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Verriello et al. Vessel Plus 2021;5:51 https://dx.doi.org/10.20517/2574-1209.2021.69 Page 9 of 11

CONCLUSIONS
Peripheral neuropathy is a common feature of hereditary and acquired forms of amyloidosis, with
considerable heterogeneity in the clinical manifestation.

The most common phenotype is a progressive length-dependent sensorimotor polyneuropathy with
autonomic dysfunction. However, there are many atypical presentations that often lead to delayed
diagnosis.

The prevalent type of hereditary amyloid neuropathy is represented by ATTRv amyloidosis with
polyneuropathy. In non-endemic areas, 52%-77% of cases do not show a positive family history and clinical
presentation of the disease is variable. Thus, diagnosis of ATTRv amyloidosis with polyneuropathy may be
missed and multiple misdiagnoses have been reported.

Often, sporadic cases may be misdiagnosed as CIDP, idiopathic axonal polyneuropathy, AL amyloidosis,
diabetic neuropathy, or lumbar spinal stenosis.

Subjects with progressively disabling polyneuropathy and one or more red flag symptoms suggestive of
multisystem involvement should be screened for ATTRv amyloidosis with polyneuropathy.

Early diagnosis of amyloid neuropathy allows rapid initiation of appropriate treatment that may change the
course and the progression of the disease.

DECLARATIONS
Authors’ contributions
Designed the review, performed all reviewing activities and drafted the manuscript: Verriello L,
Dalla Torre C, Pauletto G
Supervised all reviewing activities and provided critical contributions to the manuscript: Gigli GL
All authors approved the final version of the manuscript.

Availability of data and materials
Not applicable.

Financial support and sponsorship
None.

Conflicts of interest
All authors declared that there are no conflicts of interest.

Ethical approval and consent to participate
Not applicable.

Consent for publication
Not applicable.

Copyright
© The author(s) 2021.