Giant intracranial aneurysm and fibromuscular dysplasia in an adolescent with α1-antitrypsin deficiency: Case report

Wouter I. Schievink, Michael R. Puumala, Fredric B. Meyer, Corey Raffel, Jerry A. Katzmann, Joseph E. Parisi

Research output: Contribution to journalArticlepeer-review

46 Scopus citations


Recent studies have suggested that a deficiency of α1-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 α1-antitrypsin deficiency (PiMP phenotype). This case provides further evidence of an underlying arteriopathy in α1-antitrypsin deficiency.

Original languageEnglish (US)
Pages (from-to)503-506
Number of pages4
JournalJournal of neurosurgery
Issue number3
StatePublished - Sep 1996


  • cerebral aneurysm
  • fibromuscular dysplasia
  • subarachnoid hemorrhage
  • α-antitrypsin deficiency

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology


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