Inflammatory Vasculitides of the Central Nervous System
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(2020) 22:19
Cerebrovascular Disease and Stroke (S Silverman, Section Editor)
Inflammatory Vasculitides of the Central Nervous System Kelly L. Sloane, MD1 Pria Anand, MD2,* Address 1 Department of Neurology, Massachusetts General Hospital, Boston, MA, USA *,2 Department of Neurology, Boston University Medical Center, 72 E. Concord Street, Boston, MA, 02118, USA Email: [email protected]
* Springer Science+Business Media, LLC, part of Springer Nature 2020
This article is part of the Topical collection on Cerebrovascular Disease and Stroke Keywords Vasculitis I Neurosarcoidosis I PACNS I CAA I Neuroimmunology I Stroke
Abstract Purpose of review To provide an approach to the evaluation and management of primary and secondary inflammatory vasculitides affecting the central nervous system (CNS). Recent findings Although early descriptions of inflammatory CNS vasculitis report a universally high mortality rate, more recent cohorts demonstrate that favorable outcomes rely on early diagnosis and treatment. New and re-purposed medications have shown promise in the treatment of CNS vasculitides, and novel imaging modalities may aid in diagnosis. Summary A broad array of diseases can affect CNS vasculature. Accurate diagnosis of CNS vasculitides requires a thorough exploration of systemic inflammatory diseases, as well as a clear understanding of the rare entities that cause vasculitides limited to the CNS.
Introduction In the 1950s, neuropathologists at Bellevue and Mount Sinai Hospitals reported on a “noninfectious granulomatous angiitis with a predilection for the nervous system,” describing “a specific form of angiitis, generally designated as granulomatous angiitis, [that] regularly affects the vessels of the central nervous system, at times without concurrent involvement of other organs, at times involving other organs to a less significant degree” [1]. In the years since, clinicians have come to recognize the existence
of both primary and secondary vasculitides “with a predilection for the nervous system.” Secondary vasculitides of the CNS encompass a broad range of systemic infectious and inflammatory processes and often require a thorough exploration of other organ systems for successful diagnosis. By contrast, primary vasculitides of the CNS are confined to the brain, meninges, and occasionally spinal cord, representing a profound diagnostic dilemma that may require brain or meningeal biopsy for confirmation.
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Curr Treat Options Cardio Med
The term “CNS vasculitis” describes inflammation of blood vessel walls within the CNS. This process may be specific to blood vessels of a particular size—for instance, Takayasu arteritis favors large vessels, including the carotid and vertebral arteries, while Susac’s syndrome primarily involves small-caliber vessels—or may be indiscriminate of size, affecting blood vessels of variable sizes in multiple locations (Table 1). CNS vasculitides should thus be suspected in patients who present with new neurologic symptoms and ischemic or hemorrhagic lesions appreci
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