speaking countries in the last five years. Recently, the importance of the autoimmunogenic background of IHPM has been underlined. In that respect IHPM has become an interdisciplinary problem. Its diagnosis and treatment requires not only radiologists, neurologists, pathomorphologists and neurosurgeons, but also specialists in internal medicine, including immunologists, allergologists and rheumatologists as well - in other words, physicians that rarely take part in the processes of diagnosing and treating intracranial pathologies.
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PMID:Idiopathic hypertrophic pachymeningitis - case report and literature review. 1735 10

We present the case of a 68 year-old man with a diffused hypertrophic pachymeningitis (HP) involving both internal auditory canals. The clinical symptoms were headache, decreased vision in one eye, progressive bilateral and symmetrical sensory-neural hearing loss (PSNHL) responsive to steroid treatment. Although hearing loss is a frequent manifestation of HP, only few studies reported an adequate audiological assessment and follow-up. Mechanisms related to the auditory involvement are discussed on the basis of audiological data. Gadolinium enhanced MRI is the most adequate technique for HP detection and for the differential diagnosis. A delay in the diagnosis of HP seems to be quite common and the consequences may be severe, especially in cases of optic nerve involvement. For these reasons, a cerebral MRI should probably be included in the assessment of PSNHL, especially when neurological signs coexist or are reported in the medical history.
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PMID:Symmetric sensorineural progressive hearing loss from chronic idiopathic pachymeningitis. 1736 62

Conventional therapy for Wegener's granulomatosis, steroid and cyclophosphamide, fails to control disease activity in some refractory patients and has treatment-related toxicity. B cell depletion therapy using rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for certain autoimmune diseases including antineutrophil cytoplasmic antibody (ANCA) -associated systemic vasculitis. We report two refractory cases of Wegener's granulomatosis: one with bronchial and pulmonary involvement and retroorbital granuloma, the other with retroorbital granuloma and hypertrophic pachymeningitis causing severe headache. Rituximab was effective in both cases, with diminished granuloma and reduced ANCA titers, allowing steroids to be tapered. No adverse effects were detected.
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PMID:Two cases of refractory Wegener's granulomatosis successfully treated with rituximab. 1740 8

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


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