The anatomy of cognitive impairment in amyotrophic lateral sclerosis: More than frontal lobe dysfunction

Tsermentseli, Stella; Leigh, P. Nigel; Goldstein, Laura H.

doi:10.1016/j.cortex.2011.02.004

Cited by 103 publications

(91 citation statements)

References 130 publications

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“…In addition to PMC, we detected a significant cortical thinning in multiple extramotor areas, particularly in the frontotemporal regions. These findings confirm previous neuroimaging studies in ALS, 6,11,[13][14][15]19,20 where the spatial distribution of neurodegeneration closely resembled patterns identified in FTD variants. 42 Furthermore, the widespread cortical damage found in our ALS population overlaps ex vivo findings related to the expression of a TDP-43 pathologic feature.…”

Section: Discussionsupporting

confidence: 81%

“…[6][7][8][9][10] In vivo, independent of the structural neuroimaging approach used (VBM or SBM), the most commonly affected extramotor cortical areas were identified in the frontal and temporal lobes. [11][12][13][14][15][16][17][18][19][20][26][27][28] However, significant correlations between cortical involvement and disease progression 18,26,27 or cognitive impairment 2,14,15,20,28 were reported only in a few studies.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4][5][6] Indeed, among patients with ALS, as many as 15% meet criteria for frontotemporal dementia (FTD), whereas up to 35% show a mild to moderate cognitive impairment. 5,7 From the histochemical and genetic points of view, recent findings suggest that ALS may belong to a broader clinicopathologic spectrum, known as transactivating responsive sequence DNAbinding protein 43-kDa (TDP-43) proteinopathy, which also includes FTD.…”

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confidence: 99%

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Frontotemporal Cortical Thinning in Amyotrophic Lateral Sclerosis

d’Ambrosio

Gallo

Trojsi

et al. 2013

American Journal of Neuroradiology

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BACKGROUND AND PURPOSE:The extensive application of advanced MR imaging techniques has undoubtedly improved our knowledge of the pathophysiology of amyotrophic lateral sclerosis. Nevertheless, the precise extent of neurodegeneration throughout the central nervous system is not fully understood. In the present study, we assessed the spatial distribution of cortical damage in amyotrophic lateral sclerosis by using a cortical thickness measurement approach.

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Section: Discussionsupporting

confidence: 81%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Frontotemporal Cortical Thinning in Amyotrophic Lateral Sclerosis

d’Ambrosio

Gallo

Trojsi

et al. 2013

American Journal of Neuroradiology

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“…Similarly, neuroimaging studies have reported anatomical changes in frontal, temporal and limbic areas for ALS, FTD and ALS-FTD patients as well as decreased integrity of gray matter (GM) and white matter (WM) in the frontal, temporal and parietal lobes, including long association fibers. 8,9 On a pathological level, it is well established that TDP-43 is the main pathology found in most of ALS cases. [10][11][12][13] Interestingly, TDP-43 is also one of the main pathologies (~50%) of FTD, in particular in SD as it accounts for 75% of the cases.…”

Section: Introductionmentioning

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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“…There is evidence that emotional deficit follows selective damage in the ILF and IFOF (Philippi, Mehta et al 2009). The role of the corticostriatal pathways in neurodegeneration has recently been emphasized (Shepherd 2013), so the involvement of subcortical area may underlie the cognitive and behavioral changes in ALS (Tsermentseli, Leigh et al 2012).…”

Section: Overlap Between Als and Ftdmentioning

confidence: 99%

Measuring the progression of upper motor neuron disease using diffusion MRI and its correlation with Clinical phenotype

Alruwaili¹

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The anatomy of cognitive impairment in amyotrophic lateral sclerosis: More than frontal lobe dysfunction

Cited by 103 publications

References 130 publications

Frontotemporal Cortical Thinning in Amyotrophic Lateral Sclerosis

Frontotemporal Cortical Thinning in Amyotrophic Lateral Sclerosis

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

Measuring the progression of upper motor neuron disease using diffusion MRI and its correlation with Clinical phenotype

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