Central nervous system infections associated with hereditary hemorrhagic telangiectasia

Abstract

The clinical courses of 31 episodes of brain abscess and one episode of meningitis occurring in patients with hereditary hemorrhagic telangiectasia are reviewed. Pulmonary arteriovenous malformations were demonstrable in all but two patients and presumably permitted septic microemboli to evade the normal pulmonary capillary filter and lodge in the brain. Obtundation, headache, visual disturbances, hemiplegia, and seizures were the most common presenting features. Cyanosis, clubbing, polycythemia, and hypoxemia were routinely encountered, but leukocytosis and fever were present in a minority of cases, and all blood cultures were sterile. Anaerobic and microaerophilic streptococci were the commonest pathogens found in the brain abscesses. Thirteen patients died, and patients without abscess drainage or with delayed diagnosis had a higher mortality rate. A brain abscess may develop in approximately 1 percent of patients with hereditary hemorrhagic telangiectasia, and awareness of this risk should lead to early investigation of any patient with hereditary hemorrhagic telangiectasia who has neurologic symptoms.