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(Stroke. 2001;32:2957.)
© 2001 American Heart Association, Inc.

Letters to the Editor

Compelling Reasons to Screen Brain in HHT

Jennifer Mandzia, BSc; Katharine Henderson, MS; Marie Faughnan, MD Robert White, Jr, MD

Department of Medicine, Division of Neurology, Sunnybrook and Women’s HSC, University of Toronto
Department of Radiology, Yale University School of Medicine
Department of Respiratory Medicine, University of Toronto, HHT Program, St Michael’s Hospital, Toronto, Ontario, Canada
Department of Radiology, Yale University School of Medicine, Yale AVM Center, New Haven, Connecticut

To the Editor:

We read with great interest the article by Maher et al,¹ in which the authors reviewed the medical records from hereditary hemorrhagic telangiectasia (HHT) patients seen at their institution over 20 years. However, we disagree with the authors’ final conclusion that HHT patients should not undergo routine screening for cerebrovascular malformations (CVMs) if asymptomatic. We advocate a single baseline MRI screen in all patients with HHT, as CVMs can cause devastating neurological complications.

The findings by Maher et al of a CVM prevalence rate of 12 of 321 (3.7%) in their patient group is rather low in comparison to those in other studies.^2,3 The authors acknowledge this and attribute it to screening only symptomatic patients. We believe that neurological symptoms are not a good indicator of who should receive MRI screening, and, based on our experience, we have found that neurological symptoms are not necessarily present in individuals with CVMs.^2–4 The consequences of a CVM-related hemorrhage could be devastating.

Other studies^2–4 have described CVMs in HHT and have reported micro-arteriovenous malformations (AVMs), small AVMs, and macro-AVMs, as well as cerebral telangiectases and cavernomas. Fulbright et al,³ who used noncontrast and gadolinium-enhanced MRI, and Willemse et al,² who used digital subtraction angiography (DSA), found a prevalence of CVMs of 22% and 12.2%, respectively. The difference in prevalence rates between these 2 studies may be due to increased detection of other CVMs, such as micro-AVMs and cerebral telangiectases found on contrast-enhanced MRI in the Fulbright et al study. However, in terms of . . . [Full Text of this Article]

Cormac O. Maher, MD; David G. Piepgras, MD; Robert D. Brown, Jr, MD; Jonathan A. Friedman, MD Bruce E. Pollock, MD

Departments of Neurosurgery and Neurology, Mayo Clinic, Rochester, Minnesota