ALS and frontotemporal dysfunction: a review
Open accessAbstract
Though once believed to be a disease that was limited to the motor system, it is now apparent that amyotrophic lateral sclerosis (ALS) may be associated with cognitive changes in some patients. Changes are consistent with frontotemporal dysfunction, and may range from mild abnormalities only recognized with formal neuropsychological testing, to profound frontotemporal dementia (FTD). Executive function, behavior, and language are the most likely areas to be involved. Screening helpful in detecting abnormalities includes verbal or categorical fluency, behavioral inventories filled out by the caregiver, and evaluation for the presence of depression and pseudobulbar affect. Patients with cognitive dysfunction have shortened survival and may be less compliant with recommendations regarding use of feeding tubes and noninvasive ventilation. Evolving knowledge of genetic and pathological links between ALS and FTD has allowed us to better understand the overlapping spectrum of ALS and FTD.
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Patients with MND can feel frustration at the effects of the illness.
“This included being frustrated (2, 8, 11, 12, 15, 21, 25) at the effects of the illness.”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND can relate to peers who are in a similar situation.
“Patients could relate to peers who were in a similar situation (2, 16, 17, 19, 25, 29).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Some patients with MND identify the need for endurance of their situation and resilience against it, combined with a determination to continue to live.
“Others identified the need for patient endurance of their situation and having resilience against it, combined with a determination to continue to live (2, 4, 7, 13, 14).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Individuals with MND can feel fear and anxiety about their future deterioration, as well as being afraid of death.
“Individuals could feel fear and anxiety about their future deterioration, as well as being afraid of death (2, 10, 11, 12, 13, 14, 15, 21, 23, 25, 26, 27, 28).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND used alternative therapies, adapted health behaviors including rehabilitative exercises, considering care routines, or undertaking hobbies or activities, and made dietary adaptations.
“This included, using alternative therapies (2, 11, 16), adapting health behaviors including rehabilitative exercises, considering care routines, or undertaking hobbies or activities (5, 11, 16) and making dietary adaption (2, 9, 16).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND use the strategy of focusing on what could be done or achieved rather than what they couldn't do or what they had lost.
“(c) Focusing on what could be done or achieved (2, 5, 6, 7, 11, 12, 13, 14, 27), rather than what they couldn't or what they had lost.”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND use the strategy of bringing time closer and focusing on the ability to cope with the present circumstances.
“Several strategies were used by patients in dealing with the symptoms and problems they faced, including: (a) Bringing time closer and focusing on the ability to cope with the present circumstances (2, 10, 14, 18, 25, 27).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND use the strategy of dealing with problems as they arrive and affect their life.
“(b) Dealing with problems as they arrive and affect their life (2, 7, 10, 11, 12, 14, 18, 25, 29).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND took action as a way of controlling aspects of their life that were controllable.
“but also take action as a way of controlling aspects of their life that were controllable (2, 4, 5, 11, 12, 14, 18, 19).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND consistently identified the need to search for information in order to feel more in control.
“Individuals consistently identified the need to search for information in order to feel more in control (2, 4, 5, 9, 10, 12, 13, 14, 15, 22, 25, 26)”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
Patients with MND often suggested that the best they could do was to live positively and make the most of the time they had left.
“Patients often suggested that the best they could do was to live positively and make the most of the time they had left (2, 10, 11, 14, 15, 18, 20, 27, 29).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [2]
A spectrum of cognitive changes will affect patients' psychological processes, although only few individuals experience dementia.
“it is worth noting that a spectrum of cognitive changes will affect patients psychological processes, although only few individuals experience dementia (Achi and Rudnicki, 2012).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as Achi and Rudnicki, 2012
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Flagged first, then by citations
Research works printed but not linked: 2
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- [55]Rudnicki SA Fewell D The Cambridge Behavior Inventory in evaluating frontotemporal function in patients with motor neuron disease Amyotroph Lateral Scler 2007 8 supplement 1 27 28
Cited by
Sorted by citations
Sullivan, P. M., Zhou, X., Robins, A. M., Paushter, D. H., Kim, D., Smolka, M. B., & Hu, F. (2016) · Acta Neuropathologica Communications · doi:10.1186/s40478-016-0324-5 · Cited by 295 · see citing works
Soundy, A., & Condon, N. (2015) · Frontiers in Psychology · doi:10.3389/fpsyg.2015.00606 · Cited by 35 · see citing works
- Authors
- Achi, E. Y., & Rudnicki, S. A.
- Affiliations
- University of Arkansas for Medical Sciences (US)
- Corresponding author
- Rudnicki, Stacy A.
- Venue
- Neurology Research International
- Publisher
- Hindawi Limited
- Published
- 2012
- Volume
- 2012
- Pages
- 1-9
- ISSN
- 2090-1852 (ISSN-L)
- 2090-1860
- PMC
- PMC3423946
- OpenAlex
- W1968615349
- Cited by
- 74
- References
- 112
- Citations by year
- 2026: 4 · 2025: 2 · 2024: 4 · 2023: 1 · 2022: 1 · 2021: 6 · 2020: 6 · 2019: 5 · 2018: 2 · 2017: 9 · 2016: 10 · 2015: 12 · 2014: 8 · 2013: 3 · 2012: 1
- FWCI
- 5.02
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Neurology Research International (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- amyotrophic lateral sclerosis, frontotemporal dementia, cognitive impairment, executive function, behavior change, language impairment, pseudobulbar affect, depression, neuropsychological screening, survival, tube feeding, non-invasive ventilation, genetic overlap
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