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      Unusual presentation of Takayasu's arteritis as posterior reversible encephalopathy syndrome

      case-report

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          Abstract

          Takayasu's arteritis is a chronic, idiopathic, inflammatory disease primarily affecting aorta and its branches. It mainly affects young females in the age group of 10-30 years. Various atypical presentations of Takayasu's arteritis have been described in children. These atypical presentations can cause delayed diagnosis resulting in increased morbidity and mortality. Posterior reversible encephalopathy syndrome (PRES) is a neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging. We report a child with Takayasu's arteritis who presented a posterior reversible encephalopathy syndrome. He also had associated abdominal tuberculosis for which anti-tuberculous treatment was started. PRES was diagnosed by magnetic resonance imaging with fluid-attenuated inversion recovery sequences. The child was started on nifedipine and propranolol. The child regained his consciousness within 48 h of admission. Prompt treatment of hypertension led to rapid reversal of neurological symptoms. In view of hypertension a computed tomography aortogram was done, which showed features suggestive of high grade (>75%) focal proximal left renal artery stenosis. EULAR (European League Against Rheumatism)/PReS (Paediatric Rheumatology European Society) consensus criteria was used for the diagnosis of Takayasu's arteritis in our patient. Percutaneous transluminal balloon angioplasty of the stenotic left renal artery was performed. Post-angioplasty, nifedipine was gradually omitted and oral propranolol was continued.

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          Most cited references16

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          Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions.

          Posterior reversible encephalopathy syndrome (PRES) is typically characterized by headache, altered mental functioning, seizures, and visual loss associated with imaging findings of bilateral subcortical and cortical edema with a predominantly posterior distribution. Our goal was to determine whether fluid-attenuated inversion recovery (FLAIR) imaging improves the ability to detect subtle peripheral lesions of PRES, as compared with conventional MR techniques. Sixteen patients with clinical and imaging findings consistent with PRES were studied. Thirteen patients had undergone transplantation and had cyclosporin A neurotoxicity. Fast-FLAIR images were compared with spin-echo proton density- and T2-weighted images. FLAIR imaging improved diagnostic confidence and conspicuity of the T2 hyperintense lesions of PRES, typically in the subcortical white matter of the parietooccipital regions bilaterally. On all 23 abnormal MR studies, FLAIR was judged superior to proton density- and T2-weighted images for the detection of PRES in the supratentorial brain. In a mean of 6.7 of 23 studies, FLAIR findings prompted a raise in the grade of disease severity. FLAIR also showed cortical involvement in 94% of patients with PRES and in a mean of 46% of the total lesion burden. In four cases, subtle lesions were virtually undetectable without FLAIR. Brain stem or cerebellar disease was encountered in 56% of patients. FLAIR improves the ability to diagnose and detect subcortical and cortical lesions in PRES as compared with proton density- and T2-weighted spin-echo images. We therefore believe that FLAIR should be performed in patients with suspected PRES to allow more confident recognition of the often subtle imaging abnormalities.
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            Takayasu's arteritis: a review of the literature.

            Takayasu's arteritis is a rare, idiopathic, chronic inflammatory disease with cell-mediated inflammation, involving mainly the aorta and its major branches. It leads to stenosis, occlusion or aneurysmal degeneration of large arteries. The clinical presentation is characterised by an acute phase with constitutional symptoms, followed, months or years later, by a chronic phase in which symptoms relate to fibrosis or occlusion of vessels. Angiography is the gold standard for diagnosis and for topographical classification and it correlates with symptoms and prognosis. Here we focus on the pathophysiology, clinical and angiographical classification, diagnostic assessment and therapeutic approach of Takayasu's arteritis.
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              Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathy: three names for an old syndrome.

              Children with hypertension, seizures, lethargy, encephalopathy, headache, and occipital blindness are reviewed. After undergoing antihypertensive therapy, most children improve. Some patients have a similar syndrome associated with chemotherapy, transplantation, transfusion, or human immunodeficiency virus-1 (HIV-1) infection. These latter children can develop symptoms with only minimal or no discernible elevations in blood pressure and improve, in the case of cancer-associated encephalopathy, after discontinuing chemotherapy. The reported children with this distinctive clinical condition are compared to adults with reversible posterior leukoencephalopathy syndrome. Since both gray and white matter are involved, we had suggested previously that the name be changed to (reversible) occipitoparietal encephalopathy syndrome. However, reversible posterior leukoencephalopathy has been used in the adult population and probably should be employed in children for the sake of uniformity, since both children and adults have the same clinical presentation and presumably a similar pathophysiology for the encephalopathy syndrome. The diagnosis is confirmed by reversible posterior abnormalities seen on T2-weighted brain magnetic resonance imaging, and by the presence of either headache, altered mental status, seizures, or visual disturbances.
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                Author and article information

                Journal
                Ann Indian Acad Neurol
                AIAN
                Annals of Indian Academy of Neurology
                Medknow Publications (India )
                0972-2327
                1998-3549
                Jul-Sep 2011
                : 14
                : 3
                : 214-216
                Affiliations
                [1]Department of Pediatrics, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai, India
                Author notes
                For correspondence: Dr. Syed Ahmed Zaki, Room No. 509, New RMO Quarters, LTMG Hospital, Sion, Mumbai - 400 022, India. E-mail: drzakisyed@ 123456gmail.com
                Article
                AIAN-14-214
                10.4103/0972-2327.85900
                3200050
                22028540
                3d5c3771-bba2-4237-a0ed-1cccd2698ea2
                Copyright: © Annals of Indian Academy of Neurology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 19 April 2010
                : 22 June 2010
                : 12 August 2010
                Categories
                Case Report

                Neurology
                tuberculosis,posterior reversible encephalopathy syndrome,takayasu arteritis
                Neurology
                tuberculosis, posterior reversible encephalopathy syndrome, takayasu arteritis

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