OBSERVATION Central Nervous System Involvement in Hereditary Neuropathy With Liability to Pressure Palsies Description of a Large Family With This Association

Objective: To describe a large family with hereditary neuropathy with liability to pressure palsies associated with central nervous system demyelination. Design: We examined the 18 members of a pedigree. Genetic analysis was performed on 15 subjects, stan-dard nerve conduction studies on 10 subjects, and brain magnetic resonance imaging studies on 8 subjects. Results: Hereditary neuropathy with liability to pres-sure palsies was confirmed in 9 patients of the pedigree. Brainmagnetic resonance imaging findings showedmul-tiple areas of demyelination in 6 of 6 affected members andwere normal in 2 of 2 healthy relatives.Magnetic reso-nance imaging abnormalities were predominantly lo-cated in the subcortical frontal white matter. All pa-tients had acute and recurrent nerve palsies, while clinica