Central nervous system infections associated with hereditary hemorrhagic telangiectasia

doi:10.1016/0002-9343(84)90441-8

The American Journal of Medicine

Volume 77, Issue 1, July 1984, Pages 86-92

https://doi.org/10.1016/0002-9343(84)90441-8 Get rights and content

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.

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A case of hereditary hemorrhagic telangiectasia presenting with brain abscess
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Hereditary hemorrhagic telangiectasia (HHT), characterized by telangiectases and arteriovenous malformations that can occur in any organ but primarily the lungs, liver, and brain, is an autosomal dominant disorder. Brain abscess, though a rare and potentially fatal complication, can be an initial presentation of this condition.
A 60-year-old man presented with anemia and black tarry stools, then developed right-sided weakness initially misdiagnosed as an ischemic stroke. Subsequent onset of headaches and high-grade fever led to the diagnosis of brain abscess, and HHT was subsequently identified.
This case underscores that brain abscess can be an initial symptom of HHT. Prompt diagnosis and treatment are vital, which requires physicians to maintain a high index of suspicion and conduct appropriate investigations promptly.
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Citation Excerpt :
Pulmonary arteriovenous malformations (PAVM) also lead to right-to-left shunting of blood and may predispose people to development of brain abscesses.8–10 In 1 series, only 33% of patients who presented with brain abscesses associated with PAVMs had symptoms of PAVM before presentation with infection of the central nervous system.11 Most patients with PAVM have hereditary hemorrhagic telangiectasia,12,13 although variable presentation may lead to lack of recognition of the familial predisposition.14
Although brain abscesses are frequently cryptogenic in origin, bacteria must reach the brain either by direct or hematogenous spread. Right-to-left shunts, caused either by intrapulmonary vascular malformations or congenital heart defects, may allow microorganisms to evade the normal host defenses in the lungs and lead to development of brain abscesses. Two patients recently presented with brain abscesses and were found to have conditions associated with right-to-left shunts. The diagnosis of brain abscess should prompt the clinician to consider right-to-left shunts as a possible predisposing condition for brain abscess.
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An unusual etiology of recurrent cerebral abscesses-a report of 3 cases
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Various neurological sequelae due to a variety of vascular, hemorrhagic, embolic, and infectious complications have been reported in 8% to 12% of all patients with HHT and up to 41% patients with pulmonary AV fistula [10,12]. The mechanisms of neurological complications in pulmonary AV fistula have been enumerated by Press and Ramsey [10] in their review. The right to left shunt in pulmonary AV fistula results in hypoxemia, which causes infarcts in brain.
Cerebral abscess commonly occurs secondary to trauma, hematogenous spread from distant infection, or otitis media. Pulmonary AVF is an uncommon cause of recurrent cerebral abscess and is often overlooked. We report 3 cases of cerebral abscesses secondary to pulmonary AVF.
A 20-year-old man who presented with fever, headache, and vomiting of short duration was diagnosed as having right parietal abscess and underwent burr hole and tapping and antibiotic treatment. He later presented with many episodes of cerebral abscess at various locations involving both sides and required multiple surgical procedures. The routine workup for the source did not reveal any etiology. His hematological examination revealed polycythemia. A pulmonary angiogram was performed, which revealed pulmonary AVF. He underwent embolization of the fistula and had no further recurrence. Two other adult men were treated surgically for cerebral abscess. One patient had polycythemia and hypoxemia, and another had clubbing. Both patients were investigated with CT of the chest and were diagnosed as having pulmonary AVF. None of them had features of HHT.
Pulmonary AVF is an unusual cause of cerebral abscess. Patients with multiple recurrences of cerebral abscess, signs of pulmonary disease, and hypoxia should be investigated for pulmonary AVF.
Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database
2023, Neurology

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¹: From the Department of Medicine, University of Washington, Seattle, Washington.

²: Dr. Ramsey is a Teaching and Research Scholar of the American College of Physicians.

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ReviewCentral nervous system infections associated with hereditary hemorrhagic telangiectasia

Abstract

J Thorac Surg

J Thorac Surg

Oral Surg

Gastroenterology

Chest

Neurological implications of hereditary hemorrhagic telangiectasia

J Nerv Ment Dis

Neurologic aspects of hereditary hemorrhagic telangiectasia

Arch Neural

Neurological manifestations of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease): report of 2 cases and review of the literature

Ann Neurol

Über Heredofamiliäre Angiomatose des Gehirns und der Retina, ihre Beziehungen zueinander und zur Angiomatose der Haut

Z Neurol Psychiatr

Multiple hemangiomas of the skin and of the internal organs

Arch Pathol