Patterns of myelinated nerve fibers loss in transthyretin amyloid polyneuropathy and mimics.
Ann Clin Transl Neurol
; 9(7): 1059-1068, 2022 07.
Article
em En
| MEDLINE
| ID: mdl-35665499
ABSTRACT
OBJECTIVE:
The present study was intended to analyze the characteristics of myelinated nerve fibers density (MFD) of transthyretin amyloid polyneuropathy (ATTR-PN) and other similar neuropathies.METHODS:
A total of 41 patients with ATTR-PN, 58 patients of other common peripheral neuropathies, and 17 age-and gender-matched controls who visited the First Hospital of Peking University and performed sural nerve biopsy between June 2007 and August 2021 were included for analysis of MFD.RESULTS:
Except the vasculitic neuropathy group, the total and small MFD of patients in the ATTR-PN group were significantly lower than those of other disease groups. There was an obvious negative correlation between the total MFD and the disease course in the ATTR-PN group. The disease course of early-onset and late-onset symptoms was similar, but the loss of large myelinated nerve fibers (MF) was more severe for the latter. In addition, all late-onset and most early-onset patients had severely reduced MFD after a 2 years' disease course. The MFD in ATTR-PN patients was negatively correlated with Neuropathy Impairment Score (NIS) and Norfolk Quality of life-diabetic neuropathy (Norfolk QOL-DN) score.CONCLUSION:
MF is lost differently in ATTR-PN and in other common peripheral neuropathies. The late-onset and early-onset ATTR-PN patients have different patterns of loss of large and small MF.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neuropatias Amiloides
/
Neuropatias Amiloides Familiares
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article