A Systematic Review of Behavioral Change in Motor Neuron Disease and Fronto-Temporal Dementia: Overlap, Differences, and Similarities
DOI:
https://doi.org/10.32628/IJSRST251232Keywords:
behavior, motor neuron disease, frontotemporal dementia, amyotrophic lateral sclerosisAbstract
Introduction: Motor neuron disease (MND) is a progressive neurological disorder, previously thought to exclusively affect the motor system. However, we now know that cognitive and behavioral change is also a part of the condition. MND exists on a spectrum with frontotemporal dementia (FTD), with up to 15% of people with MND also meeting diagnostic criteria for FTD. The overlap in symptoms can make diagnosis more difficult but also offer new opportunities for intervention and treatment. Aim: Our aim is to systematically review the academic literature to explore what behavioral symptoms have been identified as occurring in people with MND, FTD and MND-FTD. Method: We conducted a systematic review of academic literature on EMBASE, PubMed and Scopus on the 21st of August 2024. We searched for articles relating to behavioral symptoms in people affected by MND, FTD and MND-FTD using key terms. We included research published in the last ten years. Results: A total of 17 studies were included in the review. The most commonly reported behavioral symptoms were found to be apathy and disinhibition, with 53% and 59% of studies referencing them, respectively. Other commonly reported behavioral symptoms included a loss of sympathy or empathy, hyperorality, and psychosis. Impulsive and compulsive behaviors, along with stereotypies, were also reported, but with less frequency. Conclusion: There was a significant overlap of behavioral symptoms between MND and FTD, with almost all symptoms co-occurring between the two disorders. However, the severity and frequency of these symptoms did differ between MND and FTD, as explored in a few of the studies
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Cosseddu, M., A. Benussi, S. Gazzina, A. Alberici, V. Dell'Era, M. Manes, V. Cristillo, B. Borroni, and A. Padovani. "Progression of behavioural disturbances in frontotemporal dementia: a longitudinal observational study." European journal of neurology 27, no. 2 (2020): 265-272.
Chatzidimitriou, Electra, Panagiotis Ioannidis, Despina Moraitou, Eleni Konstantinopoulou, and Eleni Aretouli. "The cognitive and behavioral correlates of functional status in patients with frontotemporal dementia: A pilot study." Frontiers in Human Neuroscience 17 (2023): 1087765.
Chenji, S., A. Ishaque, D. Mah, et al., Neuroanatomical associations of the Edinburgh cognitive and Behavioural ALS screen (ECAS). Brain Imaging and Behavior, 2021. 15(3): p. 1641-1654.
Chio, A., et al., Prognostic factors in ALS: a critical review. Amyotrophic lateral sclerosis, 2009. 10(5-6): p. 310-323
Flanagan, E. C., Lagarde, J., Hahn, V., Guichart-Gomez, E., Sarazin, M., Hornberger, M., & Bertoux, M. (2018). Executive and social-cognitive determinants of environmental dependency syndrome in behavioral frontotemporal dementia. Neuropsychology, 32(4), 377–384. https://doi.org/10.1037/neu0000433
Francis, Rebecca, Stacie Attrill, Ratko Radakovic, and Sebastian Doeltgen. "Exploring clinical management of cognitive and behavioural deficits in MND. A scoping review." Patient Education and Counseling 116 (2023): 107942.
Goldstein, L.H. and S. Abrahams, Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. The Lancet Neurology, 2013. 12(4): p. 368-380.
Hodgins, F., S. Mulhern, and S. Abrahams, The clinical impact of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and neuropsychological intervention in routine ALS care. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2019: p. 1-8.
Johnson, E., & Kumfor, F. (2018). Overcoming apathy in frontotemporal dementia: challenges and future directions. Current Opinion in Behavioral Sciences, 22, 82-89.
Lulé, Dorothée E., Helena EA Aho-Özhan, Cynthia Vázquez, Ulrike Weiland, Jochen H. Weishaupt, Markus Otto, Sarah Anderl-Straub, Elisa Semler, Ingo Uttner, and Albert C. Ludolph. "Story of the ALS-FTD continuum retold: rather two distinct entities." Journal of Neurology, Neurosurgery & Psychiatry 90, no. 5 (2019): 586-589.
Le Heron, C., Holroyd, C. B., Salamone, J., & Husain, M. (2019). Brain mechanisms underlying apathy. Journal of Neurology, Neurosurgery & Psychiatry, 90(3), 302-312.
Moore, Katrina M., Jennifer Nicholas, Murray Grossman, Corey T. McMillan, David J. Irwin, Lauren Massimo, Vivianna M. Van Deerlin et al. "Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study." The Lancet Neurology 19, no. 2 (2020): 145-156.
National, C.G.C.U., Motor Neurone Disease: Assessment and Management. 2016.
Murphy, Jennifer, Pam Factor-Litvak, Raymond Goetz, Catherine Lomen-Hoerth, Peter L. Nagy, Jonathan Hupf, Jessica Singleton et al. "Cognitive-behavioral screening reveals prevalent impairment in a large multicenter ALS cohort." Neurology 86, no. 9 (2016): 813-820.
Morrow, Christopher B., Grace-Anna S. Chaney, Daniel Capuzzi, Arnold Bakker, Chiadi U. Onyike, and Vidyulata Kamath. "Hyperorality in frontotemporal dementia: Cognitive and psychiatric symptom profiles in early-stage disease." Journal of Alzheimer’s Disease 89, no. 4 (2022): 1203-1209.
Piguet, O., Kumfor, F., & Hodges, J. (2017). Diagnosing, monitoring and managing behavioural variant frontotemporal dementia. Medical Journal of Australia, 207(7), 303-308.
Radakovic, R., et al., Multidimensional apathy in ALS: validation of the Dimensional Apathy Scale. Journal of Neurology, Neurosurgery & Psychiatry, 2016. 87(6): p. 663-669
Ratnavalli E, Brayne C, Dawson K, et al. The prevalence of frontotemporal dementia. Neurology 2002; 58: 1615-1621
Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011; 134: 2456-2477
Renton, Alan E., Elisa Majounie, Adrian Waite, Javier Simón-Sánchez, Sara Rollinson, J. Raphael Gibbs, Jennifer C. Schymick et al. "A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD." Neuron 72, no. 2 (2011): 257-268.
Strong, M.J., et al., Amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotrophic lateral sclerosis and frontotemporal degeneration, 2017. 18(3-4): p. 153-174.
Tan, Y. L., Lo, Y. K. J., & Ho, C. S. H. (2024). Psychological and social impacts of frontotemporal dementia on caregivers and family members–a systematic review. General Hospital Psychiatry, 86, 33-49.
Yassuda, Mônica S., Thais B. Lima da Silva, Claire M. O'Connor, Shailaja Mekala, Suvarna Alladi, Valeria S. Bahia, Viviane Almaral-Carvalho et al. "Apathy and functional disability in behavioral variant frontotemporal dementia." Neurology: Clinical Practice 8, no. 2 (2018): 120-128.
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