Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression
Introduction. Amyloid-beta-related angiitis (ABRA) is a form of CNS vasculitis in which perivascular beta-amyloid in the intracerebral vessels is thought to act as a trigger for inflammation mediated by CD68+ macrophages and CD3+ T lymphocytes. Patients with severe ABRA may develop coma responsive t...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2012/678746 |
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| author | Shennan A. Weiss David Pisapia Stephan A. Mayer Joshua Z. Willey Kiwon Lee |
| author_facet | Shennan A. Weiss David Pisapia Stephan A. Mayer Joshua Z. Willey Kiwon Lee |
| author_sort | Shennan A. Weiss |
| collection | DOAJ |
| description | Introduction. Amyloid-beta-related angiitis (ABRA) is a form of CNS vasculitis in which perivascular beta-amyloid in the intracerebral vessels is thought to act as a trigger for inflammation mediated by CD68+ macrophages and CD3+ T lymphocytes. Patients with severe ABRA may develop coma responsive to immunosuppressive treatment. Case Presentation. A 57-year-old man presented to the neurological intensive care unit febrile, obtunded, and with a left hemiparesis. He had suffered from intermittent left arm weakness and numbness for several months prior. Serum and cerebrospinal fluid studies showed a lymphocytic leukocytosis in the cerebrospinal fluid (CSF), but no other evidence of infection, and the patient underwent a brain biopsy. Histopathological examination demonstrated amyloid angiopathy, with an extensive perivascular lymphocytic infiltrate, indicative of ABRA. The patient was started on cyclophosphamide and steroids. Following a week of treatment he awakened and over several weeks made a significant neurological recovery. Conclusions. ABRA can have a variety of clinical presentations, including impairments in consciousness and coma. Accurate pathological diagnosis, followed by aggressive immunosuppression, can lead to impressive neurological improvements. This diagnosis should be considered in patients with paroxysmal recurrent neurological symptoms and an accelerated progression. |
| format | Article |
| id | doaj-art-3ec84ff0c9714d54a8791f8ddbb5b860 |
| institution | Kabale University |
| issn | 2090-6781 2090-679X |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
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| series | Case Reports in Pathology |
| spelling | doaj-art-3ec84ff0c9714d54a8791f8ddbb5b8602025-02-03T05:50:15ZengWileyCase Reports in Pathology2090-67812090-679X2012-01-01201210.1155/2012/678746678746Amyloid β-Related Angiitis Causing Coma Responsive to ImmunosuppressionShennan A. Weiss0David Pisapia1Stephan A. Mayer2Joshua Z. Willey3Kiwon Lee4Department of Neurology, Columbia University, 710 W. 168th Street, New York, NY 10032, USADepartment of Pathology, Columbia University, 630 W. 168th Street, New York, NY 10032, USADepartment of Neurology, Columbia University, 710 W. 168th Street, New York, NY 10032, USADepartment of Neurology, Columbia University, 710 W. 168th Street, New York, NY 10032, USADepartment of Neurology, Columbia University, 710 W. 168th Street, New York, NY 10032, USAIntroduction. Amyloid-beta-related angiitis (ABRA) is a form of CNS vasculitis in which perivascular beta-amyloid in the intracerebral vessels is thought to act as a trigger for inflammation mediated by CD68+ macrophages and CD3+ T lymphocytes. Patients with severe ABRA may develop coma responsive to immunosuppressive treatment. Case Presentation. A 57-year-old man presented to the neurological intensive care unit febrile, obtunded, and with a left hemiparesis. He had suffered from intermittent left arm weakness and numbness for several months prior. Serum and cerebrospinal fluid studies showed a lymphocytic leukocytosis in the cerebrospinal fluid (CSF), but no other evidence of infection, and the patient underwent a brain biopsy. Histopathological examination demonstrated amyloid angiopathy, with an extensive perivascular lymphocytic infiltrate, indicative of ABRA. The patient was started on cyclophosphamide and steroids. Following a week of treatment he awakened and over several weeks made a significant neurological recovery. Conclusions. ABRA can have a variety of clinical presentations, including impairments in consciousness and coma. Accurate pathological diagnosis, followed by aggressive immunosuppression, can lead to impressive neurological improvements. This diagnosis should be considered in patients with paroxysmal recurrent neurological symptoms and an accelerated progression.http://dx.doi.org/10.1155/2012/678746 |
| spellingShingle | Shennan A. Weiss David Pisapia Stephan A. Mayer Joshua Z. Willey Kiwon Lee Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression Case Reports in Pathology |
| title | Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression |
| title_full | Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression |
| title_fullStr | Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression |
| title_full_unstemmed | Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression |
| title_short | Amyloid β-Related Angiitis Causing Coma Responsive to Immunosuppression |
| title_sort | amyloid β related angiitis causing coma responsive to immunosuppression |
| url | http://dx.doi.org/10.1155/2012/678746 |
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