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Diagnosis and Treatment of Primary Central Nervous System Angiitis

  • Cerebrovascular Disorders (DG Jamieson, Section Editor)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of review

Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these patients. This unfortunately may lead to both under- or over-diagnosis and unnecessary treatment. It is important to recognize the different conditions that may mimic the clinical and radiologic presentation. We present a contemporary literature review that should update physicians who encounter this patient population.

Recent findings

Recent additions to the literature in form of case reports and single-center series show that digital subtraction angiography was the most widely used test to diagnose PCNSA. It is also important to note that brain biopsy is still considered “gold standard” and should be pursued as it not only has information that establishes the diagnosis but also may help in ruling out the diagnosis from mimics. In around 39% of suspected cases, an alternate diagnosis was eventually confirmed highlighting the importance of a comprehensive work-up. For treatment, almost all the literature supports the use of glucocorticoids as the initial treatment and if the patient has a relapse or develops steroid toxicity then the second most commonly used agent was cyclophosphamide. There are increasing reports of benefits with other agents such as methotrexate, azathioprine, mycophenolate, infliximab, and etanercept.

Summary

The diagnosis and management of PCNSA continues to be a challenge. Formation of prospective patient registries with continued immunologic research for novel targets and immunomodulatory modalities may hold promise for future care of these patients.

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Acknowledgements

We thank Dr. Steven Moore MD, PhD, Professor of Pathology, University of Iowa for allowing us to use the histology slide depicting primary CNS vasculitic changes from his teaching archive.

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Authors and Affiliations

  1. Cerebrovascular Diseases, University of Iowa, Iowa City, IA, USA

    Kaustubh Limaye MD

  2. Neurology, Neurosurgery and Radiology, University of Iowa, Iowa City, IA, USA

    Edgar A. Samaniego MD, MS

  3. Neurology, University of Iowa, Iowa City, IA, USA

    Harold P. Adams Jr. MD

Authors
  1. Kaustubh Limaye MD
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  2. Edgar A. Samaniego MD, MS
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  3. Harold P. Adams Jr. MD
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Contributions

Study concept and design: Adams and Limaye

Acquisition of data: Adams and Limaye

Analysis and interpretation of data: Adams, Samaniego, and Limaye

Drafting of manuscript: Limaye

Images: Unidentified images from teaching archives of Dr. Samaniego

Critical revision of manuscript for important intellectual content: Adams and Samaniego

Study supervision: Adams

Corresponding author

Correspondence to Harold P. Adams Jr. MD.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Cerebrovascular Disorders

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Limaye, K., Samaniego, E.A. & Adams, H.P. Diagnosis and Treatment of Primary Central Nervous System Angiitis. Curr Treat Options Neurol 20, 38 (2018). https://doi.org/10.1007/s11940-018-0522-5

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