Central neurodegeneration in Kennedy’s disease accompanies peripheral motor dysfunction

Autor:	Sicong Tu, Tiffany Li, Antonia S. Carroll, Colin J. Mahoney, William Huynh, Susanna B. Park, Robert Henderson, Steve Vucic, Matthew C. Kiernan, Cindy S-Y Lin
Jazyk:	angličtina
Rok vydání:	2024
Předmět:	Kennedy’s disease Spinobulbar muscular atrophy Amyotrophic lateral sclerosis MRI Diffusion-weighted imaging Peripheral nerve Medicine Science
Zdroj:	Scientific Reports, Vol 14, Iss 1, Pp 1-10 (2024)
Druh dokumentu:	article
ISSN:	2045-2322
DOI:	10.1038/s41598-024-69393-5
Popis:	Abstract Spinal and bulbar muscular atrophy (SBMA), or Kennedy’s disease (KD), is a rare hereditary neuromuscular disorder demonstrating commonalities with amyotrophic lateral sclerosis (ALS). The current study aimed to define functional and central nervous system abnormalities associated with SBMA pathology, their interaction, and to identify novel clinical markers for quantifying disease activity. 27 study participants (12 SBMA; 8 ALS; 7 Control) were recruited. SBMA patients underwent comprehensive motor and sensory functional assessments, and neurophysiological testing. All participants underwent whole-brain structural and diffusion MRI. SBMA patients demonstrated marked peripheral motor and sensory abnormalities across clinical assessments. Increased abnormalities on neurological examination were significantly associated with increased disease duration in SBMA patients (R2 = 0.85, p
Databáze:	Directory of Open Access Journals
Externí odkaz:	https://doaj.org/article/3a729a0a8aaa4b3e9eddf7e3187b6ec6 Zobrazit plný text záznamu View record in DOAJ Plný text ve formátu PDF Plný text ve formátu HTML
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