Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T1- and T2-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/microl) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out. Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI. On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T1-weighted MRI images and was also enhanced by gadolinium. Cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia.
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PMID:Hypertrophic cranial pachymeningitis in a patient with aplastic anemia. 987 65

Rheumatoid arthritis is a systemic disease in which cerebral and eye involvement is neither common nor fully understood. Although it is rarely the cause of pachymeningitis and optic neuritis, rheumatoid arthritis should always be kept in mind in these two conditions. We present a 52-year-old male with an 8 month history of rheumatoid arthritis who was referred to the neurology department with headache and decreasing vision and was diagnosed as having rheumatoid pachymeningitis and optic neuritis on the basis of MRI findings.
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PMID:Pachymeningitis and optic neuritis in rheumatoid arthritis: MRI findings. 1047 6

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.
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PMID:[An old man presenting with fluctuating bilateral multiple cranial nerve palsies and positive test for perinuclear antineutrophil cytoplasmic antibody]. 1051 63

We reported two cases of silicosis exhibiting MPO-ANCA associated disorder. Case 1 was a 69 year-old man with silicosis and chronic interstitial pneumonia. He was admitted because of fever, dry cough, left chest pain, dyspnea and body weight loss. He was diagnosed as acute exacerbation of interstitial pneumonia, pericarditis and gastrointestinal bleeding. Case 2 was a 67 year-old man with silicosis. He repeated attack of fever, hoarseness, dysphagia and headache. The cell counts of cerebrospinal fluid increased and the thickness of cerebellar tent and left dura mater was observed in the brain MRI. Therefore, he was diagnosed as pachymeningitis and neuropathy of cranial nerves. Both cases were complicated by silicosis and the laboratory findings showed high serum levels of P-ANCA, ANA and rheumatoid factor and inflammatory responses, indicating they were suspected vasculitis. The two cases were treated by steroid and immunosuppressive therapy and had good clinical response. Silicosis may affect multiple organ involvement associated with P-ANCA.
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PMID:[Two cases of silicosis exhibiting MPO-ANCA associated disorder]. 1069 8

We report on a 48-year-old man with idiopathic hypertrophic cranial pachymeningitis (IHCP) manifesting headache, facial pain, and tongue pain with unilateral atrophy, dysarthria, and dysphagia. Although steroid therapy ameliorated these symptoms, they recurred after he developed steroid-induced diabetes mellitus. We treated the patient by lymphocytapheresis (LCP), which resulted in an improvement of his symptoms, a reduction in the CD4 lymphocyte population, a reduction of the CD4/CD8 ratio, and a reduced thickening of the dura mater that lasted for more than 14 months. Results presented here suggest that LCP can be effective in the treatment of IHCP.
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PMID:Long-term improvement of idiopathic hypertrophic cranial pachymeningitis by lymphocytapheresis. 1097 80

Hypertrophic cranial pachymeningitis (HCP) is a rare inflammatory disease of unknown origin in which recurrence is frequently observed in spite of the initial response to steroid therapy. Three patients, 1 man and 2 women, aged 63, 66, and 67 years, with severe intractable headache were evaluated by brain MRI. All patients were initially given prednisolone (60 mg/day, oral) or dexamethasone (20 mg/day, i.v.), and followed by long-term (at least 1 year) azathioprine therapy. All patients were evaluated by follow-up laboratory tests and brain MRI study, and completed the 2-year follow-up period. Symptoms including headache were initially improved with corticosteroid therapy, but patients became steroid-dependent. Azathioprine administration in these steroid-dependent patients permitted the complete cessation of corticosteroid and led to the clinical and radiological recovery. In conclusion, initial high-dose corticosteroid administration followed by long-term azathioprine therapy may be the ideal treatment of HCP at present.
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PMID:Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis. 1109 16

In cranial pachymeningitis, headache, cranial nerve palsies, and ataxia were the most common clinical manifestations. We reported two rare cases presenting only headache or convulsion as a main symptom. A 66-year-old man was suffering from severe continuous headache for years with no other clinical symptoms. Laboratory data revealed positive RA test and elevated RAPA. With oral steroid therapy, his headache was subsided. A 39-year-old woman visited us with convulsive attacks as only symptom. Biopsy of the thickened dura revealed granulated epithelioid cells without central necrosis. With antituberculosis therapy, hypertrophy of dura, as well as the symptom, was disappeared. It is difficult to diagnose pachymeningitis when patients manifest only headache or convulsion. We suggest to carry out Gd-enhanced MR scan in these cases.
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PMID:[Two cases of cranial pachymeningitis presenting only severe continuous headache or convulsion]. 1121 64

Tumors of the central nervous system are an unusual cause of sudden death. This report describes the sudden death of a presumed healthy 28-year-old woman from primary diffuse leptomeningeal gliomatosis. She presented to an emergency room with headache and vomiting, subsequently became unresponsive and was pronounced dead 14 h later. Autopsy revealed a diffuse extensive infiltrate of well-differentiated astrocytoma in the leptomeninges of the brain and spinal cord without an underlying parenchymal tumor. Primary diffuse leptomeningeal gliomatosis is a rare tumor that arises within the leptomeninges from small neuroglial heterotopic rests that undergo neoplastic transformation. Grossly. this tumor can mimic leptomeningeal carcinomatosis, pachymeningitis, tuberculosis, sarcoidosis, and fungal infections. However, the histologic features of primary diffuse leptomeningeal gliomatosis should allow it to be readily distinguished from grossly similar conditions. The mechanism of death in this case is most likely tumor obstruction of cerebrospinal fluid outflow resulting in the usual complications seen with increased intracranial pressure. Although this tumor is aggressive and is associated with a rapidly progressive fatal course, it has not been previously associated with sudden death.
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PMID:Sudden death due to primary diffuse leptomeningeal gliomatosis. 1130 49

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.
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PMID:Idiopathic hypertrophic cranial pachymeningitis. 1196 Jan 42

We reported a 64-years-old woman with pachymeningitis associated with a ruptured mycotic cerebral aneurysm due to Aspergillus infection. She had suffered from diabetes mellitus and been treated since she was 49 years old. She complained of headache at the age of 62 and loss of her left visual acuity three months later. She was treated by the pulse therapy of methylprednisolone as neuritis retrobulbaris and her visual acuity recovered. But her headache continued. Three months later, her right visual acuity was lost, and the pulse therapy was not effective this time. Six months later, she died of subarachnoid hemorrhage following acute meningitis. The autopsy was granted, but limited to the cranial cavity. Macroscopically, it disclosed brownish thickened dura around sella turucica involving trigeminal ganglion and optic nerve, and fresh subarachnoid hemorrhage in the basal cisterns and a ruptured aneurysm (3 mm in diameter) between internal carotid and posterior cerebral artery on the left side. Histologically, the brownish thickened dura was infiltrated by lymphocytes, plasma cells, and multinucleated giant cells. The wall around the aneurysm was infiltrated by lymphocytes and plasma cells as well as many fungi. Immunohistochemistry demonstrated the presence of Aspergillus in the thickened dura and the arterial wall around the aneurysm. There were lymphocytes and plasma cell infiltration in the basal subarachnoid space and scattered microabcesses in the brain. Although the first entry of Aspergillus to the dura was unclear, we assume that the final intravascular dissemination of Aspergillus from the dura caused meningitis and mycotic aneurysm.
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PMID:[An autopsied case of pachymeningitis associated with a ruptured, cerebral aneurysm due to Aspergillus infection]. 1199 87


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