Title: B-cell dominant lymphocytic primary angiitis of the central nervous system: Four biopsy-proven cases
Abstract: NeuropathologyVolume 30, Issue 2 p. 123-130 B-cell dominant lymphocytic primary angiitis of the central nervous system: Four biopsy-proven cases Jaekyung Myung, Jaekyung Myung Departments of Pathology,Search for more papers by this authorBomi Kim, Bomi Kim Departments of Pathology,Search for more papers by this authorByung-Woo Yoon, Byung-Woo Yoon Neurololgy, Korea Institute of Neuroscience, Seoul National University College of Medicine, Seoul, South KoreaSearch for more papers by this authorSang Kun Lee, Sang Kun Lee Neurololgy,Search for more papers by this authorJung-Joon Sung, Jung-Joon Sung Neurololgy,Search for more papers by this authorChun Kee Chung, Chun Kee Chung Korea Institute of Neuroscience, Seoul National University College of Medicine, Seoul, South Korea Neurosurgery, andSearch for more papers by this authorKee-Hyun Chang, Kee-Hyun Chang Radiology, Seoul National University Hospital, andSearch for more papers by this authorSung-Hye Park, Corresponding Author Sung-Hye Park Departments of Pathology, Korea Institute of Neuroscience, Seoul National University College of Medicine, Seoul, South KoreaSung-Hye Park, MD, PhD, Department of Pathology, Seoul National University, College of Medicine, 103 Daehangno, Jongno-gu, Seoul 110-799, South Korea. Email: [email protected]Search for more papers by this author Jaekyung Myung, Jaekyung Myung Departments of Pathology,Search for more papers by this authorBomi Kim, Bomi Kim Departments of Pathology,Search for more papers by this authorByung-Woo Yoon, Byung-Woo Yoon Neurololgy, Korea Institute of Neuroscience, Seoul National University College of Medicine, Seoul, South KoreaSearch for more papers by this authorSang Kun Lee, Sang Kun Lee Neurololgy,Search for more papers by this authorJung-Joon Sung, Jung-Joon Sung Neurololgy,Search for more papers by this authorChun Kee Chung, Chun Kee Chung Korea Institute of Neuroscience, Seoul National University College of Medicine, Seoul, South Korea Neurosurgery, andSearch for more papers by this authorKee-Hyun Chang, Kee-Hyun Chang Radiology, Seoul National University Hospital, andSearch for more papers by this authorSung-Hye Park, Corresponding Author Sung-Hye Park Departments of Pathology, Korea Institute of Neuroscience, Seoul National University College of Medicine, Seoul, South KoreaSung-Hye Park, MD, PhD, Department of Pathology, Seoul National University, College of Medicine, 103 Daehangno, Jongno-gu, Seoul 110-799, South Korea. Email: [email protected]Search for more papers by this author First published: 21 March 2010 https://doi.org/10.1111/j.1440-1789.2009.01051.xCitations: 12Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat Abstract We report four cases of biopsy-proven B-cell-rich primary angiitis of the central nervous system (PACNS). The mean age of the patients was 29 years (range, 23–37 years). The patients suffered from unilateral weakness (n = 2), seizure (n = 1), and hypersomnia, anorexia and confusion (n = 1). The vital signs and the results of laboratory tests were within normal limits in all the four cases except erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). ESR was elevated in one patient and CRP was elevated in two patients. The magnetic resonance imaging (MRI) scans revealed single (n = 2) or multiple (n = 2) irregularly enhancing lesions. Radiological studies initially indicated tumors such as glioma (n = 2) or lymphoma (n = 1), except in one case, in which the radiological analysis indicated vasculitis or demyelinating disease. All the cases involved both medium-sized (50–250 µm in diameter) and small-sized vessels (20–49 µm in diameter). The vascular, perivascular and parenchymal lymphocytes were polymorphous; however, CD20-positive B-cells were predominated in blood vessels while the CD8-positive T-cells infiltrated predominantly in brain parenchyma. Therefore, our patients revealed B-cell dominant lymphocytic vasculitis. Two patients who underwent active treatment (corticosteroid alone or with cyclophosphamide) showed remarkable clinical and radiological improvement but two patients still have initial neurological symptoms, namely, confusion and newly developed seizures, respectively, during the 19–101-month follow-up periods; this effect can be attributed to irreversible brain damage. Therefore, although early brain biopsy may be associated with histopathologic diagnostic pitfalls, it is a mandatory procedure for obtaining a confirmative diagnosis as well initiating early therapy, thereby reducing brain damage. REFERENCES 1 Volcy M, Toro ME, Uribe CS, Toro G. Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia. J Neurol Sci 2004; 227: 85–89. 2 Harbitz F. Unknown forms of arteritis with special reference to their relation to syphilitic arteritis and periarteritis nodosa. Am J Med Sci 1922; 163: 250–272. 3 Lie JT. 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Publication Year: 2010
Publication Date: 2010-04-01
Language: en
Type: article
Indexed In: ['crossref', 'pubmed']
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Cited By Count: 19
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