Hereditary pressure-sensitive neuropathy

Abstract

Clinical and neurophysiological studies were carried out in hereditary pressure-sensitive neuropathy. In 4 generations, 19 out of 87 family members were affected. The transmission of the disorder is autosomal dominant with total penetrance but variable expression. The motor conduction velocities of peripheral nerves were slowed even in clinically unaffected nerves. Light- and electron-microscopic studies were performed in the fibular nerve of one patient. A characteristic tomaculous neuropathy was discovered.