Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 36-year-old woman presented with acute-onset right lower extremity paresthesias, dysarthria, right facial droop, and right hemiparesis. CT and MR imaging of the brain revealed extensive white matter disease and left basal ganglia infarction with dural and leptomeningeal enhancement. Differential considerations included vasculitis, granulomatous disease, and neoplasm. Chest, abdomen, and pelvis CTs were normal. Right temporal lobe biopsy revealed noncaseating granulomatous inflammation consistent with neurosarcoidosis.
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PMID:Neurosarcoidosis presenting as acute infarction on diffusion-weighted MR imaging: summary of radiologic findings. 1721 30

Extra-axial restriction on diffusion weighted imaging (DWI) is an unusual finding on brain magnetic resonance imaging (MRI). Intra-axial restriction on DWI, however, is common, and can represent brain parenchymal infarction, tumor, abscess, or toxic-metabolic process. The infrequency of extra-axial DWI restriction and the paucity of clinico-pathological correlation in the literature limit its differential diagnosis. Scant case reports suggest that extra-axial DWI restriction could be a lymphoma, neurenteric cyst, or, in one patient, subdural empyema [1,2,3]. We postulate that pus formation must be excluded first, because it can provoke an aggressive meningo-vasculitis with rapidly fatal, intra-axial infarctions. Our patient was a 45-year-old man, presenting to our hospital with left facial droop and right (contralateral) arm and leg weakness. Initial MRI revealed DWI restriction in the left lateral pons, consistent with a classic Millard-Gubler stroke. Also noted was a subtle, extra-axial area of curvilinear diffusion restriction in the left cerebellar-pontine angle's subarachnoid space. Days later, the patient had a headache, and repeat MRI revealed extension of the two DWI lesions - both the intra-axial pontine infarction and the extra-axial area of restricted diffusion in the subarachnoid space. The patient became comatose, a third MRI revealed more extensive DWI restrictions, and he expired despite aggressive care. Autopsy revealed massive brainstem infarcts, a thick lymphoplasmacytic infiltrate, copious Gram-Positive cocci (likely MRSA) and arteries partially occluded with fibrointimal proliferation. This emphasizes the concept that extra-axial DWI restriction can represent pus development in the subarachnoid space - a radiographic marker to identify a patient at risk for demise due to septic, meningo-vasculitic infarctions.
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PMID:Restricted Diffusion of Pus in the Subarachnoid Space: MRSA Meningo-Vasculitis and Progressive Brainstem Ischemic Strokes - A Case Report. 2104 37

Giant cell arteritis (GCA) is the most common form of systemic vasculitis in adults. Patients usually present with headache and visual symptoms, and have an elevated erythrocyte sedimentation rate. It has been reported that 3-4% of patients with GCA develop ischemic events secondary to vertebral artery stenosis or occlusion. The mainstay of therapy of GCA is high dose steroid and/or methotrexate. A case is described of a patient who initially presented with intermittent double vision, mild headache and unremarkable MRI and MR angiography of the head and neck. The patient was diagnosed and treated for ocular myasthenia. The patient was readmitted 2 months later with imbalance and worsening headache, and workup suggested bilateral cerebellar infarction, complete occlusion of the left vertebral artery and a high grade stenosis of the right vertebral artery. Erythrocyte sedimentation rate and C reactive protein were elevated. Temporal artery biopsy demonstrated changes consistent with GCA. During the course of the treatment with corticosteroids and immunosuppressant, the patient developed dysarthria, left facial droop and left hemiplegia, and was found to have complete occlusion of both vertebral arteries. The patient was emergently taken for revascularization of the occluded segment using angioplasty and stent placement. The patient had significant improvement of neurological symptoms within 3 days after the procedure and continued to improve during hospitalization. Endovascular treatment of vasculitis affecting the intracranial vessels is not yet established. Our experience with successful treatment of complete occlusion of the vertebral artery secondary to GCA using endovascular intracranial angioplasty and stent placement is reported.
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PMID:Angioplasty and stent placement for complete occlusion of the vertebral artery secondary to giant cell arteritis. 2199 Apr 65

A 28-year-old man with a bicuspid aortic valve presented with facial droop and slurred speech with several months of constitutional symptoms of night sweats, weight loss and productive cough. Examination confirmed aortic regurgitation, palpable spleen and left facial droop. Multiple peripheral blood cultures were negative. Inflammatory markers, cytoplasmic staining antineutrophil cytoplasmic antibodies (cANCA) and anti-PR3 antibody were all elevated. MRI of the brain and CT of the chest and abdomen confirmed embolic infarcts to brain, kidney and spleen. Transoesophageal echocardiogram (ECG) showed valve vegetations and severe aortic regurgitation. Endocardial Wegener's granulomatosis was considered. Aortic valve replacement was performed. Grindings from aortic valve leaflets were analysed for rpoB gene, which confirmed the presence of Bartonella henselae. Serological assays demonstrated B henselae IgM 20 (normal <20) and IgG >2048 (normal < 64). The patient completely recovered after prolonged antibiotic treatment. Culture-negative infective endocarditis may mimic vasculitis and be associated with positive cANCA. Serology and molecular techniques may aid diagnosis.
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PMID:Bartonella henselae aortic valve endocarditis mimicking systemic vasculitis. 2279 85