Hereditary liability to pressure palsy : a clinical electrophysiological and histomorphometric case study

An 18 years old female patient presenting with a peroneal nerve palsy after trivial pressure has been reported. A diagnosis of hereditary liability to pressure palsy was not suspected until electrodiagnostic studies were performed. These revealed the presence of a demyelinating polyneuropathy with conduction block and differential affection of various segments of the nerves. Sural nerve histology was charcterised by the presence of suasage like swellings and segmental demyelination and remyelination in teased fibres and mild loss of large myelinated fibres.