Nodular Lesions of Choroid Plexus in Hurler Disease

Abstract

Neuropathologic examination of six brains from children with Hurler disease revealed nodular lesions in the glomus of choroid plexus caused by proliferation of vacuolated pericytes, fibroblasts, and arachnoid cells on the background of collagenized and myxoid stroma. This localization of lesions can be explained by the presence of a rich vascular network, as well as cellular heterogeneity greater in the glomus than in other parts of the choroid plexus or in the brain parenchyma. The development of nodules did not correlate with the age, severity of hydrocephalus, or the degree of expansion of the perivascular spaces in the brain.

Keywords :

• Hurler disease
• mucopolysaccharidosis
• pericyte
• choroid plexus

ACKNOWLEDGMENTS

Supported by the grant-in-aid research OMHF-214 from the Ontario Mental Health Foundation, and T3–11 from the Ontario Heart Foundation, Toronto, Ontario, Canada.