Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia

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

Research output: Contribution to journalArticle

163 Citations (Scopus)

Abstract

Background and Purpose - Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. Methods - Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. Any evidence of prior neurological symptoms or presence of an intracranial vascular malformation was recorded. All cases of possible cerebral arteriovenous malformation were confirmed by conventional arteriography. Results - Twelve patients (3.7%) had a history of cerebral vascular malformations. Ten patients had arteriovenous malformations, 1 had a dural arteriovenous fistula, and 1 had a cavernous malformation. Seven patients (2.1%) presented with intracranial hemorrhage, 2 presented with seizures alone, and 3 were discovered incidentally. The average age at the time of symptomatic intracranial hemorrhage was 25.4 years. All patients with a history of intracranial hemorrhage were classified as Rankin grade I or II at a mean follow-up interval of 6.0 years. A history of cerebral infarction or transient ischemic attack was found in 29.6% of patients with HHT and a pulmonary arteriovenous fistula. Conclusions - The risk of intracranial hemorrhage is low among people with HHT. Furthermore, a majority of these patients have a good functional outcome after hemorrhage. The data do not suggest a compelling indication for routine screening of patients with HHT for asymptomatic cerebral vascular malformations. By comparison, pulmonary arteriovenous fistulae are a much more frequent cause of neurological symptoms in this population.

Original languageEnglish (US)
Pages (from-to)877-882
Number of pages6
JournalStroke
Volume32
Issue number4
StatePublished - 2001

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Vascular Malformations
Intracranial Hemorrhages
Central Nervous System Vascular Malformations
Intracranial Arteriovenous Malformations
Arteriovenous Malformations
Transient Ischemic Attack
Cerebral Infarction
Fistula
Angiography
Seizures
Hemorrhage

Keywords

  • Cerebral arteriovenous malformations
  • Hereditary disease
  • Intracerebral hemorrhage
  • Stroke

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Neuroscience(all)

Cite this

Maher, C. O., Piepgras, D. G., Brown, R. D. J., Friedman, J. A., & Pollock, B. E. (2001). Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. Stroke, 32(4), 877-882.

Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. / Maher, Cormac O.; Piepgras, David G.; Brown, Robert D Jr.; Friedman, Jonathan A.; Pollock, Bruce E.

In: Stroke, Vol. 32, No. 4, 2001, p. 877-882.

Research output: Contribution to journalArticle

Maher, CO, Piepgras, DG, Brown, RDJ, Friedman, JA & Pollock, BE 2001, 'Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia', Stroke, vol. 32, no. 4, pp. 877-882.
Maher CO, Piepgras DG, Brown RDJ, Friedman JA, Pollock BE. Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. Stroke. 2001;32(4):877-882.
Maher, Cormac O. ; Piepgras, David G. ; Brown, Robert D Jr. ; Friedman, Jonathan A. ; Pollock, Bruce E. / Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. In: Stroke. 2001 ; Vol. 32, No. 4. pp. 877-882.
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