HEREDITARY NEUROPATHY WITH LIABILITY TO PRESSURE PALSIES MASQUERADINGAS SLOWLY PROGRESSIVE POLYNEUROPATHY

Hereditary neuropathy with liability to pressure palsies (HNLPP) is a peripheral nerve disorder in which recurrent pressure palsies are asso ciated with pathological swelling ('tomacula') of myelinated nerve int ernodes. We report a patient with a slowly progressive polyneuropathy associated with substantial slowing of conduction velocities initially suggestive of an acquired or hereditary demyelinating polyneuropathy. However, repeat electrodiagnostic studies demonstrated that conductio n slowing and partial conduction block were localized primarily to com mon sites of nerve compression and entrapment. Sural nerve biopsy reve aled multiple tomacula consistent with the diagnosis of HNLPP.