2008
DOI: 10.1007/s10165-008-0052-1
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Reversible posterior leukoencephalopathy syndrome: a possible manifestation of Wegener’s granulomatosis-mediated endothelial injury

Abstract: We present the case of a 15-year-old girl who had Wegener's granulomatosis with severe intestinal involvement. During the clinical course, she developed generalized seizures and was diagnosed with reversible posterior leukoencephalopathy syndrome (RPLS). Plasma exchange combined with steroid pulse therapy was initiated and showed marked improvement. This is one of the few cases of RPLS without severe hypertension or renal failure, suggesting that RPLS is likely to be a manifestation of Wegener's granulomatosis… Show more

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Cited by 14 publications
(8 citation statements)
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“…Corticosteroids with mineral corticoid effects, in particular, may cause hypertension and induce RPLS. On the other hand, some RPLS patients were treated with high-dose methylprednisolone, cyclophosphamide, and/or plasmapheresis as final resolution [9,14,16,18,20,[25][26][27][28]32], as shown in Table 1. There is controversy regarding the relationship of these agents to RPLS.…”
Section: Discussionmentioning
confidence: 99%
“…Corticosteroids with mineral corticoid effects, in particular, may cause hypertension and induce RPLS. On the other hand, some RPLS patients were treated with high-dose methylprednisolone, cyclophosphamide, and/or plasmapheresis as final resolution [9,14,16,18,20,[25][26][27][28]32], as shown in Table 1. There is controversy regarding the relationship of these agents to RPLS.…”
Section: Discussionmentioning
confidence: 99%
“…However, steroids are also used in the treatment of vasogenic edema, such as in patients with intracranial mass lesions [9]. Case reports have implicated steroids as both the precipitant [5, 8, 12, 19, 20, 23, 24, 30, 31, 34, 38, 47, 48] and treatment [1, 6, 8, 12, 13, 18, 25, 28, 30, 33, 36, 37] of PRES. Thus, the role of steroids in PRES remains unclear.…”
Section: Introductionmentioning
confidence: 99%
“…To our knowledge, only four cases of PRES associated with ANCA-associated vasculitis have been reported in the literature [4][5][6]. There have been as well other reports of PRES in patients with diagnosed vasculitis including Wegener's, Takayasu's, and Polyarteritis Nodosa, in which similar mechanisms can be theorized as pathogenic in development of PRES.…”
Section: Discussionmentioning
confidence: 81%