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Secondary Prevention After Non-atherosclerotic Cerebral Vasculopathies

  • Chapter
Ischemic Stroke Therapeutics

Abstract

Uncommon causes of stroke represent up to 5 % of all ischemic strokes. Non-atherosclerotic vasculopathies (NAVs) account for a minority of strokes and are of particular importance in children and young adults, accounting for 14–25 % of strokes in patients under the age of 50. NAVs comprise a great variety of diseases with various underlying mechanisms including immunological, infective, collagen vascular, and hematological conditions. Increased availability of multimodal brain imaging and improved quality of noninvasive angiographic imaging has allowed more accurate and timely diagnoses, as well as better differentiation among such vasculopathies, resulting in appropriate management and enhanced outcomes. Arterial dissection, both traumatic and spontaneous, is the most common of the NAVs. Dissection is often interlinked with other arteriopathies including fibromuscular dysplasia and collagen vascular disorders. Other NAVs are being increasingly recognized, including reversible cerebral vasoconstriction syndrome, unilateral intracranial arteriopathy of childhood, moyamoya disease, post-radiation vasculopathies, and cerebral vasculitides. Mechanisms of stroke in NAVs vary from traumatic vessel injury to inflammation and infection, often in the setting of an underlying genetic predisposition. This chapter reviews the clinical manifestations, diagnosis, and management of the most common NAVs.

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Abbreviations

ABRA:

Amyloid β-related angiitis

ACA:

Anterior cerebral arteries

CNS:

Central nervous system

CAA:

Cerebral amyloid angiopathy

CSF:

Cerebrospinal fluid

CCAD:

Cervicocephalic arterial dissection

FMD:

Fibromuscular dysplasia

GCA:

Giant cell arteritis

ICA:

Internal carotid artery

MCA:

Middle cerebral artery

NAV:

Non-atherosclerotic vasculopathy

PRES:

Posterior reversible ischemic encephalopathy syndrome

PACNS:

Primary angiitis of the CNS

RCVS:

Reversible cerebral vasoconstriction syndrome

SAH:

Subarachnoid hemorrhage

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Acknowledgment

The author would like to thank Vimal Patel, PhD, medical and scientific writer at NorthShore Neurological Institute for assistance with preparing figures and copy-editing of the text.

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  1. Department of Neurology, NothShore University HealthSystem, Glenview, IL, 60026, USA

    Rima M. Dafer M.D., M.P.H.

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  1. Admiral Pihl Professor and Chairman of Neurology, Medical University of South Carolina, Charleston, South Carolina, USA

    Bruce Ovbiagele

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Dafer, R.M. (2016). Secondary Prevention After Non-atherosclerotic Cerebral Vasculopathies. In: Ovbiagele, B. (eds) Ischemic Stroke Therapeutics. Springer, Cham. https://doi.org/10.1007/978-3-319-17750-2_16

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