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 present a 66-year-old woman undergoing hemodialysis who developed intracranial hypertrophic pachymeningitis. Neurological examinations revealed a loss of bilateral visual acuity with optic atrophy, headache, and markedly restricted bilateral extraocular movement. MRI examinations demonstrated homogenous hypertrophic dural enhancement compatible with hypertrophic cranial pachymeningitis, and biopsied dural specimen revealed chronic inflammatory changes with proliferation of dense collagen fibers. There was no direct evidence of vasculitis and specific infections including tuberculosis and troponema pallidum. Most of the inflammatory infiltrates were demonstrated to be T lymphocytes. Intriguingly, p-ANCA was found to be highly elevated at x 220 and decreased to x 110 after steroid treatment. Neurological manifestations and radiological findings also improved in accordance with the lowering of p-ANCA. Although a few reports have described similar conditions such as chronic renal failure accompanying hemodialysis and pachymeningitis, and though vasculitis was not depicted histologically in this patient, we considered that immunological mechanisms probably provoked the patient's glomerulonephritis and pachymeningitis. Additionally, positive reaction against hepatitis c virus might have influenced the immunological system leading to the occurrence of the pachymeningitis.
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PMID:[Intracranial hypertrophic pachymeningitis with high perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) occurred in a patient on hemodialysis]. 1247 97

Spinal anaesthesia developed in the late 1800s with the work of Wynter, Quincke and Corning. However, it was the German surgeon, Karl August Bier in 1898, who probably gave the first spinal anaesthetic. Bier also gained first-hand experience of the disabling headache related to dural puncture. He correctly surmised that the headache was related to excessive loss of cerebrospinal fluid (CSF). In the last 50 yr, the development of fine-gauge spinal needles and needle tip modification, has enabled a significant reduction in the incidence of post-dural puncture headache. Though it is clear that reducing the size of the dural perforation reduces the loss of CSF, there are many areas regarding the pathogenesis, treatment and prevention of post-dural puncture headache that remain contentious. How does the microscopic pattern of collagen alignment in the spinal dura affect the dimensions of the dural perforation? How do needle design, size and orientation influence leakage of CSF through the dural perforation? Can pharmacological methods reduce the symptoms of post-dural puncture headache? By which mechanism does the epidural blood patch cure headache? Is there a role for the prophylactic epidural blood patch? Do epidural saline, dextran, opioids and tissue glues reduce the rate of CSF loss? This review considers these contentious aspects of post-dural puncture headache.
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PMID:Post-dural puncture headache: pathogenesis, prevention and treatment. 1513 91

Over a 5-year period, we investigated 77 consecutive patients (36 males, 41 females, mean age 40.9 years) referred to our hospital with the diagnosis of CNS vasculitis. Extensive workup including MRI, echocardiography, laboratory tests, angiography ( n=53), and biopsies at appropriate sites ( n=26) was performed based on individual history and symptoms. Prominent symptoms were stroke ( n=61), encephalopathy ( n=14), and headaches ( n=2). Vasculitis was finally diagnosed in 13 patients (17%) including isolated angiitis of the CNS ( n=3), giant cell arteritis ( n=4), and septic arteritis ( n=3). Thirty-two patients (42%) presented noninflammatory vasculopathies including moyamoya ( n=6), Sneddon's syndrome ( n=5), dissection ( n=4), CADASIL ( n=2), and collagen vascular disease ( n=9). Coagulopathy was found in 14 cases (18%) including antiphospholipid syndrome ( n=8) and APC resistance ( n=4). Other causes were cardiogenic embolism ( n=8), multiple sclerosis ( n=5), and migraine stroke ( n=3). Only a minority of patients referred for evaluation of suspected CNS vasculitis actually present with inflammatory vascular disease. Main differential diagnosis includes noninflammatory vasculopathies, coagulopathies, and cardiac disease. Since septic processes may be responsible for the symptoms, "blind" treatment with immunosuppressive agents should be strictly avoided.
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PMID:[Diagnosis and differential cerebral vasculitis diagnosis]. 1477 Feb 79

Pulmonary arterial hypertension (PAH) is a rare debilitating disease characterized by an increase in pulmonary vascular resistance and progressive right ventricular failure. PAH may be primary or associated with other conditions such as collagen vascular disease, portal hypertension, and HIV. Intravenous epoprostenol improves the survival, exercise tolerance, hemodynamics, and quality of life in patients with PAH and is believed to work through multiple pathways including vasodilation, opposition of smooth-muscle hypertrophy, and inhibition of platelet aggregation. Common dose-limiting side effects are flushing, jaw pain, arthralgias, myalgias, and headache, which are attributed to the vasodilatory effects of epoprostenol. In clinical practice, patients often develop persistent rash that is distinct from the flushing associated with epoprostenol. The specific findings both on physical examination and on dermatopathology have not, however, been well described. This report describes the cutaneous and dermatopathologic findings of 12 patients who developed persistent rash while receiving long-term prostacyclin for PAH.
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PMID:Cutaneous findings in patients with pulmonary arterial hypertension receiving long-term epoprostenol therapy. 1524 33

The authors present the case of a 50-year-old woman with a history of CREST syndrome (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia), a variant of scleroderma, who was incidentally found to have an irregular intracranial aneurysm. The patient presented with migraine headaches. A magnetic resonance image of the brain obtained during the headache workup revealed a right posterior carotid artery wall aneurysm in the region of the anterior choroidal artery (AChA). On digital subtraction angiograms, the lesion measured 3.5 mm at its largest diameter. Because of the irregular shape of the aneurysm, the patient's relatively young age, and the potential for further aneurysm growth due to collagen disease, surgical clip application was recommended following a discussion of available treatment options. At surgery, the aneurysm was identified as bilobed and broad based, and the AChA was found to be associated with the aneurysm neck. Satisfactory clipping of the aneurysm was achieved with preservation of the parent vessels. An association of CREST syndrome with intracranial aneurysms has only been reported once before. This case is presented to draw attention to the possibility of a pathophysiological connection between CREST syndrome and intracranial aneurysms and to postulate a possible mechanism whereby this condition may result in aneurysm formation. The association of aneurysms with other pathological collagen-related conditions is well known, and literature relevant to a possible connection between CREST syndrome and aneurysms is reviewed and discussed.
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PMID:Association of an irregularly shaped anterior choroidal aneurysm with CREST syndrome. Case report. 1554 Sep 26

Primary extraskeletal osteosarcoma occurring in the brain parenchyma is distinctly uncommon, with only five cases having been reported. The authors describe the case of a 45-year-old man who presented with progressive headache and diplopia. Computerized tomography scanning and magnetic resonance imaging results revealed a pineal region tumor with obstructive hydrocephalus. The patient underwent partial resection of the tumor. The histological examination showed large pleomorphic tumor cells embedded in osteoid matrix. Immunohistochemical analysis was negative for various antibodies and thus excluded a glial, germ cell, epithelial, and lymphoid tumor origin. Only vimentin showed strong positivity in most of the tumor cells. Ultrastructurally, the tumor cells were rich in dilated rough endoplasmic reticula. Clear zones between tumor cells and osteoid matrix were observed. The osteoid matrix was made up of small collagen fibrils and hydroxyapatite deposits. The tumor was not attached to the bone structure of the skull. These findings are consistent with the features of extraskeletal osteosarcoma. Data from complete medical and radiological studies excluded a metastatic origin for this tumor. Partial resection and postoperative radiotherapy had provided tumor control at 11 months after the onset of symptoms. This is the first reported case of a primary extraskeletal osteosarcoma occurring in the pineal region.
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PMID:Primary extraskeletal osteosarcoma in the pineal region. Case report. 1559 71

This is a case report of pulmonary sarcoidosis presenting with acute respiratory failure. A 33-year-old black man presented with 4 days of headache and fever. He had subsequent development of respiratory failure on the seventh hospital day. Workup showed high serum angiotensin-converting enzyme and increased pulmonary parenchymal uptake of gallium. Extensive workup for collagen vascular disease and infectious pathology were negative. Transbronchial biopsy specimens of the lung parenchyma showed noncaseating epithelioid granulomas consistent with sarcoidosis. The patient was treated with intravenous methylprednisolone succinate with dramatic clinical improvement.
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PMID:Pulmonary sarcoidosis presenting with acute respiratory failure. 1581 51

D-003 is a mixture of high molecular weight aliphatic primary acids purified from sugar cane wax with antiplatelet and cholesterol-lowering effects. Previous studies showed that D-003 (10-20 mg/day) administered for a short time inhibits platelet aggregation, 14 days being the longest duration investigated. This study was conducted to investigate the effects of D-003 (5 and 10 mg/day) for 30 days on platelet aggregation in normocholesterolemic subjects. This report shows the effects of D-003 on platelet aggregation to arachidonic acid (AA) (1.5 mM), collagen (2 microg/ml) and adenosine 5'-diphosphate ADP (2 microM) assessed at baseline and at treatment completion. Fifty-four subjects were randomized to placebo or D-003 (5 or 10 mg/day) for 30 days. Platelet aggregation to AA, collagen and ADP were assessed. D-003 at the lowest dose (5 mg/day) significantly but modestly inhibited (p < 0.01) platelet aggregation to AA (5.0%) and (p < 0.01) to collagen (7.5%). D-003 at 10 mg/day inhibited (p < 0.001) platelet aggregation to AA and collagen (p < 0.01) by 20.3% and 14.7%, respectively. ADP-induced aggregation, however, was unchanged. D-003 at 10 mg/day, but not at 5 mg/day, lowered (p < 0.01) plasma fibrinogen. D-003 (5 and 10 mg/day) reduced low-density lipoprotein cholesterol (LDL-C) by 17.7% and 26.4%, respectively, and total cholesterol (TC) by 14.5% and 18.5%, while at 10 mg/day, but not at 5 mg/day, it increased high-density lipoprotein cholesterol (HDL-C) by 9.6%. Triglycerides, however, were unchanged with D-003. No disturbances in safety indicators were induced with D-003. One subject (D-003 5 mg/day) discontinued the study and four patients (three taking D-003 and one taking placebo) reported adverse effects (AE) (headache in two patients taking D-003 and one patient taking placebo, and polyphagia in one patient taking D-003). In conclusion, D-003 (5-10 mg/day) for 30 days inhibited platelet aggregation to AA and collagen but not to ADP Therefore, the antiplatelet effect was present with the longer treatment, even at a dose of 5 mg/day. The cholesterol-lowering effects of D-003 were consistent with those expected for such a short treatment. In addition, D-003 at 10 mg/day significantly lowered plasma fibrinogen. The treatment was well tolerated.
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PMID:Effects of D-003, a mixture of very long chain fatty acids purified from sugar cane wax, at 5 and 10 mg/day on platelet aggregation in healthy volunteers. 1586 55

We present the case of a distal anterior inferior cerebellar artery (AICA) aneurysm masquerading as a cerebellopontine angle tumor in a 60-year-old right-handed man with previously undiagnosed polyarteritis nodosa (PAN). The patient presented with a 2-month history of progressive right-sided hearing loss, intermittent severe headache, and sudden onset of complete facial paralysis 3 weeks before admission. Magnetic resonance imaging, including post-gadolinium images, showed a 1.2-cm heterogeneously enhancing mass that slightly enlarged the right internal auditory canal. A right suboccipital craniotomy was performed, and a partially thrombosed fusiform AICA aneurysm was discovered just anterior to the VII/VIII nerve complex. The aneurysm was trapped and opened, and a thrombectomy was performed. Postoperatively, the patient experienced abdominal pain; liver function tests were abnormal. Investigation revealed a small retroperitoneal hemorrhage and aneurysms of the celiac axis and gastroduodenal arteries. Further investigation revealed an increased erythrocyte sedimentation rate, and a diagnosis of PAN was made. PAN is a well-identified factor in the genesis of peripheral vascular aneurysms. Aneurysms involving the hepatic, renal, coronary, pancreatic, and tibial arteries have been described. PAN is an extremely rare cause of intracranial aneurysm. Patients who present with aneurysms in unusual locations (e.g., distal AICA) should be investigated for vasculopathy and collagen vascular disorders.
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PMID:Distal anterior inferior cerebellar artery aneurysm masquerading as a cerebellopontine angle tumor: case report and review of literature. 1614 91

We encountered a 10-year-old girl with fluctuating sensorineural hearing loss, episodic headache, and white matter stroke. Strenuous exercise, febrile illness, and general anesthesia all temporarily worsened hearing. Audiologic findings were asymmetric: left-sided retrocochlear dysfunction consistent with auditory neuropathy contrasted with cochlear hearing loss in the right ear. Platelets obtained during a headache-free period showed excessive responsiveness to collagen in vitro, while episodic elevations of thromboxane B(2) and thrombin-antithrombin III complex were noted in blood sampled during headache. Treatment of hyperaggregability of platelets with aspirin and antioxidant vitamins relieved headache, while adenosine triphosphate administration improved hearing thresholds. In this patient, hearing impairment and white matter strokes appeared to respectively related to impaired blood flow to the cochlea and white matter caused by platelet dysfunction triggered by physiologic stresses.
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PMID:Fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability: coexistence of auditory neuropathy and cochlear hearing loss. 1616


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