Scapuloperoneal neuropathy: a distinct clinicopathologic entity

Peripheral neuropathy as a cause for the scapuloperoneal syndrome continues to be controversial. This report provides further evidence in support of a scapuloperoneal neuropathy as a separate nosologic entity. Three men had a slowly progressive disorder of 5-17 years duration with prominent weakness and atrophy of scapular stabilizer, shoulder girdle and distal lower extremity muscles accompanied by a distal pan-modality sensory loss. Electrodiagnostic studies and sural nerve biopsies indicated a primary axonal neuropathy with secondary demyelination and remyelination.