{"title":"Cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA): a systematic review.","authors":"Giulia De Luca, Edoardo Nicolò Aiello, Arianna Moreschi, Beatrice Curti, Sonia Marcato, Federica Paredi, Federico Verde, Vincenzo Silani, Nicola Ticozzi, Barbara Poletti, Gianni Sorarù","doi":"10.1080/21678421.2025.2608228","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>This study aimed at reviewing currently available evidence on cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>Out of 123 unique records identified, 11 studies were included (<i>N</i> = 5 case reports/series; <i>N</i> = 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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"652-670"},"PeriodicalIF":2.8000,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Amyotrophic lateral sclerosis & frontotemporal degeneration","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1080/21678421.2025.2608228","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2026/3/13 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
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Abstract
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.
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