F. Nasim, M. Fasrina, Sunil Bowattage, Wickramasooriyage K. Kularatne, V. J. Kobbegala
{"title":"一名对皮质类固醇反应良好的年轻女性患上急性出血性白质脑炎","authors":"F. Nasim, M. Fasrina, Sunil Bowattage, Wickramasooriyage K. Kularatne, V. J. Kobbegala","doi":"10.4038/cmj.v68i1.9677","DOIUrl":null,"url":null,"abstract":"Background: Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant entity of acute disseminated leukoencephalitis (ADEM) that has a rapidly progressive polysymptomatology corresponding to areas of brain affected. It’s characterized by rapid cognitive decline, coma or even rapid death. It is postulated to be a post infective immune mediated monophasic demyelinating disease. Diagnosis is clinical supported by CSF and MRI findings. It is challenging to differentiate the disease from MDEM (multiphasic demyelinating encephalomyelitis) and multiple sclerosis during the initial presentation.Case presentation: Here we report a case of a 36-yearold Sri Lankan Sinhalese lady who presented with seizure following febrile illness followed by gross right sided hemiparesis later progressing to mutism and cognitive decline. Her CSF showed pleocytosis 12/cumm with 5% polymorphs, red cells, mildly high proteins with normal glucose and positive oligoclonal bands. MRI showed evidence of multiple focal supra and infratentorial signal abnormalities and gyral thickening with hemorrhages, largest lesions being in left frontal region with mass effect but no herniation. She was diagnosed with AHLE and treated with high dose IV methyl prednisolone later converting to oral prednisolone tapering regime. She was continued on intravenous cefotaxime and acyclovir as per meningoencephalitis. Improvement with steroids was dramatic with complete neurological recovery, minimal sequel and no recurrences at 6 months follow up.Conclusion: The case is a representation of a very rare entity of an infrequently reported disease which needs high degree of clinical suspicion with early imaging for aggressive and early immunosuppressive treatment that may hinder progression to commonly described sequel including coma, death or residual neurology. The predictions on future MDEM or MS on first presentation and overlap are areas that need further exploration.","PeriodicalId":9777,"journal":{"name":"Ceylon Medical Journal","volume":null,"pages":null},"PeriodicalIF":0.3000,"publicationDate":"2023-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Acute hemorrhagic leukoencephalitis in a young female with excellent response to corticosteroids\",\"authors\":\"F. Nasim, M. Fasrina, Sunil Bowattage, Wickramasooriyage K. Kularatne, V. J. Kobbegala\",\"doi\":\"10.4038/cmj.v68i1.9677\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Background: Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant entity of acute disseminated leukoencephalitis (ADEM) that has a rapidly progressive polysymptomatology corresponding to areas of brain affected. It’s characterized by rapid cognitive decline, coma or even rapid death. It is postulated to be a post infective immune mediated monophasic demyelinating disease. Diagnosis is clinical supported by CSF and MRI findings. It is challenging to differentiate the disease from MDEM (multiphasic demyelinating encephalomyelitis) and multiple sclerosis during the initial presentation.Case presentation: Here we report a case of a 36-yearold Sri Lankan Sinhalese lady who presented with seizure following febrile illness followed by gross right sided hemiparesis later progressing to mutism and cognitive decline. Her CSF showed pleocytosis 12/cumm with 5% polymorphs, red cells, mildly high proteins with normal glucose and positive oligoclonal bands. MRI showed evidence of multiple focal supra and infratentorial signal abnormalities and gyral thickening with hemorrhages, largest lesions being in left frontal region with mass effect but no herniation. She was diagnosed with AHLE and treated with high dose IV methyl prednisolone later converting to oral prednisolone tapering regime. She was continued on intravenous cefotaxime and acyclovir as per meningoencephalitis. Improvement with steroids was dramatic with complete neurological recovery, minimal sequel and no recurrences at 6 months follow up.Conclusion: The case is a representation of a very rare entity of an infrequently reported disease which needs high degree of clinical suspicion with early imaging for aggressive and early immunosuppressive treatment that may hinder progression to commonly described sequel including coma, death or residual neurology. The predictions on future MDEM or MS on first presentation and overlap are areas that need further exploration.\",\"PeriodicalId\":9777,\"journal\":{\"name\":\"Ceylon Medical Journal\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.3000,\"publicationDate\":\"2023-11-16\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Ceylon Medical Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4038/cmj.v68i1.9677\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Ceylon Medical Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4038/cmj.v68i1.9677","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
Acute hemorrhagic leukoencephalitis in a young female with excellent response to corticosteroids
Background: Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant entity of acute disseminated leukoencephalitis (ADEM) that has a rapidly progressive polysymptomatology corresponding to areas of brain affected. It’s characterized by rapid cognitive decline, coma or even rapid death. It is postulated to be a post infective immune mediated monophasic demyelinating disease. Diagnosis is clinical supported by CSF and MRI findings. It is challenging to differentiate the disease from MDEM (multiphasic demyelinating encephalomyelitis) and multiple sclerosis during the initial presentation.Case presentation: Here we report a case of a 36-yearold Sri Lankan Sinhalese lady who presented with seizure following febrile illness followed by gross right sided hemiparesis later progressing to mutism and cognitive decline. Her CSF showed pleocytosis 12/cumm with 5% polymorphs, red cells, mildly high proteins with normal glucose and positive oligoclonal bands. MRI showed evidence of multiple focal supra and infratentorial signal abnormalities and gyral thickening with hemorrhages, largest lesions being in left frontal region with mass effect but no herniation. She was diagnosed with AHLE and treated with high dose IV methyl prednisolone later converting to oral prednisolone tapering regime. She was continued on intravenous cefotaxime and acyclovir as per meningoencephalitis. Improvement with steroids was dramatic with complete neurological recovery, minimal sequel and no recurrences at 6 months follow up.Conclusion: The case is a representation of a very rare entity of an infrequently reported disease which needs high degree of clinical suspicion with early imaging for aggressive and early immunosuppressive treatment that may hinder progression to commonly described sequel including coma, death or residual neurology. The predictions on future MDEM or MS on first presentation and overlap are areas that need further exploration.
期刊介绍:
The Ceylon Medical Journal, is the oldest surviving medical journal in Australasia. It is the only medical journal in Sri Lanka that is listed in the Index Medicus. The CMJ started life way back in 1887 as the organ of the Ceylon Branch of the British Medical Association. Except for a brief period between 1893 and 1904 when it ceased publication, the CMJ or its forbear, the Journal of the Ceylon Branch of the British Medical Association, has been published without interruption up to now. The journal"s name changed to the CMJ in 1954.