Association of multiple intracranial aneurysms and collagen type III deficiency

The results of a collagen assay performed on cultured skin fibroblasts from a 55-year-old man with multiple cerebral aneurysms are presented. Estimation of the collagen type III production by gel electrophoresis and scanning densitometry shows a reduction of collagen type III levels to 5% of normal. These findings point to the presence of a constitutional connective tissue disorder related to Ehlers-Danlos Syndrome type IV. It is suggested that biochemical collagen studies may be a useful alternative to angiography for detecting relatives at risk in families affected by intracranial aneurysms.