{"title":"Granulomatous polyangiitis with pachymeningitis: a case report.","authors":"Whei Chuern Yeoh, Cristina Estrach, Jagdish Nair","doi":"10.1136/pn-2024-004463","DOIUrl":null,"url":null,"abstract":"<p><p>A 33-year-old man had 1 month of right-sided headache with multiple right-sided cranial neuropathies. He had a history of recurrent sinusitis, elevated inflammatory markers and positive cytoplasmic anti-neutrophilic cytoplasmic antigens (c-ANCA) anti-proteinase 3 (anti-PR3). MR scans of the brain and cervical spine identified an inflammatory mass at the skull base with hypertrophic pachymeningitis and a sigmoid sinus thrombosis. Cerebrospinal fluid examination excluded infection and a meningeal biopsy confirmed vasculitis. CT scan of the chest showed multiple lung nodules with cavitation. We gave intravenous cyclophosphamide and corticosteroids as induction therapy for granulomatosis with polyangiitis. Unfortunately, during the induction phase, he developed diffuse alveolar haemorrhage and progressive mononeuritis multiplex. We subsequently gave intravenous rituximab and C5a receptor antagonist, avacopan. He recovered well and remains in remission.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":""},"PeriodicalIF":2.4000,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"PRACTICAL NEUROLOGY","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1136/pn-2024-004463","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
A 33-year-old man had 1 month of right-sided headache with multiple right-sided cranial neuropathies. He had a history of recurrent sinusitis, elevated inflammatory markers and positive cytoplasmic anti-neutrophilic cytoplasmic antigens (c-ANCA) anti-proteinase 3 (anti-PR3). MR scans of the brain and cervical spine identified an inflammatory mass at the skull base with hypertrophic pachymeningitis and a sigmoid sinus thrombosis. Cerebrospinal fluid examination excluded infection and a meningeal biopsy confirmed vasculitis. CT scan of the chest showed multiple lung nodules with cavitation. We gave intravenous cyclophosphamide and corticosteroids as induction therapy for granulomatosis with polyangiitis. Unfortunately, during the induction phase, he developed diffuse alveolar haemorrhage and progressive mononeuritis multiplex. We subsequently gave intravenous rituximab and C5a receptor antagonist, avacopan. He recovered well and remains in remission.
期刊介绍：
The essential point of Practical Neurology is that it is practical in the sense of being useful for everyone who sees neurological patients and who wants to keep up to date, and safe, in managing them. In other words this is a journal for jobbing neurologists - which most of us are for at least part of our time - who plough through the tension headaches and funny turns week in and week out. Primary research literature potentially relevant to routine clinical practice is far too much for any neurologist to read, let alone understand, critically appraise and assimilate. Therefore, if research is to influence clinical practice appropriately and quickly it has to be digested and provided to neurologists in an informative and convenient way.
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