A new SPG4 mutation in a variant form of spastic paraplegia with congenital arachnoid cysts.

Orlacchio, A; Gaudiello, F; Totaro, A; Floris, R; St George-Hyslop, P H; Bernardi, G; Kawarai, T · Neurology · 2004

case_series · Level IV

Where this comes from

Abstract

The clinical and genetic findings are described for 16 patients from a large Italian family with a variant form of hereditary spastic paraplegia and congenital arachnoid cysts inherited as an autosomal dominant trait. A molecular study has revealed a novel missense mutation, T614I, in exon 17 of SPG4, which may play a role in both focal cortical dysgenesis and neurodegeneration of the motor neurons in the corticospinal tract.

Medical subject headings

Anatomy