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)

The idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome typically occurs in young patients and may produce multiple retinal macroaneurysms, neuroretinitis, and peripheral capillary nonperfusion. Optic disc edema has been described, but elevated intracranial pressure has not been previously documented. We report a case of a 12-year-old girl who presented with bilateral disc swelling and peripapillary hemorrhage. Brain magnetic resonance imaging (MRI) was normal, but lumbar puncture yielded an opening pressure of 360 mm H2O with normal constituents. Fluorescein angiography delineated saccular aneurysms of the retinal arteriolar vasculature, and IRVAN syndrome was diagnosed. MR venography disclosed poor filling of both transverse venous sinuses. Acetazolamide treatment of 14 months did not alter the fundus findings. IRVAN syndrome may present initially with optic nerve swelling and elevated intracranial pressure with subsequent development of the characteristic retinal vascular abnormalities.
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PMID:Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. 1534 89

The patient (woman), 54 years of age, treated for p-ANCA positive vasculitis, hypertension, and thyroid gland hypofunction was referred to our Department due to the decrease of the visual acuity of her left eye. Since the year 2003, she was followed-up in the Department of Neurology due to the idiopathic intracranial hypertension (the opening pressure of the intracranial liquid was 480 -500 mm of H2O column), treated by acetazolamide tablets (Diluran) 1/2 tablet once daily. She was followed-up for the bilateral persistent papilledema by the local ophthalmologist. Due to the progression of the visual acuity loss the decompression (fenestration) of the optic nerve sheath was performed, and the neurosurgeon recommended the lumbo-peritoneal shunting. Adequate indication and choice of the surgical treatment helped to stabilize visual functions.
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PMID:[Benefit of the surgical treatment of the idiopathic intracranial hypertension--a case report]. 2005 24