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

We report three patients with chronic headaches and optic neuropathy due to widespread meningeal thickening shown on enhanced MRI; all had biopsy-proven intracranial pachymeningitis (fibrosclerosis of the meninges). Two patients had bilateral optic neuropathy, elevated CSF protein, and polyclonal serum hypergammaglobulinemia. They developed temporal lobe cortical necrosis or sagittal sinus thrombosis, presumably due to compromised dural venous drainage from extensive meningeal fibrosis. The other patient had multiple cranial nerve palsies and unilateral optic neuropathy with normal CSF. Corticosteroid therapy improved visual function in all three patients, although all had persisting visual deficits. Gadolinium-enhanced MRI was essential in identifying meningeal inflammation and locating suitable biopsy sites.
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PMID:Visual loss from idiopathic intracranial pachymeningitis. 816 28

This is the first report describing hypertrophic cranial pachymeningitis which developed in association with mixed connective tissue disease (MCTD). A 56-year-old man with a two-year history of MCTD gradually developed symptoms of headache and blurred vision. Bilateral papilledema and increased opening pressure and mild pleocytosis of the cerebrospinal fluid were noted. Magnetic resonance imaging of the brain revealed a diffuse thickening and a gadolinium-enhancement of the cranial dura mater. While five previous patients, diagnosed as idiopathic or infectious hypertrophic cranial pachymeningitis in our department, had either otitis media or sinusitis and showed multiple cranial nerve involvement, the present patient had neither otitis media nor sinusitis and did not show any cranial nerve involvement other than papilledema.
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PMID:Hypertrophic cranial pachymeningitis associated with mixed connective tissue disease; a comparison with idiopathic and infectious pachymeningitis. 824 99

We report seven patients with cranial pachymeningitis of unknown origin in whom the main clinical features were headaches, ataxia, and cranial nerve palsies. CSF showed inflammatory changes. CT and MRI showed thickening of the falx and of the tentorium. The clinical course was chronic. Four patients improved with prednisolone but became steroid-dependent: in two cases, radiotherapy had no lasting improvement and in one, azathioprine permitted a reduction of the corticosteroids. Five patients had biopsy of the tentorium cerebelli or of the temporal dura mater. In two cases, autopsy revealed extensive pachymeningitis without parenchymal changes. In all instances, microscopic examination of the dura mater showed a cellular infiltrate of polymorphic cells; there were no epithelioid granulomas. Review of the literature discloses seven similar cases. We discuss the relationship of these lesions with inflammatory meningeal masses, the focal pachymeningitis of the Tolosa-Hunt syndrome, and multifocal fibrosis.
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PMID:Cranial pachymeningitis of unknown origin: a study of seven cases. 832 33

Hypertrophic cranial pachymeningitis is a rare, idiopathic form of granulomatous pachymeningitis. This report describes three cases of hypertrophic cranial pachymeningitis and discusses the clinical, radiographic, and pathological findings in these and other reported cases. These lesions typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction. They occur in patients of all age groups; the peak incidence is in the sixth decade. Hypertrophic cranial pachymeningitis is best identified by magnetic resonance imaging. The diagnosis is established by excluding all other granulomatous and infectious diseases. A dural biopsy is essential to confirm the diagnosis. Hypertrophic cranial pachymeningitis is initially responsive to steroid therapy, but in most cases it recurs or progresses despite treatment. Surgical excision of granulomas is occasionally necessary to alleviate a mass effect. The long-term outcome remains uncertain for most patients, but progressive disease is usually fatal owing to cranial neuropathies.
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PMID:Idiopathic hypertrophic cranial pachymeningitis. Report of three cases. 811 80

We evaluated 3 patients with biopsy-proven hypertrophic cranial pachymeningitis apparently unrelated to any systemic disease. Each patient had chronic headache, cranial neuropathy, an elevated ESR, and a mild CSF pleocytosis. Neuro-ophthalmic findings included bilateral sixth nerve palsies in two patients and the third had bilateral optic neuropathies. MR imaging revealed thickened dura that enhanced with Gd-DTPA administration. Histologic examination showed thickened, fibrotic dura with a sterile, chronic, nongranulomatous inflammation. The response to treatment was variable with corticosteroids, immunosuppressive drugs, or radiation. The distinctive MR appearance should help physicians recognize this rarely reported disease.
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PMID:Idiopathic hypertrophic cranial pachymeningitis. 834 Apr 79

A 34-year-old woman was admitted to our hospital in 1991, because of progressive headache, nausea and generalized edema. She was diagnosed as Crow-Fukase syndrome associated with plasma cell dyscrasia (IgA lambda type) in 1987, presenting with polyneuropathy, edema, and dermatologic changes. Those manifestations were improved with irradiation and corticosteroids, but headache, nausea and generalized edema gradually developed after the discontinuation of corticosteroid therapy in 1991. On admission, marked bilateral papilledema was noted but fever and meningeal irritation signs were absent. A spinal tap showed a clear cerebrospinal fluid (CSF) with an open pressure of more than 400 mmH2O, normal cell count, total protein level of 87 mg/dl, and IgG level of 12.3 mg/dl. The CSF culture for microorganisms was negative and the cytological study of CSF also was normal. De novo synthesis rate of CSF IgG was markedly elevated (35.3 mg/day). MRI of the head using Gd-DTPA revealed diffuse hypertrophic dura mater, which made the diagnosis of chronic pachymeningitis. Cerebral angiographies were normal. An RI cisternography demonstrated delayed absorption of the CSF without ventricular reflux. Gallium and bone scintigrams did not show any pathological accumulation of the isotopes in the head. The lack of abnormalities causing chronic pachymeningitis in this case suggests that the chronic pachymeningitis might be associated with Crow-Fukase syndrome. The development of increased intracranial pressure is not rare in Crow-Fukase syndrome but the etiology remained unknown in most cases. We therefore suggest that MRI study with contrast enhancement should be performed in cases of this condition with increased intracranial pressure.
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PMID:[A case of Crow-Fukase syndrome associated with chronic pachymeningitis]. 837 Feb 4

Spontaneous intracranial hypotension is uncommon. Clinically it consists mainly of postural headache associated with CSF pressure lower than 5 cm H2O. Recently it has been reported that in this syndrome cerebral MRI shows diffuse and symmetrical meningeal enhancement increasing with Gadolinium and disappearing spontaneously without any particular treatment. We report the cases of two women in whom CSF pressure, several weeks after the appearance of headache, was in normal limits and whose cerebral MRI showed such meningeal enhancement. Meningeal biopsy in a search for chronic pachymeningitis was normal in both cases. Diagnosis was established afterwards in the light of recent publications.
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PMID:[Spontaneous cerebrospinal fluid hypotension: value of cerebral MRI]. 851 29

A 67-year-old woman with a one-year history of tinnitus and headache had multiple cranial nerve palsies of V, VII, VIII, IX, X, XI and spastic paraparesis. She also had a secretory otitis media. Gd-DTPA-enhanced magnetic resonance imaging (MRI) revealed hypertrophy of the dura of the posterior fossa and spinal epidural mass which extended from C7 to T10. A biopsy of the epidural mass showed chronic granulomatous change. These lesions were completely cured with administration of antibiotics. We believe this case of double-lesion of hypertrophic cranial pachymeningitis and spinal epidural granulomatous lesion originated from a bacterial infection secondary to the secretory otitis media.
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PMID:Hypertrophic cranial pachymeningitis with spinal epidural granulomatous lesion. 856 27

A 36-year-old woman presented suffering from severe headache and diploia. Magnetic resonance imaging showed a diffuse thickening of the cranial dura mater which was enhanced by gadolinium-DTPA. Two months later, an improvement of the clinical symptoms and a spontaneous resolution of the dural thickening were noted. Hypertrophic cranial pachymeningitis and other diseases causing diffuse dural thickening are discussed.
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PMID:Spontaneous resolution of hypertrophic cranial pachymeningitis. 857 68

A case of cranial hypertrophic pachymeningitis of unknown etiology in a patient with 15-year history of headaches, cranial nerve palsies, and gait disturbance is reported. A 77-year-old woman was brought to our institute in a coma. CT revealed intracerebral hemorrhage in the right temporal lobe and thickening of the falx and tentorium. Fifteen years previously the patient had undergone CT scanning because of headaches, cranial nerve palsies, and progressive gait disturbance and a thickened tentorium, mild hydrocephalus and edematous change in the right temporal lobe had been reported. Since the etiology of her symptoms was unclear at the time, she did not receive adequate treatment. Her symptoms gradually progressed thereafter, and her visual acuity and hearing deteriorated. MR imaging in 1994 showed the thickened tentorium as a hypointense area with hyperintense edges on Gd-DTPA enhanced images. Angiography revealed narrowing of posterior portion of the superior sagittal sinus. The patient's condition rapidly deteriorated due to the intracranial hypertension and she subsequently died. Autopsy revealed a thickened tentorium with xanthochromic surface. This hypertrophic change was also seen in the dura mater of the posterior and middle cranial fossa. Microscopic examination of the thickened tentorium revealed extensive fibrous tissue with a chronic inflammatory infiltrate, predominantly of lymphocytes. No specific lesions were revealed by staining with hematoxylin-eosin, PAS, Gram's or Ziehl-Neelsen stains. The patient had no inflammatory or infectious diseases of other organs, and a diagnosis of idiopathic cranial hypertrophic pachymeningitis of unknown etiology was made. Considering the above findings, the thickened tentorium depicted as a hypointense area on the T1- and T2-weighted images and the Gd-enhanced edges of the tentorium are thought to be represent fibrous tissue and inflammatory regions, respectively.
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PMID:[A case of cranial hypertrophic pachymeningitis with intracranial hemorrhage]. 867 7


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