Ocular fixation instabilities in motor neurone disease

Donaghy, Colette; Pinnock, Ralph; Abrahams, Sharon; Cardwell, Christopher; Hardiman, Orla; Patterson, Victor; McGivern, R. Canice; Gibson, J. M.

doi:10.1007/s00415-009-0109-x

Cited by 49 publications

(37 citation statements)

References 38 publications

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“…Activity of the FEF has been demonstrated during visual fixation, 23 and disturbance of visual fixation has been documented in ALS and associated with executive dysfunction. 31 In addition, an increase in the incidence of early saccades has been identified in several contexts in which executive function would be expected to be impaired, including frontal lobe lesions, 12,32 concussion, 33 and in ALS itself. 34 The recording of saccadic eye movements offers a rapid, accurate, and safe method of studying decision-making processes after minimal training, even in the context of significant cognitive or motor disability.…”

Section: Discussionmentioning

confidence: 98%

Saccadic abnormalities in frontotemporal dementia

et al. 2012

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Section: Discussionmentioning

confidence: 98%

Saccadic abnormalities in frontotemporal dementia

et al. 2012

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“…This deficit is present early in the disease course [30], is more pronounced in ALS patients with pseudobulbar palsy [28], and in some familial forms of the disease (non-SOD1 but is absent in SOD1) [35] and in those with progressive muscular atrophy [36]. This deficit has also been shown to correlate with eye-movement abnormalities (ocular fixation) a neurological marker [37]. Moreover, evidence of a prefrontal substrate to this cognitive profile has come from a range of brain imaging techniques further strengthening the view of an ALS-FTD spectrum [38].…”

Section: Amyotrophic Lateral Sclerosis To Frontotemporal Dementia: a mentioning

confidence: 89%

Social Cognition in Amyotrophic Lateral Sclerosis

Abrahams

2011

Neurodegen. Dis. Manage.

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There is an overlap between amyotrophic lateral sclerosis and frontotemporal dementia. Approximately 15% of amyotrophic lateral sclerosis patients suffer from frontotemporal dementia characterized by behavioral change while a further third experience subtle executive dysfunction (typically letter fluency deficits) and corresponding prefrontal changes. Behavior change appears prevalent with apathy being the most prominent feature. Reports of social and emotional cognition deficits are increasing. Deficits have been described on theory of mind tasks including interpretation of stories and cartoons, faux pas detection and in the judgment of preference based on direction of eye-gaze. Impairments in emotional face and prosody perception and emotional enhancement of memory have been reported, and decision making (with and without risk) appears affected. The role of executive dysfunction in this social cognition deficit remains unresolved and more direct evidence of oribitofrontal involvement has yet to be found. Implications for healthcare provision are discussed with deterioration of social interaction with carers predicted.There is a clinical overlap between amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), with 15% of ALS patients suffering from a full-blown FTD.A further third of ALS patients will have more subtle cognitive and behavioral change indicative of a 'subclinical FTD' syndrome.Executive dysfunction is a predominant symptom, in particular with letter fluency deficits.Behavioral change is prevalent with increased apathy and self-centeredness commonly reported.Underlying cognitive deficits can be found on tests of social cognition, theory of mind, emotional processing and decision making.Some ALS patients may have particular problems in understanding another person's perspective, or interpreting emotions and hence may appear egocentric and unresponsive in their viewpoint.It is predicted that some patients may experience a breakdown in social interaction and communication with carers.Clinicians should direct education strategies for those involved in the daily care of patients to shed light on this as an integral feature of the disease.

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“…More recently the neuropsychological profile of apparently nondemented MND patients has been characterised as a subclinical frontal lobe dysfunction [1, 3, 4, 13-15, 20, 24, 26]. Recent work by the authors formally examined ocular fixation for the first time in MND and found that saccadic intrusion amplitude correlated with tests sensitive to lesions of the frontal lobe [11]. These findings point to frontal lobe impairment as the basis of eye movement abnormality in MND.…”

Section: Introductionmentioning

confidence: 92%

“…The VFI employed letter fluency using 's' and 'c' letters. Further details of their use can be found in a previous publication [11].…”

Section: Neuropsychological Testsmentioning

confidence: 99%