Neurological manifestations of hereditary hemorrhagic telangiectasia (rendu‐osler‐weber disease): Report of 2 cases and review of the literature Academic Article uri icon

Overview

MeSH Major

  • Cerebral Hemorrhage
  • Cerebrovascular Disorders
  • Intracranial Aneurysm
  • Telangiectasia, Hereditary Hemorrhagic

abstract

  • Two cases of hereditary hemorrhagic telangiectasia (HHT) with neurological involvement are presented. One patient had multiple vascular malformations including telangiectasias of the brain, medulla, and spinal cord and a berry aneurysm of the internal carotid artery; she also had a large cerebellar abscess, presumably reflecting the presence of a pulmonary arteriovenous fistula. The second patient had an idiopathic subarachnoid hemorrhage. In more than 200 reported patients with HHT involving the nervous system, 61% had lesions seondary to a pulmonary arteriovenous fistula (cerebral hypoxemia, paradoxical and septic emboli, and brain abscess). The findings emphasize the need for early surgical correction of such fistulas. In 36% of the patients with neurological involvement and HHT, vascular malformations of the brain and spinal cord were documented, and in 3%, portal-systemic encephalopathy was noted. Multiple lesions were frequent. HHT should be considered a generalized vascular dysplasia (universal or systemic angiomatosis), and not simply a benign mucocutaneous disease.

publication date

  • January 1978

Research

keywords

  • Academic Article

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1002/ana.410040207

PubMed ID

  • 707984

Additional Document Info

start page

  • 130

end page

  • 44

volume

  • 4

number

  • 2