Brain responses to emotional stimuli in patients with amyotrophic lateral sclerosis (ALS)

Lulé, Dorothée; Diekmann, V.; Anders, Silke; Kassubek, Jan; Kübler, Andrea; Ludolph, Albert C.; Birbaumer, Niels

doi:10.1007/s00415-006-0409-3

Cited by 70 publications

(73 citation statements)

References 35 publications

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“…Papps et al [16] found a selective failure in memory for emotional material in ALS, while Lulé et al [17] found altered emotional responses to a picture test with respect to positive valence and decreased excitability for extremely emotional stimuli. In a more recent fMRI study, the same authors [18] found that ALS patients showed increased activation in the right supramarginal area and a reduced response in extrastriate visual areas when presented with emotional stimuli depicting social interaction and facial expression. Thus, compared to healthy controls, ALS patients showed enhanced (i.e.…”

Section: Introductionmentioning

confidence: 90%

Right hemisphere dysfunction and emotional processing in ALS: an fMRI study

et al. 2010

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Emotional processing may be abnormal in amyotrophic lateral sclerosis (ALS). Our aim was to explore functional anatomical correlates in the processing of aversive information in ALS patients. We examined the performance of nine non-demented ALS patients and 10 healthy controls on two functional MRI (fMRI) tasks, consisting of an emotional attribution task and a memory recognition task of unpleasant versus neutral stimuli. During the emotional decision task, subjects were asked to select one of three unpleasant or neutral words. During the memory task, subjects were asked to recognize words presented during the previous task. Controls showed, as expected, greater activation in the right middle frontal gyrus during selection of unpleasant than neutral words, and a greater activation mainly in right-sided cerebral areas during the emotional recognition task. Conversely, patients showed a general increase in activation of the left hemisphere, and reduced activation in right hemisphere in both emotional tasks. Such findings may suggest extra-motor neurodegeneration involving key circuits of emotions, mostly negative, commonly involved in FTD.

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Section: Introductionmentioning

confidence: 90%

Right hemisphere dysfunction and emotional processing in ALS: an fMRI study

et al. 2010

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“…94 Functional cortical changes have been observed in patients with ALS without cognitive/behavioral impairment during processing of socioemotional stimuli (ie, pictures of persons in emotional situations). 95 Compared with controls, patients with ALS showed an increased activation of the right supramarginal gyrus. 95 …”

Section: 93mentioning

confidence: 96%

“…95 Compared with controls, patients with ALS showed an increased activation of the right supramarginal gyrus. 95 …”

Section: 93mentioning

confidence: 96%

The Present and the Future of Neuroimaging in Amyotrophic Lateral Sclerosis

Agosta¹,

Chiò²,

Cosottini³

et al. 2010

AJNR Am J Neuroradiol

117

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SUMMARY:In patients with ALS, conventional MR imaging is frequently noninformative, and its use has been restricted to excluding other conditions that can mimic ALS. Conversely, the extensive application of modern MR imagingϪbased techniques to the study of ALS has undoubtedly improved our understanding of disease pathophysiology and is likely to have a role in the identification of potential biomarkers of disease progression. This review summarizes how new MR imaging technology is changing dramatically our understanding of the factors associated with ALS evolution and highlights the reasons why it should be used more extensively in studies of disease progression, including clinical trials.ABBREVIATIONS: ALS ϭ amyotrophic lateral sclerosis; ALSFRS ϭ ALS Functional Rating Scale; Cho ϭ choline; Cr ϭ creatine; CST ϭ corticospinal tract; DTI ϭ diffusion tensor imaging; FA ϭ fractional anisotropy; FLAIR ϭ fluid-attenuated inversion recovery; fMRI ϭ functional MR imaging; FTD ϭ frontotemporal dementia; FUS/TLS ϭ fused in sarcoma/translocated in liposarcoma gene; GM ϭ gray matter; 1 H-MR spectroscopy ϭ proton MR spectroscopy; L ϭ left; LMN ϭ lower motor neuron; MD ϭ mean diffusivity; mIns ϭ myo-inositol; MT ϭ magnetization transfer; MTR ϭ MT ratio; NAA ϭ N-acetylaspartate; ns ϭ not significant; PD ϭ proton density; R ϭ right; SOD1 ϭ superoxide dismutase 1; SPM ϭ statistical parametric mapping; TDP-43 ϭ TAR DNA-binding protein gene; UMN ϭ upper motor neuron; VBM ϭ voxel-based morphometry; WM ϭ white matter A LS, also known as motor neuron disease, is a neurodegenerative disorder characterized by a progressive muscular paralysis reflecting degeneration of motor neurons in the primary motor cortex, brain stem, and spinal cord. The phenotypic expression of ALS is highly heterogeneous and determined by 4 elements: 1) body region of onset, 2) relative mix of UMN and LMN involvement, 3) rate of progression, and 4) cognitive impairment.1 In approximately 5%-10% of patients, the disease is inherited; 20% of these individuals have a mutation of the SOD1 gene; approximately 2%-5%, of the TARDBP (TDP-43) gene; and 2%-4%, of the FUS/TLS gene. 2Most patients with ALS, however, have no obvious family history and have sporadic ALS.2 To date, the only specific marker of sporadic ALS is the presence of inclusions staining positively for ubiquitin and TDP-43 in degenerating motor neurons. 3Despite technical advances in medicine in the last century, the diagnosis of sporadic ALS relies on the interpretation of clinical symptoms and signs (ie, signs suggestive of combined UMN and LMN degeneration, together with disease progression compatible with a neurodegenerative disorder). Paraclinical and laboratory tests are used only to exclude "ALS-mimic" syndromes. 4,5 In ALS, the absence of a disease marker for UMN and LMN involvement has 2 main negative consequences. First, the delay from onset of the disease to diagnosis of ALS can vary between 13 and 18 months 6,7

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“…Un estudio con RM funcional demostró una mayor activación en el giro supramarginal derecho en pacientes con una sensibilidad alterada a claves sociales y emocionales 83,84 . También se han reportado déficits en la Teoría de la Mente y detección del sarcasmo 77,85,86 .…”

Section: Procesamiento Emocional Y Cognición Socialunclassified