Abel T Oommen, Arunodaya R Gujjar, Suad Al-Jahdhami, Abdullah R Al-Asmi, Ali Sajjad
{"title":"抗中性粒细胞细胞质抗体相关的中枢和周围神经系统血管炎。","authors":"Abel T Oommen, Arunodaya R Gujjar, Suad Al-Jahdhami, Abdullah R Al-Asmi, Ali Sajjad","doi":"10.18295/2075-0528.2820","DOIUrl":null,"url":null,"abstract":"<p><p>The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) usually manifests as systemic vasculitis. AAV with central nervous system involvement in the form of mesenrhombencephalitis and peripheral neuropathy is extremely rare. We report a 59-year-old male patient who presented to a tertiary care hospital in Muscat, Oman, in 2024. He had a subacute presentation of altered sensorium, multiple cranial nerve palsy and ataxia with 3 months history of bilateral foot drop. Brain imaging was suggestive of mesenrhombencephalitis with subarachnoid and intraparenchymal haemorrhage. Nerve conduction studies revealed sensorimotor axonal neuropathy. Vasculitis profile showed perinuclear-ANCA positivity and the nerve biopsy was suggestive of vasculitic neuropathy. He was treated with intravenous steroids and maintained on rituximab. His neurological deficits improved with residual bilateral foot drop.</p>","PeriodicalId":22083,"journal":{"name":"Sultan Qaboos University Medical Journal","volume":"25 1","pages":"282-287"},"PeriodicalIF":0.0000,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240024/pdf/","citationCount":"0","resultStr":"{\"title\":\"Anti-Neutrophil Cytoplasmic Antibody-Associated Central and Peripheral Nervous System Vasculitis.\",\"authors\":\"Abel T Oommen, Arunodaya R Gujjar, Suad Al-Jahdhami, Abdullah R Al-Asmi, Ali Sajjad\",\"doi\":\"10.18295/2075-0528.2820\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) usually manifests as systemic vasculitis. AAV with central nervous system involvement in the form of mesenrhombencephalitis and peripheral neuropathy is extremely rare. We report a 59-year-old male patient who presented to a tertiary care hospital in Muscat, Oman, in 2024. He had a subacute presentation of altered sensorium, multiple cranial nerve palsy and ataxia with 3 months history of bilateral foot drop. Brain imaging was suggestive of mesenrhombencephalitis with subarachnoid and intraparenchymal haemorrhage. Nerve conduction studies revealed sensorimotor axonal neuropathy. Vasculitis profile showed perinuclear-ANCA positivity and the nerve biopsy was suggestive of vasculitic neuropathy. He was treated with intravenous steroids and maintained on rituximab. His neurological deficits improved with residual bilateral foot drop.</p>\",\"PeriodicalId\":22083,\"journal\":{\"name\":\"Sultan Qaboos University Medical Journal\",\"volume\":\"25 1\",\"pages\":\"282-287\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-05-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240024/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Sultan Qaboos University Medical Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.18295/2075-0528.2820\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Sultan Qaboos University Medical Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.18295/2075-0528.2820","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Anti-Neutrophil Cytoplasmic Antibody-Associated Central and Peripheral Nervous System Vasculitis.
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) usually manifests as systemic vasculitis. AAV with central nervous system involvement in the form of mesenrhombencephalitis and peripheral neuropathy is extremely rare. We report a 59-year-old male patient who presented to a tertiary care hospital in Muscat, Oman, in 2024. He had a subacute presentation of altered sensorium, multiple cranial nerve palsy and ataxia with 3 months history of bilateral foot drop. Brain imaging was suggestive of mesenrhombencephalitis with subarachnoid and intraparenchymal haemorrhage. Nerve conduction studies revealed sensorimotor axonal neuropathy. Vasculitis profile showed perinuclear-ANCA positivity and the nerve biopsy was suggestive of vasculitic neuropathy. He was treated with intravenous steroids and maintained on rituximab. His neurological deficits improved with residual bilateral foot drop.