Hereditary transthyretin amyloidosis in middle-aged and elderly patients with idiopathic polyneuropathy: a nationwide prospective study.

Amyloidosis TTR gene epidemiology neuropathy transthyretin

Journal

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
ISSN: 1744-2818
Titre abrégé: Amyloid
Pays: England
ID NLM: 9433802

Informations de publication

Date de publication:
19 Oct 2023
Historique:
medline: 19 10 2023
pubmed: 19 10 2023
entrez: 19 10 2023
Statut: aheadofprint

Résumé

Hereditary transthyretin amyloidosis (ATTRv) is an adult-onset autosomal dominant disease resulting from In this 5 year-long (2017-2021) nationwide prospective study, we systematically analysed the 553 patients (70% males) with a mean age of 70 years were included. A In this nationwide prospective study, we found ATTRv in 2.7% of patients with age >50 years with a progressive polyneuropathy. These results are highly important for the early identification of patients in need of disease-modifying treatments.

Sections du résumé

BACKGROUND UNASSIGNED
Hereditary transthyretin amyloidosis (ATTRv) is an adult-onset autosomal dominant disease resulting from
METHODS UNASSIGNED
In this 5 year-long (2017-2021) nationwide prospective study, we systematically analysed the
RESULTS UNASSIGNED
553 patients (70% males) with a mean age of 70 years were included. A
CONCLUSION UNASSIGNED
In this nationwide prospective study, we found ATTRv in 2.7% of patients with age >50 years with a progressive polyneuropathy. These results are highly important for the early identification of patients in need of disease-modifying treatments.

Identifiants

pubmed: 37855400
doi: 10.1080/13506129.2023.2270661
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1-8

Auteurs

Guillaume Fargeot (G)

Neurology Department, CHU de Bicêtre, AP-HP, Le-Kremlin-Bicêtre, France.

Andoni Echaniz-Laguna (A)

Neurology Department, CHU de Bicêtre, AP-HP, Le-Kremlin-Bicêtre, France.
French National Reference Center for Rare Neuropathies (NNERF), Le-Kremlin-Bicêtre, France.
Inserm U1195, Paris-Saclay University, Le-Kremlin-Bicêtre, France.

Céline Labeyrie (C)

Neurology Department, CHU de Bicêtre, AP-HP, Le-Kremlin-Bicêtre, France.
French National Reference Center for Rare Neuropathies (NNERF), Le-Kremlin-Bicêtre, France.

Juliette Svahn (J)

Electroneuromyography and Neuromuscular Department, Pierre Wertheimer Hospital, Hospices Civils de Lyon, Lyon, France.

Jean-Philippe Camdessanché (JP)

Department of Neurology, University Hospital of Saint-Etienne, Saint-Etienne, France.

Pascal Cintas (P)

Department of Neurology, AOC (Atlantique-Occitanie-Caraïbes) Reference Centre for Neuromuscular Diseases, Pierre Paul Riquet Hospital, CHU Toulouse, Toulouse, France.

Jean-Baptiste Chanson (JB)

Neurology Department, CHU de Strasbourg, Strasbourg, and Neuromuscular Reference Center Nord/Est/Ile de France (NEIF), Strasbourg, France.

Florence Esselin (F)

Explorations Neurologiques et Centre SLA, CHU et Université de Montpellier, INSERM, Montpellier, France.

Céline Piedvache (C)

Unité de Recherche Clinique Paris-Saclay, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.

Céline Verstuyft (C)

Service de Génétique Moléculaire, Pharmacogénétique et Hormonologie, Centre de Ressources Biologiques Paris Saclay, AP-HP, GH Paris Saclay, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.

Steeve Genestet (S)

Reference Centre for Neuromuscular Diseases AOC, University Hospital of Brest, Brest, France.

Emmeline Lagrange (E)

Department of Neurology, Grenoble Alpes University Hospital, Grenoble, France.

Laurent Magy (L)

Service de Neurologie, Centre de Référence Neuropathies Périphériques Rares, NNerf, UR 20218 NeurIT, CHU de Limoges, Hôpital Dupuytren, Limoges, France.

Yann Péréon (Y)

CHU Nantes, Reference Centre for Neuromuscular Diseases AOC, Hôtel-Dieu, Filnemus, Euro-NMD, Nantes, France.

Sabrina Sacconi (S)

Department of Clinical Neurosciences, Neuromuscular Diseases Centre, University Hospital of Nice (CHU), Nice, France.

Aissatou Signate (A)

Department of Neurology, CHU Martinique (University Hospital of Martinique), Fort de France, France.

Aleksandra Nadaj-Pakleza (A)

Neurology Department, CHU de Strasbourg, Strasbourg, and Neuromuscular Reference Center Nord/Est/Ile de France (NEIF), Strasbourg, France.

Frédéric Taithe (F)

Department of Neurology, University Hospital of Clermont-Ferrand (CHU Clermont-Ferrand - Gabriel Montpied Hospital), Clermont-Ferrand, FT, France.

Karine Viala (K)

Neurophysiology Department, AP-HP, Sorbonne Université, Pitié-Salpêtrière Hospital, Paris, France.

Céline Tard (C)

Centre de référence des maladies Neuromusculaires Nord/Est/Ile-de-France, U1172, CHU de Lille, Lille, France.

Vianney Poinsignon (V)

Service de Génétique Moléculaire, Pharmacogénétique et Hormonologie de Bicêtre, Hôpitaux Universitaires Paris-Saclay, Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Le Kremlin Bicêtre, France.

Cécile Cauquil (C)

Neurology Department, CHU de Bicêtre, AP-HP, Le-Kremlin-Bicêtre, France.
French National Reference Center for Rare Neuropathies (NNERF), Le-Kremlin-Bicêtre, France.

Shahram Attarian (S)

Centre de Référence des Maladies Neuromusculaires et de la SLA, APHM, CHU Timone, Marseille, France.

David Adams (D)

Neurology Department, CHU de Bicêtre, AP-HP, Le-Kremlin-Bicêtre, France.
French National Reference Center for Rare Neuropathies (NNERF), Le-Kremlin-Bicêtre, France.

Classifications MeSH