ALS and Frontotemporal Dysfunction: A Review

Achi, Eugene; Rudnicki, Stacy A.

doi:10.1155/2012/806306

Cited by 60 publications

(49 citation statements)

References 103 publications

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“…58 Problems with compliance with therapy and support could contribute to this difference. 52,59 Alternatively, the difference in prognosis might simply reflect the disease burden (Figure 4).…”

Section: Cognitive Impairmentmentioning

confidence: 99%

The phenotypic variability of amyotrophic lateral sclerosis

2014

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| Classic textbook neurology teaches that amyotrophic lateral sclerosis (ALS) is a degenerative disease that selectively affects upper and lower motor neurons and is fatal 3-5 years after onset-a description which suggests that the clinical presentation of ALS is very homogenous. However, clinical and postmortem observations, as well as genetic studies, demonstrate that there is considerable variability in the phenotypic expression of ALS. Here, we review the phenotypic variability of ALS and how it is reflected in familial and sporadic ALS, in the degree of upper and lower motor neuron involvement, in motor and extramotor involvement, and in the spectrum of ALS and frontotemporal dementia. Furthermore, we discuss some unusual clinical characteristics regarding presentation, age at onset and disease progression. Finally, we address the importance of this variability for understanding the pathogenesis of ALS and for the development of therapeutic strategies.

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Section: Cognitive Impairmentmentioning

confidence: 99%

The phenotypic variability of amyotrophic lateral sclerosis

2014

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“…Sensory nerves and the autonomic nervous system are generally unaffected, meaning the majority of people with ALS maintain hearing, sight, touch, smell, and taste. Cognitive function is generally spared for most people, although some (approximately 5%) may develop frontotemporal dementia (Achi and Rudnicki, 2012). A higher proportion of people (30%-50%) also have more subtle cognitive changes, which may be unnoticed but are revealed by detailed neuropsychological testing.…”

Section: Tbi and Alsmentioning

confidence: 99%

Traumatic brain injury: a risk factor for neurodegenerative diseases

Gupta

Sen

2016

Reviews in the Neurosciences

114

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AbstractTraumatic brain injury (TBI), a major global health and socioeconomic problem, is now established as a chronic disease process with a broad spectrum of pathophysiological symptoms followed by long-term disabilities. It triggers multiple and multidirectional biochemical events that lead to neurodegeneration and cognitive impairment. Recent studies have presented strong evidence that patients with TBI history have a tendency to develop proteinopathy, which is the pathophysiological feature of neurodegenerative disorders such as Alzheimer disease (AD), chronic traumatic encephalopathy (CTE), and amyotrophic lateral sclerosis (ALS). This review mainly focuses on mechanisms related to AD, CTE, and ALS that are induced after TBI and their relevance to the advancement of these neurodegenerative diseases. This review encompasses acute effects and chronic neurodegenerative consequences after TBI for a better understanding of TBI-induced neuronal death and to design therapies that will effectively treat patients in the primary or secondary progressive stages.

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“…68 Contradicting this classical view that memory is relatively spared in ALS and FTD patients, a growing number of studies have recently reported severe and genuine memory impairments in these disorders. 72,[94][95][96] Indeed, a good proportion of pathologically confirmed FTD patients show significant episodic memory problems, indicating that MTL areas, particularly hippocampal and parahippocampal regions, are clearly affected in the syndrome 43,45,46,97 and that executive dysfunctions caused by frontal atrophy are not the main contributor of memory impairment in FTD. 43 As for ALS, memory deficits have been controversial and most studies link memory impairment to executive dysfunction.…”

Section: Clinical/neuropsychological Findingsmentioning

confidence: 99%

How predictive are temporal lobe changes of underlying TDP-43 pathology in the ALS-FTD continuum?

Bueno

Bertoux

Souza

et al. 2017

Ann Clin Neurophysiol

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Detection of underling proteinopathies is becoming increasingly important across neurodegenerative conditions due to upcoming disease intervention trials. In this review, we explored how temporal lobe changes in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) can potentially predict underlying TDP-43 pathology subtypes in FTD. To date, emphasis has been given to frontal lobe changes in the study of the cognitive and behavioural impairments in both syndromes but an increasing number of pathological, imaging and neuropsychological studies suggest how temporal lobe changes could critically affect the cognition and behaviour of these conditions. In this current article, we reviewed pathological, imaging as well as clinical/neuropsychological findings of temporal involvement in the ALS-FTD continuum, how they relate to temporal lobe changes and the underlying TDP-43 pathology in FTD. Findings across studies show that TDP-43 pathology occurs and coincides in many structures in ALS and FTD, but especially in the temporal lobes. In particular, anterior and medial temporal lobes atrophy is consistently found in ALS and FTD. In addition, memory and language impairment as well as emotional and Theory of Mind processing deficits that are characteristics of the two diseases are highly correlated to temporal lobe dysfunction. We conclude by showing that temporal lobe changes due to TDP-43 type B might be particular predictive of TDP-43 type B pathology in behavioural variant FTD, which clearly needs to be investigated further in the future.

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ALS and Frontotemporal Dysfunction: A Review

Cited by 60 publications

References 103 publications

The phenotypic variability of amyotrophic lateral sclerosis

The phenotypic variability of amyotrophic lateral sclerosis

Traumatic brain injury: a risk factor for neurodegenerative diseases

How predictive are temporal lobe changes of underlying TDP-43 pathology in the ALS-FTD continuum?

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