Peripheral nerve involvement in wild-type transthyretin amyloidosis.
Neurol Sci
; 44(1): 351-354, 2023 Jan.
Article
em En
| MEDLINE
| ID: mdl-36260260
ABSTRACT
INTRODUCTION AND AIMRPOSE Neurological involvement other than carpal tunnel syndrome (CTS) has rarely been observed in wild-type transthyretin amyloidosis (ATTRwt). The aim of our study was to investigate peripheral nerve involvement in ATTRwt. METHODS:
Patients diagnosed with ATTRwt (negative molecular testing, confirmed cardiac uptake at bone scintigraphy, Perugini score 2 or 3) were considered. Sixteen men (mean age 75 ± 6.2, range 65-86 years) were enrolled. Neurological examination (Neuropathy Impairment Score, NIS), questionnaires on autonomic function and quality of life (QoL), electrodiagnostic studies (EDX), nerve ultrasound, and Sudoscan (electrochemical skin conductance, ESC) were performed. The presence of peripheral neuropathy was defined according to the detection of any abnormal finding at lower limbs other than CTS at EDX studies, regardless of NIS scores.RESULTS:
Ten (62.5%) ATTRwt had abnormal NIS scores. At EDX, CTS was observed in 13/16 (81.2%), with 3/16 (18.8%) presenting also axonal peripheral neuropathy. Extensive workup ruled out common causes of neuropathy. Eight (50%) ATTRwt patients had orthostatic hypotension (OH). Abnormal ESC was observed in 9/14 (64%) ATTRwt patients.DISCUSSION:
Despite being uncommon, we observed peripheral nervous system involvement in ATTRwt (large and small fiber dysfunction). Being elderly, ATTRwt patients may have age-related conditions acting as confounding factors for the diagnosis of neuropathy that however can be detected with a careful examination and use of specific tests, including those for autonomic dysfunction.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome do Túnel Carpal
/
Neuropatias Amiloides Familiares
Limite:
Aged
/
Aged80
/
Humans
/
Male
Idioma:
En
Revista:
Neurol Sci
Assunto da revista:
NEUROLOGIA
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Itália