Primary Systemic Amyloidosis presenting with Demyelinating Features

Abstract

PURPOSE: Primary systemic amyloidosis is a rare disorder that has multisystemic manifestations. The most common neuropathy in systemic amyloidosis is a small-fiber axonal polyneuropathy. When the neuropathy is the presenting feature, diagnosis is usually delayed. The diagnosis of systemic amyloidosis may be more difficult when patients present with an atypical polyneuropathy. METHODS: Two cases of primary systemic amyloidosis with a multifocal polyneuropathy with demyelinating features are presented. RESULTS: The patients reported in this series with autopsy proven amyloidosis had evidence of a polyneuropathy with demyelinating features. CONCLUSIONS: Amyloidosis should be considered in the differential when a patients presents with a polyneuropathy that has demyelinating features.