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Central Nervous System Vasculitis: Primary Angiitis of the Central Nervous System

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Cerebrovascular Disorders

Part of the book series: Neuromethods ((NM,volume 170))

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Abstract

Primary angiitis of the central nervous system (CNS) is a rare disorder involving idiopathic inflammation of small- and medium-sized leptomeningeal and parenchymal arteries within the CNS. The disease may cause headaches, multifocal strokes of either ischemic or hemorrhagic subtypes, cognitive impairment, seizures, and visual changes; however, there is a wide variation in symptomatology and clinical course of patients presenting with primary angiitis of the CNS. This chapter reviews the standard diagnostic evaluation for primary angiitis of the CNS, which includes serological testing for signs of inflammation and potential alternative diagnoses, vascular neuroimaging, lumbar puncture, diagnostic cerebral angiogram, and possible brain biopsy. Although the disease carries high mortality if left untreated, it is relatively responsive to corticosteroids as part of an acute treatment course. This chapter also reviews several immunosuppressive agents used for maintenance therapy.

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Abbreviations

ABRA:

Aβ-related angiitis

ACE:

Angiotensin-converting enzyme

ANCA:

Antineutrophil cytoplasmic antibodies

CNS:

Central nervous system

CRP:

C-reactive protein

CSF:

Cerebrospinal fluid

EBV:

Epstein-Barr virus

ESR:

Erythrocyte sedimentation rate

HHV-6:

Human herpesvirus 6

HIV:

Human immunodeficiency virus

HSV:

Herpes simplex virus

MRA:

Magnetic resonance angiography

MRI:

Magnetic resonance imaging

mRS:

Modified Rankin Scale

PACNS:

Primary angiitis of the CNS

PRES:

Posterior reversible encephalopathy syndrome

RCVS:

Reversible cerebral vasoconstriction syndrome

SSA/B:

Sjögren’s syndrome-related antigen A/B

VDRL:

Venereal disease research laboratory test

VZV:

Varicella zoster virus

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Author information

Authors and Affiliations

  1. Department of Neurology, Weill Cornell Medical College, New York, NY, USA

    Aaron M. Gusdon, Mackenzie P. Lerario & Gary L. Bernardini

  2. NewYork-Presbyterian Queens, Flushing, NY, USA

    Mackenzie P. Lerario & Gary L. Bernardini

  3. Fordham University Graduate School of Social Service, New York, NY, USA

    Mackenzie P. Lerario

  4. Department of Pathology, Weill Cornell Medical College, New York, NY, USA

    Ehud Lavi

Authors
  1. Aaron M. Gusdon
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  2. Mackenzie P. Lerario
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  3. Ehud Lavi
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  4. Gary L. Bernardini
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Corresponding author

Correspondence to Mackenzie P. Lerario .

Editor information

Editors and Affiliations

  1. Associate Chair of Neurology for Research, New York Medical College, Associate Professor of Neurology, Neurosurgery and Radiology, New York Medical College, Director of the Neuroendovascular Surgery Fellowship, Neuroendovascular Surgery (Interventional Neurologist) Attending Medical Director of Neurocritical Care, Westchester Medical Center at New York Medical College, New York, USA

    Fawaz Al-Mufti, MD

  2. Division of Neurointerventional Radiology, Goodman Campbell Brain and Spine, Division of Neurointerventional Radiology, Ascension St. Vincent Medical Center, Department of Neurosurgery, Marian College of Osteopathic Medicine, Indianapolis, IN, USA

    Krishna Amuluru MD

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Gusdon, A.M., Lerario, M.P., Lavi, E., Bernardini, G.L. (2021). Central Nervous System Vasculitis: Primary Angiitis of the Central Nervous System. In: Al-Mufti, MD, F., Amuluru MD, K. (eds) Cerebrovascular Disorders. Neuromethods, vol 170. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-1530-0_21

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  • DOI: https://doi.org/10.1007/978-1-0716-1530-0_21

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  • Publisher Name: Humana, New York, NY

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