HNPP-Hereditary neuropathy

Liability to Pressure Palsy
HNPP-Hereditary neuropathy with liability to pressure palsy·Autosomal dominant trait with total penetrance but variable expression.·       Deletion of the PMP-22 gene on chromosome 17.

·       Histopathology studies-tomaculous “sausage like” neuropathy.  Focal thickening of myelin sheaths, with apparent compression and flattening of axons at such sites.  May have extensive demyelination with re-myelination and onion bulb formation.

·       Clinical presentation-single or multiple nerve involvement.  May occur after mild compression or traction.  Symptoms may reduce, but neurologic abnormalities may persist.

·       Median, ulnar, radial, peroneal neuropathy common. NCV changes may occur in clinically unaffected nerves.

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Ray Jurewicz
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