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Primary Angiitis of the Center Nervous System: A Clinical Challenge Diagnosed Postmortem
Joint Authors
Al-Share, Bayan
Hiner, Evan
Iskenderian, Ziyad
Warra, Nader
Zakaria, Ali H.
Source
Case Reports in Neurological Medicine
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-07-05
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis involving medium and small blood vessels of the brain, spinal cord, and meninges, without systemic involvement.
The diffuse and patchy nature of its pathology is reflected by a wide spectrum of nonspecific clinical symptoms.
Diagnosis is challenging due to lack of defined clinical criteria or specific imaging findings.
Specific workup should be done only after exclusion of other etiologies, including infectious, neoplastic, toxic, and other vascular etiologies including systemic vasculitis.
Given the fact that it is a patchy disease with 25% of the biopsies being falsely negative, treating physician should have a high index of suspicion despite negative initial neurovascular imaging and biopsy results.
Once diagnosed, early treatment with immunosuppressive therapy is essential to avoid permanent neurologic damage.
Herein, we are reporting a case of 66-year-old female patient who presented with insidious onset right-sided frontal headache.
Her hospital course progressively worsened and family decision based on her wishes was to refer her to hospice and comfort care.
Despite an extensive workup with advanced imaging techniques, no diagnosis was established until postmortem autopsy and histopathology confirmed primary angiitis of the central nervous system.
American Psychological Association (APA)
Al-Share, Bayan& Zakaria, Ali H.& Hiner, Evan& Iskenderian, Ziyad& Warra, Nader. 2017. Primary Angiitis of the Center Nervous System: A Clinical Challenge Diagnosed Postmortem. Case Reports in Neurological Medicine،Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1147135
Modern Language Association (MLA)
Al-Share, Bayan…[et al.]. Primary Angiitis of the Center Nervous System: A Clinical Challenge Diagnosed Postmortem. Case Reports in Neurological Medicine No. 2017 (2017), pp.1-4.
https://search.emarefa.net/detail/BIM-1147135
American Medical Association (AMA)
Al-Share, Bayan& Zakaria, Ali H.& Hiner, Evan& Iskenderian, Ziyad& Warra, Nader. Primary Angiitis of the Center Nervous System: A Clinical Challenge Diagnosed Postmortem. Case Reports in Neurological Medicine. 2017. Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1147135
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1147135