Evidence map›Paper›PMID 31907599›Full record

ReviewJournal of neurology2021

Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy.

David Adams, Yukio Ando, João Melo Beirão, Teresa Coelho, Morie A Gertz, Julian D Gillmore, Philip N Hawkins, Isabelle Lousada, Ole B Suhr, Giampaolo Merlini

Open access · hybridAbstract readReviewConsensus Statement
In one paragraph

Review in Journal of neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 152 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
152citing papers in PubMed, 1 pooled it
13.5field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

152 citing papers in PubMed, 1 synthesis or guideline pooled it, 304 citations in OpenAlex.

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  13. [Genes & diseases · 2026
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  18. Eplontersen for the Treatment of Hereditary Transthyretin Amyloidosis.Methods in molecular biology (Clifton, N.J.) · 2026
    Article
  19. Article
  20. Review

92 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 9 institutions in 7 countries.

David AdamsDepartment of Neurology, French National Reference Centre for Familial Amyloidotic Polyneuropathy, CHU Bicêtre, Université Paris-Saclay APHP, INSERM U1195, 94276, Le Kremlin-Bicêtre, France. david.adams@aphp.fr.ORCID http://orcid.org/0000-0002-8722-4108
Yukio AndoDepartment of Neurology, Graduate School of Medical Sciences, Kumamoto, Japan.
João Melo BeirãoOphthalmology Service, Hospital de Santo António, Porto, Portugal.
Teresa CoelhoCentro Hospitalar Do Porto, Porto, Portugal.
Morie A GertzMayo Clinic, Rochester, MN, USA.
Julian D GillmoreNational Amyloidosis Centre, University College London, London, UK.
Philip N HawkinsNational Amyloidosis Centre, University College London, London, UK.
Isabelle LousadaAmyloidosis Research Consortium, Boston, MA, USA.
Ole B SuhrDepartment of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
Giampaolo MerliniAmyloidosis Center Foundation, IRCCS Policlinico San Matteo, San Matteo, Italy.
University College London · GBAmyloidosis Foundation · USCentro Hospitalar do Porto · PTHospital de Santo António · PTInserm · FRKumamoto University · JPMayo Clinic in Arizona · USUmeå University · SEUniversity of Pavia · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Amyloid transthyretin (ATTR) amyloidosis with polyneuropathy (PN) is a progressive, debilitating, systemic disease wherein transthyretin protein misfolds to form amyloid, which is deposited in the endoneurium. ATTR amyloidosis with PN is the most serious hereditary polyneuropathy of adult onset. It arises from a hereditary mutation in the TTR gene and may involve the heart as well as other organs. It is critical to identify and diagnose the disease earlier because treatments are available to help slow the progression of neuropathy. Early diagnosis is complicated, however, because presentation may vary and family history is not always known. Symptoms may be mistakenly attributed to other diseases such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), idiopathic axonal polyneuropathy, lumbar spinal stenosis, and, more rarely, diabetic neuropathy and AL amyloidosis. In endemic countries (e.g., Portugal, Japan, Sweden, Brazil), ATTR amyloidosis with PN should be suspected in any patient who has length-dependent small-fiber PN with autonomic dysfunction and a family history of ATTR amyloidosis, unexplained weight loss, heart rhythm disorders, vitreous opacities, or renal abnormalities. In nonendemic countries, the disease may present as idiopathic rapidly progressive sensory motor axonal neuropathy or atypical CIDP with any of the above symptoms or with bilateral carpal tunnel syndrome, gait disorders, or cardiac hypertrophy. Diagnosis should include DNA testing, biopsy, and amyloid typing. Patients should be followed up every 6-12 months, depending on the severity of the disease and response to therapy. This review outlines detailed recommendations to improve the diagnosis of ATTR amyloidosis with PN.

Indexed as

Amyloid Neuropathies, FamilialPolyneuropathiesAdultBrazilConsensusHumansJapanPortugalPrealbuminSwedenPrealbuminATTR amyloidosisATTRvDiagnosishATTRPeripheral neuropathyTransthyretin amyloidosis

Identifiers

PMID31907599
PMCPMC8179912
OpenAlexW2996947593

What OpenQuestion holds

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LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.