Syntactic comprehension deficits across the FTD-ALS continuum

Background A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone. Methods A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms. Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment. Results A number of behavioural and affective changes were reported more frequently in bvFTD than ALS-FTD: social disinhibition (p<0.001), inertia (p<0.001), loss of sympathy and empathy (p=0.008), repetitive behaviours (p<0.001) and dietary changes (p<0.001). Warmth of affect demonstrated in the clinic setting was reported more often in ALS-FTD than bvFTD (p<0.001). Executive impairments occurred equally in both groups. Language impairments were more common in ALS-FTD than bvFTD: agrammatism (p<0.017) and impaired sentence comprehension (p<0.036). Psychotic features were relatively rare and did not distinguish the groups. Conclusions Our findings suggest differences between bvFTD and ALS-FTD. In particular, while changes in social behaviour are prominent in bvFTD alone, there may be a comparatively greater degree of language impairment in ALS-FTD. Prospective exploration of the pattern of differences between these groups will be essential. Identification of a distinct neuropsychological phenotype in ALS-FTD may have clinical implications for early diagnosis, disease management and care planning and theoretical implications for our understanding of the relationship between ALS and FTD.

show abstract

“…These features have been reported in the ALS literature, 21 22 and recently, syntactic comprehension deficits have been noted in ALS-FTD. 17 …”

Section: Discussionmentioning

confidence: 99%

Examining the language and behavioural profile in FTD and ALS-FTD

Saxon

Thompson

Jones

et al. 2017

J Neurol Neurosurg Psychiatry

View full text Add to dashboard Cite

show abstract

“…Neuroimaging, neuropathological data, and genetic studies suggest that ALS and FTD might form part of a disease continuum [143][144][145]. Indeed, a number of genes linked with ALS and FTD are involved in the same cellular pathways.…”

Section: Amyotrophic Lateral Sclerosis and Frontotemporal Dementiamentioning

confidence: 99%

Innate Immunity: A Common Denominator between Neurodegenerative and Neuropsychiatric Diseases

Novellino

Saccà

Donato

et al. 2020

IJMS

View full text Add to dashboard Cite

The intricate relationships between innate immunity and brain diseases raise increased interest across the wide spectrum of neurodegenerative and neuropsychiatric disorders. Barriers, such as the blood–brain barrier, and innate immunity cells such as microglia, astrocytes, macrophages, and mast cells are involved in triggering disease events in these groups, through the action of many different cytokines. Chronic inflammation can lead to dysfunctions in large-scale brain networks. Neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, amyotrophic lateral sclerosis, and frontotemporal dementia, are associated with a substrate of dysregulated immune responses that impair the central nervous system balance. Recent evidence suggests that similar phenomena are involved in psychiatric diseases, such as depression, schizophrenia, autism spectrum disorders, and post-traumatic stress disorder. The present review summarizes and discusses the main evidence linking the innate immunological response in neurodegenerative and psychiatric diseases, thus providing insights into how the responses of innate immunity represent a common denominator between diseases belonging to the neurological and psychiatric sphere. Improved knowledge of such immunological aspects could provide the framework for the future development of new diagnostic and therapeutic approaches.

show abstract

“…Linguistic impairments may include deficits in syntactic processing, verb naming and action verb processing, semantic and verbal paraphasias, and syntactic comprehension deficits. Kamminga et al (2016) found that syntactic comprehension was defective in 25% of patients with ALS without dementia. Leslie et al (2015) observed semantic deficits in 35% of not demented ALS patients, and Hervieu-Bègue et al (2016) in as many as 60% of such patients.…”

Section: Cognitive Emotional and Behavioral Changes In Alsmentioning

confidence: 99%

Cognitive, Emotional and Psychological Manifestations in Amyotrophic Lateral Sclerosis at Baseline and Overtime: A Review

et al. 2019

View full text Add to dashboard Cite

It is now well recognized that, in addition to motor impairment, amyotrophic lateral sclerosis (ALS) may cause extra-motor clinical signs and symptoms. These can include the alteration of certain cognitive functions, impaired social cognition, and changes in the perception and processing of emotions. Where these extra-motor manifestations occur in ALS, they usually do so from disease onset. In about 10% of cases, the cognitive and behavioral changes meet the diagnostic criteria for frontotemporal dementia. The timecourse of behavioral and cognitive involvement in ALS is unclear. Whereas longitudinal studies have failed to show cognitive decline over time, some cross-sectional studies have demonstrated poorer cognitive performances in the advanced stages of the disease. Neuroimaging studies show that in ALS, extra-motor signs and symptoms are associated with specific brain lesions, but little is known about how they change over time. Finally, patients with ALS appear less depressed than might be expected, given the prognosis. Moreover, many patients achieve satisfactory psychosocial adjustment throughout the course of the disease, regardless of their degree of motor disability. There are scant longitudinal data on extra-motor impairment in ALS, and to our knowledge, no systematic review on this subject has yet been published. Even so, a better understanding of patients’ clinical trajectory is essential if they are to be provided with tailored care and given the best possible support. We therefore undertook to review the evidence for extra-motor changes and their time course in ALS, in both the cognitive, emotional and psychological domains, with a view to identifying mechanisms that may help these patients cope with their disease.

show abstract

Syntactic comprehension deficits across the FTD-ALS continuum

Cited by 26 publications

References 49 publications

Examining the language and behavioural profile in FTD and ALS-FTD

Examining the language and behavioural profile in FTD and ALS-FTD

Innate Immunity: A Common Denominator between Neurodegenerative and Neuropsychiatric Diseases

Cognitive, Emotional and Psychological Manifestations in Amyotrophic Lateral Sclerosis at Baseline and Overtime: A Review

Contact Info

Product

Resources

About