Arteriovenous Malformations Clinical Presentation

  • Author: Souvik Sen, MD, MS, MPH, FAHA; Chief Editor: Helmi L Lutsep, MD   more...
 
Updated: May 18, 2010
 

History

  • AVMs tend to be clinically silent until the presenting event occurs. Therefore, the diagnosis usually is made at the time of the first seizure or hemorrhage.
  • A history of minor learning disability is present in as many as two thirds of patients; such dysfunction is rarely apparent in adult life.
  • A history of headaches is present in as many as half of patients with cerebral AVM. The headaches subsequently may take the form of classic migraine or more generalized headache.
  • If seizures have occurred, a careful seizure history should be obtained. Seizures are simple, partial, or secondarily generalized.
  • The effectiveness of anticonvulsant therapy should be observed carefully and monitored before and during treatment.
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Physical

  • Focal neurologic findings are rare in the absence of seizure or hemorrhage in patients with cerebral AVMs. They are more common in brainstem and deeply located AVMs. They are associated with patient’s age and are more common among women.
  • Detailed neuropsychological testing may disclose subtle right or left hemisphere dysfunction.
  • If parenchymal hemorrhage is present, the physical findings are indistinguishable from those due to intracranial hemorrhage of other causes.
  • Intraventricular hemorrhage generally produces a less severe neurological deficit than hemorrhage into other areas of the brain.
  • In the rare patients in whom focal neurological deficits are present, the deficit may reflect the location of the AVM.
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Causes

  • No genetic, demographic, or environmental risk factors for cerebral AVM have been identified clearly.
  • Families with cerebral AVMs are rare, and such pedigrees have been too small to enable linkage studies. From the few family cases reported, the inheritance appears to be autosomal dominant.
  • In a small minority of cases, cerebral AVMs are associated with other inherited disorders, such as the Osler-Weber-Rendu syndrome (ie, hereditary hemorrhagic telangiectasia), Sturge-Weber disease, neurofibromatosis, and von Hippel-Lindau syndrome.
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Contributor Information and Disclosures
Author

Souvik Sen, MD, MS, MPH, FAHA  Professor and Chair, Dept. of Neurology, University of South Carolina School of Medicine

Souvik Sen, MD, MS, MPH, FAHA is a member of the following medical societies: American Academy of Neurology, American Heart Association, and Association for Patient Oriented Research

Disclosure: American Heart Association Grant/research funds Research project; BMS/Sanofi Aventis Honoraria Speaking and teaching; Boehringer Ingelheim Honoraria Speaking and teaching; Coaxia Consulting fee Independent contractor

Coauthor(s)

Sharon W Webb, MD  Assistant Professor of Clinical Neurosurgery, University of South Carolina School of Medicine

Sharon W Webb, MD is a member of the following medical societies: American Association of Neurological Surgeons, Congress of Neurological Surgeons, and Neurocritical Care Society

Disclosure: Nothing to disclose.

James Selph, MD  Assistant Professor of Neurology, University of South Carolina; Director of Neurophysiology Lab and Services, Palmetto Richland Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

Edward L Hogan, MD  Professor, Department of Neurology, Medical College of Georgia; Emeritus Professor and Chair, Department of Neurology, Medical University of South Carolina

Edward L Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Neurological Association, American Society for Biochemistry and Molecular Biology, Phi Beta Kappa, Sigma Xi, Society for Neuroscience, and Southern Clinical Neurological Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Howard S Kirshner, MD  Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center

Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association

Disclosure: BMS/Sanofi Honoraria Speaking and teaching

Chief Editor

Helmi L Lutsep, MD  Professor, Department of Neurology, Oregon Health & Science University; Associate Director, Oregon Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association

Disclosure: Co-Axia Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Concentric Medical Consulting fee Review panel membership; Abbott Consulting fee Consulting; Sanofi Consulting fee Consulting

References

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  3. Stapf C, Mast H, Sciacca RR, Berenstein A, Nelson PK, Gobin YP, et al. The New York Islands AVM Study: design, study progress, and initial results. Stroke. May 2003;34(5):e29-33. [Medline].

  4. Mast H, Young WL, Koennecke HC. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. Oct 11 1997;350(9084):1065-8. [Medline].

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  6. Park SH, Hwang SK. Transcranial Doppler study of cerebral arteriovenous malformations after gamma knife radiosurgery. J Clin Neurosci. March 2009;16(3):378-384.

  7. ARUBA Investigators. Unruptured brain arteriovenous malformation trial. [The Internet Stroke Center]. Feb 2006;[Full Text].

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  9. [Guideline] Ogilvy CS, Stieg PE, Awad I, Brown RD Jr, Kondziolka D, Rosenwasser R. AHA Scientific Statement: Recommendations for the management of intracranial arteriovenous malformations: a statement for healthcare professionals from a special writing group of the Stroke Council, American Stroke Association. Stroke. Jun 2001;32(6):1458-71. [Medline].

  10. Lawton MT, Kim H, McCulloch CE, Mikhak B, Young WL. A Supplementary Grading Scale for Selecting Patients With Brain Arteriovenous Malformations for Surgery. Neurosurgery. 2010;66(4):702-713.

  11. Hernesniemi J, Romani R, Lehecka M, Isarakul P, Dashti R, Celik O, et al. Present state of microneurosurgery of cerebral arteriovenous malformations. Acta Neurochir Suppl. 2010;107:71-6. [Medline].

  12. Castel JP, Kantor G. [Postoperative morbidity and mortality after microsurgical exclusion of cerebral arteriovenous malformations. Current data and analysis of recent literature]. Neurochirurgie. May 2001;47(2-3 Pt 2):369-83. [Medline].

  13. Al-Shahi R, Bhattacharya JJ, Currie DG, Papanastassiou V, Ritchie V, Roberts RC, et al. Prospective, population-based detection of intracranial vascular malformations in adults: the Scottish Intracranial Vascular Malformation Study (SIVMS). Stroke. May 2003;34(5):1163-9. [Medline].

  14. ApSimon HT, Reef H, Phadke RV, Popovic EA. A population-based study of brain arteriovenous malformation: long-term treatment outcomes. Stroke. Dec 2002;33(12):2794-800. [Medline].

  15. Flickinger JC, Kondziolka D, Lunsford LD, Pollock BE, Yamamoto M, Gorman DA. A multi-institutional analysis of complication outcomes after arteriovenous malformation radiosurgery. Int J Radiat Oncol Biol Phys. Apr 1 1999;44(1):67-74. [Medline].

  16. Hartmann A, Mast H, Mohr JP, Pile-Spellman J, Connolly ES, Sciacca RR. Determinants of staged endovascular and surgical treatment outcome of brain arteriovenous malformations. Stroke. Nov 2005;36(11):2431-5. [Medline]. [Full Text].

  17. Hillman J. Population-based analysis of arteriovenous malformation treatment. J Neurosurg. Oct 2001;95(4):633-7. [Medline].

  18. Hofmeister C, Stapf C, Hartmann A, et al. Demographic, morphological, and clinical characteristics of 1289 patients with brain arteriovenous malformation. Stroke. Jun 2000;31(6):1307-10. [Medline]. [Full Text].

  19. Maruyama K, Kawahara N, Shin M. The risk of hemorrhage after radiosurgery for cerebral arteriovenous malformations. N Engl J Med. Jan 13 2005;352(2):146-53. [Medline].

  20. Nataf F, Ghossoub M, Schlienger M. Bleeding after radiosurgery for cerebral arteriovenous malformations. Neurosurgery. Aug 2004;55(2):298-305; discussion 305-6. [Medline].

 
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Axial T2 MRI showing an arteriovenous malformation, with hemorrhage, in the territory of the left posterior cerebral artery.
T1 axial MRI showing a small subcortical arteriovenous malformation in the right frontal lobe.
T2 coronal MRI showing an arteriovenous malformation in the left medial temporal lobe.
Magnetic resonance angiography showing a left medial temporal arteriovenous malformation.
Angiogram (anteroposterior view) showing an arteriovenous malformation in the deep left middle cerebral artery territory measuring approximately 3 cm in diameter, with a deep draining vein (arrow).
 
 
 
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