Key result
Among 12 consecutive patients with spontaneous cervical artery dissection, 3 cases (25%) were associated with alpha-1-antitrypsin deficiency.
Case Report (n=12)
Alpha-1-antitrypsin deficiency may predispose individuals to spontaneous cervical artery dissection by causing a disequilibrium between proteolytic enzymes and protease inhibitors.
This small case series association is hypothesis-generating; does not support routine testing or practice change.
The pathogenesis of cervical artery dissection is poorly understood. Deficiency of the elastase inhibitor alpha-1-antitrypsin may represent a predisposing condition. Biochemical and genetic analyses in a series of 12 consecutive patients with spontaneous dissection of the neck vessels showed 3 cases associated to alpha-1-antitrypsin deficiency, in combination with transient precipitating factors. A disequilibrium between proteolytic enzymes and protease inhibitors may contribute to the pathogenesis of cervical artery dissection leading to structural abnormalities of the extracellular matrix and increasing the susceptibility of the vessel wall to additional short-lived trigger mechanisms.
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Pezzini et al. (2002) conducted a case report in Spontaneous dissection of the neck vessels (n=12). Alpha-1-antitrypsin deficiency was evaluated on Presence of alpha-1-antitrypsin deficiency. Among 12 consecutive patients with spontaneous cervical artery dissection, 3 cases (25%) were associated with alpha-1-antitrypsin deficiency.
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