Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis
Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion reco...
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| Vydáno v: | Multiple sclerosis Ročník 14; číslo 8; s. 1139 - 1141 |
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| Hlavní autoři: | , , , , |
| Médium: | Journal Article |
| Jazyk: | angličtina |
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London, England
SAGE Publications
01.09.2008
Arnold Sage Publications Ltd |
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| ISSN: | 1352-4585, 1477-0970 |
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| Abstract | Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS. |
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| AbstractList | Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS. [PUBLICATION ABSTRACT] Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS. Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS. Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS.Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS. |
| Author | Arias-Rivas, S Arias, M Osorio, XR Vázquez, F Dapena, D |
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| Cites_doi | 10.1001/archneur.1993.00540090032007 10.1046/j.0004-8461.2003.01140.x 10.1097/00019052-200406000-00014 10.1177/014107680009301108 10.1007/s004150050285 10.1097/00005792-198801000-00002 10.1159/000052120 10.1002/ana.410370713 10.1093/qjmed/hci098 |
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| Keywords | demyelinating encephalopathy primary angiitis of CNS echo-gradient MRI Nervous system diseases Multiple sclerosis Central nervous system Leucoencephalopathy Recurrent Nuclear magnetic resonance imaging Inflammatory disease Cerebral disorder Gradient echo Encephalopathy Central nervous system disease Degenerative disease Diagnosis |
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| References | Iwase T, Ojika K, Mitake S, Katada E, Katano H, Mase M 2001; 45 Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OAC 1993; 50 Singh S, John S, Joseph TP, Soloman T 2003; 47 MacLaren K, Gillespie J, Shrestha S, Neary D, Ballardie FW 2005; 98 Younger DS 2004; 17 Ferro JM 1998; 245 Moore PM 1995; 37 Ay H, Sahin G, Saatci I, Söylemezoglu F, Saribas O 2002; 23 Calabrese LH, Mallek JA 1988; 67 Rehman HU 2000; 93 atypb8 atypb1 Ay H (atypb9) 2002; 23 atypb3 atypb2 atypb10 atypb5 atypb4 atypb7 atypb6 |
| References_xml | – volume: 23 start-page: 1561 year: 2002 end-page: 1563 article-title: Primary angiitis of the central nervous system and silent cortical hemorrhages publication-title: AJNR Am J Neuroradiol – volume: 98 start-page: 643 year: 2005 end-page: 654 article-title: Primary angiitis of the central nervous system: emerging variants publication-title: QJM – volume: 245 start-page: 766 year: 1998 end-page: 776 article-title: Vasculitis of the central nervous system publication-title: J Neurol – volume: 37 start-page: 131 year: 1995 end-page: 141 article-title: Neurological manifestation of vasculitis: update on immuno pathogenic mechanisms and clinical features publication-title: Ann Neurol – volume: 93 start-page: 586 year: 2000 end-page: 588 article-title: Primary angiitis of the central nervous system publication-title: J R Soc Med – volume: 50 start-page: 925 year: 1993 end-page: 930 article-title: Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges publication-title: Arch Neurol – volume: 45 start-page: 184 year: 2001 end-page: 185 article-title: Involvement of CD45RO+ T lymphocyte infiltration in a patient with primary angiitis of the central nervous system restricted to small vessels publication-title: Eur Neurol – volume: 17 start-page: 317 year: 2004 end-page: 336 article-title: Vasculitis of the nervous system publication-title: Curr Opin Neurol – volume: 47 start-page: 127 year: 2003 end-page: 134 article-title: Primary angiitis of the central nervous system: MRI features and clinical presentation publication-title: Australas Radiol – volume: 67 start-page: 29 year: 1988 end-page: 39 article-title: Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria publication-title: Medicine (Baltimore) – ident: atypb6 doi: 10.1001/archneur.1993.00540090032007 – ident: atypb8 doi: 10.1046/j.0004-8461.2003.01140.x – ident: atypb10 doi: 10.1097/00019052-200406000-00014 – ident: atypb5 doi: 10.1177/014107680009301108 – ident: atypb2 doi: 10.1007/s004150050285 – ident: atypb1 doi: 10.1097/00005792-198801000-00002 – ident: atypb3 doi: 10.1159/000052120 – ident: atypb7 doi: 10.1002/ana.410370713 – ident: atypb4 doi: 10.1093/qjmed/hci098 – volume: 23 start-page: 1561 year: 2002 ident: atypb9 publication-title: AJNR Am J Neuroradiol |
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| SubjectTerms | Adult Biological and medical sciences Cerebral Hemorrhage - pathology Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Humans Investigative techniques, diagnostic techniques (general aspects) Leukoencephalitis, Acute Hemorrhagic - pathology Magnetic Resonance Imaging Male Medical sciences Nervous system Neurology Radiodiagnosis. Nmr imagery. Nmr spectrometry Recurrence Vasculitis, Central Nervous System - diagnosis Vasculitis, Central Nervous System - pathology |
| Title | Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis |
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