A Case Report of Tomaculous Neuropathy (hereditary pressure sensitive neuropathy).

概要

論文の詳細を見る
A case of pressure-sensitive neuropathy, a 14 years old boy manifested by left drop foot, was reported.Physical examination revealed significant weakness of his E. H. L., T. A. and E. D. L muscles and diminished superficial sensation of his left ankle and foot. The left ulnar nerve showed reduced velocity at the elbow. The peroneal nerves showed reduced velocity at the head of the fibula. Sensory nerve conduction velocities were moderately slowed on the median, ulnar and sural nerves bilaterally, indicating generalized peripheral neuropathy. There were same findings in relatives who were studied on the nerve conduction velocities of the asymptomatic nerves.Histological examination of the sural nerve finally revealed segmental sausage-shaped thickenings of the myelin sheath (Tomacula) on teased nerve fibers. So the diagnosis of tomaculous neuropathy was made.It seems to be very important for the orthopaedic treatment of the entrapment neulopathy to differentiate this disease.The common clinical and histopathologic aspects of Tomaculous neuropathy were mentioned and the pathogenesis of acute nereve palsies, concerning axonal function, was shortly discussed.
西日本整形・災害外科学会の論文