Giant intracranial aneurysm and fibromuscular dysplasia in an adolescent with alpha 1-antitrypsin deficiency.

Abstract

Recent studies have suggested that a deficiency of alpha 1-antitrypsin may be a genetic risk factor for the development of intracranial aneurysms and arterial fibromuscular dysplasia. The authors report a 16-year-old girl with a history of lung disease who suffered a cerebral hemorrhage due to the rupture of a giant intracranial aneurysm arising from the middle cerebral artery. This fusiform aneurysm was associated with fibromuscular dysplasia of the intimal type. She was found to have an unusual alpha 1-antitrypsin deficiency (PiMP phenotype). This case provides further evidence of an underlying arteriopathy in alpha 1-antitrypsin deficiency.

• Authors: W I Schievink, M R Puumala, F B Meyer, C Raffel, J A Katzmann, J E Parisi
• Journal: Journal of neurosurgery (J Neurosurg) Vol. 85 Issue 3 Pg. 503-6 (Sep 1996) ISSN: 0022-3085 [Print] United States
• PMID: 8751640 (Publication Type: Case Reports, Journal Article)

Chemical References

• alpha 1-Antitrypsin

Topics

• Adolescent
• Female
• Fibromuscular Dysplasia (pathology)
• Humans
• Intracranial Aneurysm (pathology)
• alpha 1-Antitrypsin (analysis)
• alpha 1-Antitrypsin Deficiency