Interpreting signals from the peripheral nerve in amyloidosis: a call for action

Abstract

Systemic amyloidosis includes a group of disorders, characterized by deposition of insoluble aggregates of amyloid fibrils in various tissues that lead to disruption of normal tissue structure and impaired function. They are currently categorized as hereditary and secondary. Peripheral neuropathy is a frequent complication of systemic amyloidosis. The most common phenotype is a length-dependent sensorimotor polyneuropathy with autonomic dysfunction, but there are many atypical presentations that often lead to delayed diagnosis. In this review, we emphasize the neurological clinical aspects that induce a suspicion of amyloidosis, the possible differential diagnosis and the diagnostic pitfalls. An early diagnosis of the disease is crucial for rapid initiation of appropriate treatment that may change the course and the progression of the disease.

Keywords

Amyloidosis / neuropathy / transthyretin

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