A rare case of cerebral vasculitis in Henoch‐Schönlein purpura with emphasis on the diagnostic value of magnetic resonance angiography (MRA) and single‐photon emission computed tomography (SPECT) given normal magnetic resonance imaging (MRI)

Department of Pediatrics, College of Medicine, Kyunghee University, Seoul, Korea.
International journal of dermatology (Impact Factor: 1.31). 07/2010; 49(7):803-5. DOI: 10.1111/j.1365-4632.2010.04478.x
Source: PubMed
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    ABSTRACT: Neurological complications are rare during the course of Henoch-Schönlein purpura (HSP). We report a 7-year-old girl with HSP who presented with seizures, loss of vision and disturbance of consciousness. Magnetic resonance imaging (MRI) showed high signal intensity in the gray and white matter over the left parietal and both occipital lobes, compatible with MRI findings of cerebral vasculitis. The eye fundi revealed multiple branches of retinal artery occlusion. Intravenous pulse methylprednisolone (MTP) followed by oral steroid therapy was initially administered for HSP nephritis. Cerebral vasculitis developed 10 days post-MTP treatment, with progressive worsening of consciousness. Oral steroid was discontinued and plasmapheresis was performed alone. Her level of consciousness dramatically improved after plasmapheresis. The brain MRI and eye fundi findings were consistent with her clinical improvement. To the best of our knowledge, this is the first description of MRI abnormalities and multiple retinal artery branch occlusion of cerebral vasculitis in a patient with HSP that was successfully treated by plasmapheresis alone. In conclusion, we propose that plasmapheresis may be used as a first-line therapy or rescue therapy for cerebral vasculitis in HSP.
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    ABSTRACT: Severe neurological complications in Henoch-Schonlein purpura (HSP) are rare. We report a 13-year-old girl with HSP receiving oral steroid therapy for HSP nephritis who presented with acute onset of headache, confusion, and loss of bilateral vision. However, immediate cranial computed tomography showed merely a small intracranial hematoma in the left occipital area, which was not consistent with her clinical manifestations. The subsequent magnetic resonance imaging of the brain demonstrated bilateral, multifocal abnormalities with features consistent with cerebral vasculitis. Therapeutic plasmapheresis was performed immediately and there was rapid clinical improvement. In conclusion, we propose that plasmapheresis is a reliable and effective therapy and may be considered as the first-line treatment in this critical condition to prevent massive intracerebral hemorrhagic complication of this immune complex-mediated cerebral vasculitis leading to irreversible neurological sequelae or mortality.
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