Cited by 80Dementia and Cognitive Impairment ResearchAmyotrophic Lateral Sclerosis ResearchNeurobiology of Language and Bilingualism

Speech and language impairments in behavioral variant frontotemporal dementia: A systematic review

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Geraudie, Amandine, Battista, Petronilla, García, Adolfo M., Allen, Isabel E., Miller, Zachary A., Gorno-Tempini, Maria Luisa, Montembeault, Maxime·2021·Neuroscience & Biobehavioral Reviews

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Abstract

Although behavioral variant frontotemporal dementia (bvFTD) is classically defined by behavioral and socio-emotional changes, impairments often extend to other cognitive functions. These include early speech and language deficits related to the disease's core neural disruptions. Yet, their scope and clinical relevance remains poorly understood. This systematic review characterizes such disturbances in bvFTD, considering clinically, neuroanatomically, genetically, and neuropathologically defined subgroups. We included 181 experimental studies, with at least 5 bvFTD patients diagnosed using accepted criteria, comparing speech and language outcomes between bvFTD patients and healthy controls or between bvFTD subgroups. Results reveal extensive and heterogeneous deficits across cohorts, with (a) consistent lexico-semantic, reading & writing, and prosodic impairments; (b) inconsistent deficits in motor speech and grammar; and (c) relative preservation of phonological skills. Also, preliminary findings suggest that the severity of speech and language deficits might be associated with global cognitive impairment, predominantly temporal or fronto-temporal atrophy and MAPT mutations (vs C9orf72). Although under-recognized, these impairments contribute to patient characterization and phenotyping, while potentially informing diagnosis and management.

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  • Distinct linguistic deficits have been observed in behavioural variant frontotemporal dementia, including picture naming deficits, atypical speech rhythm and poor reading skills.

    “behavioural variant frontotemporal de mentia (picture naming deficits, 6,32 atypical speech rhythm, poor reading skills 6 ).”
  • The evidence on cross-linguistic differences in behavioural variant frontotemporal dementia is altogether mixed.

    “That being said, the evidence is altogether mixed, 6 calling for harmonized protocols that enable robust comparisons across languages while ac counting for socio-cultural factors in this syndrome. 32”
  • English has been targeted by 65% of verbal production studies on Parkinson's disease and by 74% of speech and language studies on behavioural variant frontotemporal dementia.

    “Furthermore, English has been targeted by 65% of verbal production studies on Parkinson's disease 82 and by 74% of speech and language stud ies on behavioural variant frontotemporal dementia 6”
  • Some evidence suggests that lexico-semantic skills are more frequently impaired in English than in Spanish-speaking cohorts with behavioural variant frontotemporal dementia.

    “Yet, some evidence suggests that lexico-semantic skills are more fre quently impaired in English than in Spanish-speaking cohorts. 6”
  • Recent works underscore the broad clinical utility of speech and language testing in these and other neurodegenerative disorders, even if many deficits are secondary to broader motoric or cognitive dysfunction.

    “However, recent works underscore the broad clinical utility of speech and language testing in these 4,6 and other 140 neurodegenerative disor ders, even if many deficits are secondary to broader motoric or cogni tive dysfunction.”

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Authors
Geraudie, A., Battista, P., García, A. M., Allen, I. E., Miller, Z. A., Gorno-Tempini, M. L., & Montembeault, M.
Affiliations
  • University of California, San Francisco (US)
  • Universidad de Santiago de Chile (CL)
Corresponding author
Montembeault, Maxime
Venue
Neuroscience & Biobehavioral Reviews
Publisher
Elsevier BV
Published
2021-12
Volume
131
Pages
1076-1095
ISSN
0149-7634 (ISSN-L)
OpenAlex
W3205923164
Cited by
80
References
228
Citations by year
2026: 13 · 2025: 16 · 2024: 20 · 2023: 16 · 2022: 13 · 2021: 2
FWCI
2.27
Type
journal article
Language
en
Open access
Locations
  • Neuroscience & Biobehavioral Reviews (publishedVersion)
  • medRxiv (acceptedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
  • Conicet (submittedVersion, cc-by-nc-sa)
Funders
  • Universidad de Santiago de Chile
  • Consejo Nacional de Investigaciones Científicas y Técnicas (1210176, 1210195)
  • Agencia Nacional de Investigación y Desarrollo (1210195, 1210176)
  • University of California, San Francisco
  • Canadian Institutes of Health Research
  • Fonds de Recherche du Québec - Santé
  • National Institute on Aging (P01AG019724)
  • National Institute of Neurological Disorders and Stroke (R01NS050915)
Topics
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Neurobiology of Language and Bilingualism
Keywords
behavioral variant frontotemporal dementia, speech disorders, language impairment, motor speech, grammar, phonological skills, MAPT mutations, C9orf72, global cognitive impairment, disease phenotyping
MeSH terms
Atrophy, Humans, Language, Language Development Disorders, Neuropsychological Tests, Speech, Frontotemporal Dementia

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