Amyotrophic lateral sclerosis & frontotemporal degeneration · 2026
Cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA): a systematic review.
СтатьяСистематический обзорНазвание и аннотация: Перевод в очереди
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De Luca Giulia, Aiello Edoardo Nicolò, Moreschi Arianna, Curti Beatrice, Marcato Sonia, Paredi Federica, Verde Federico, Silani Vincenzo, Ticozzi Nicola, Poletti Barbara, Sorarù Gianni
Аннотация
OBJECTIVE: This study aimed at reviewing currently available evidence on cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA).
METHODS: This review was performed in accordance with the 2020 PRISMA guidelines. We included case reports/series and group studies reporting data on the neuropsychological features of SBMA patients.
RESULTS: Out of 123 unique records identified, 11 studies were included (N = 5 case reports/series; N = 6 group studies), comprising 151 SBMA patients. Among case reports and series, 6 out of 10 patients were reported to exhibit some degree of neuropsychological involvement, with two being diagnosed with a dementia condition resembling the characteristics of behavioral variant-frontotemporal dementia. Three of the 6 case-control studies found no cognitive differences between SBMA patients and healthy controls. Cognitive deficits reported in group studies mostly involved the executive, attentional, memory and visuo-spatial/-constructive domains.
CONCLUSIONS: The available evidence on cognitive and behavioral involvement in SBMA is scarce and conflicting. However, it is conceivable that at least some patients may exhibit dysexecutive-type changes, possibly reflecting a frontal system involvement.
METHODS: This review was performed in accordance with the 2020 PRISMA guidelines. We included case reports/series and group studies reporting data on the neuropsychological features of SBMA patients.
RESULTS: Out of 123 unique records identified, 11 studies were included (N = 5 case reports/series; N = 6 group studies), comprising 151 SBMA patients. Among case reports and series, 6 out of 10 patients were reported to exhibit some degree of neuropsychological involvement, with two being diagnosed with a dementia condition resembling the characteristics of behavioral variant-frontotemporal dementia. Three of the 6 case-control studies found no cognitive differences between SBMA patients and healthy controls. Cognitive deficits reported in group studies mostly involved the executive, attentional, memory and visuo-spatial/-constructive domains.
CONCLUSIONS: The available evidence on cognitive and behavioral involvement in SBMA is scarce and conflicting. However, it is conceivable that at least some patients may exhibit dysexecutive-type changes, possibly reflecting a frontal system involvement.