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NEUROLOGY 2009;72:e45
© 2009 American Academy of Neurology
Resident and Fellow Section

Teaching NeuroImages: Cerebral arteriovenous malformation in hereditary hemorrhagic telangiectasia

Aleksandra Pikula, MD, Jorge L. Almodovar, MD and Thanh N. Nguyen, MD

From the Departments of Neurology (A.P., J.L.A., T.N.N.) and Neurosurgery and Radiology (T.N.N.), Boston University Medical Center, Boston, MA.

Address correspondence and reprint requests to Dr. Aleksandra Pikula, Boston University School of Medicine, Department of Neurology, 715 Albany Street, C-329, Boston, MA 02118 Aleksandra.Pikula{at}bmc.org

A 41-year-old woman with a history of recurrent epistaxis presented with left hemiplegia, right gaze deviation, and dysarthria. Physical examination confirmed skin and lip telangiectasia (figure 1). Her son had pulmonary arteriovenous malformation (AVM). Her head CT and cerebral angiography revealed right parietal hemorrhage with parasagittal AVM (figure 2).


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  		Figure 1 Skin telangiectasias (A) and lip telangiectasias (B)

 

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  		Figure 2 CT (A), cerebral angiography (B), and magnified view of AVM nidus (C)

(A) CT: right parietal hemorrhage. (B) Cerebral angiography: right parasagittal arteriovenous malformation (AVM) supplied by the right distal anterior cerebral artery (white arrowhead), draining to superior sagittal sinus. (C) Microcatheter injection: magnified view of AVM nidus with aneurysmal appearance.

 

Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu syndrome) is a disorder characterized by recurrent epistaxis, skin and mucosal telangiectasias, visceral and cerebral AVMs, and positive family history. Diagnosis is made when three of the above criteria are present.1 Cerebral AVMs affect 10% of patients with HHT. Routine screening for cerebral AVMs in asymptomatic patients with HHT remains controversial.1,2

Disclosure: The authors report no disclosures.


   REFERENCES
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 REFERENCES
 

  1. Begbie ME, Wallace GMF, Shovlin CL. Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J 2003;79:18–24.[Abstract/Free Full Text]
  2. Willemse RB, Mager JJ, Westermann CJJ, et al Bleeding risk of cerebrovascular malformations in hereditary hemorrhagic telangiectasia. J Neurosurg 2000;92:779–784.[Medline]




This Article
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Right arrow Arteriovenous malformation

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