[Hereditary hypersensitivity of the peripheral nerves to pressure]

The authors describe a case of hereditary neuropathy with increased sensitivity to pressure stressing its characteristic course facilitating the diagnosis. Electrophysiological investigations of the peripheral nerves demonstrated a generalized character of the disease process, morphological examination of the peripheral nerves demonstrated presence of characteristic although non-specific, limited myelin thickenings, different length of internodal segments, slight evidence of demyelination and remyelination, and presence of axonal component. The mechanism of development of these changes is still unexplained, possible myelinogenesis disturbances are discussed.