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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.
Insights
Patients with hereditary hemorrhagic telangiectasia (HHT) have a higher risk of brain abscess due to pulmonary arteriovenous malformations. Early investigation of neurologic symptoms in HHT patients is crucial for timely diagnosis and treatment.
Area of Science:
- Neurology
- Vascular Medicine
- Infectious Disease
Background:
- Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder affecting blood vessels.
- Pulmonary arteriovenous malformations (PAVMs) are common in HHT and can lead to paradoxical embolism.
Observation:
- This study reviewed 31 brain abscess and 1 meningitis case in HHT patients.
- PAVMs were present in most patients, allowing septic microemboli to reach the brain.
- Common symptoms included obtundation, headache, visual disturbances, hemiplegia, and seizures.
Findings:
- Leukocytosis and fever were infrequent, and blood cultures were sterile in many cases.
- Anaerobic and microaerophilic streptococci were the most common pathogens.
- Mortality was higher in patients with delayed diagnosis or without abscess drainage.
Implications:
- Brain abscess occurs in approximately 1% of HHT patients.
- Awareness of this risk is vital for prompt diagnosis and management.
- Early neurological evaluation in HHT patients with symptoms can improve outcomes.