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.

journal_name

J Neurosurg

journal_title

Journal of neurosurgery

authors

Schievink WI,Puumala MR,Meyer FB,Raffel C,Katzmann JA,Parisi JE

doi

10.3171/jns.1996.85.3.0503

subject

Has Abstract

pub_date

1996-09-01 00:00:00

pages

503-6

issue

3

eissn

0022-3085

issn

1933-0693

journal_volume

85

pub_type

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