source: Current Problems in Cancer

year: 2016

authors: Pearson KT, Vota S

The amyloid neuropathies are rare, rapidly progressive systemic disorders that often are misdiagnosed when initially presenting as a peripheral neuropathy.1 Light chain (AL) amyloidosis, an acquired disorder, and familial amyloid polyneuropathy, a hereditary disorder, may each cause severe and disabling peripheral neuropathies, in addition to other systemic manifestations.

Diagnosis may be delayed, especially in the familial amyloid neuropathies (FAP), based on atypical presentation, late onset, or lack of positive family history. Early diagnosis is imperative as there are available therapies that may slow disease progression. This review will focus on the presentation, diagnosis, pathology, and treatment of both the FAP and light chain (AL) amyloidosis to encourage and assist in more rapid diagnosis and early intervention.

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