The management of amyotrophic lateral sclerosis

Phukan, Julie; Hardiman, Orla

doi:10.1007/s00415-009-0142-9

Cited by 42 publications

(28 citation statements)

References 77 publications

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“…These results are in line with many reports of cognitive impairment as a frequent feature in degenerative diseases of the motor system [6,7]. There is increasing evidence that measurement of alterations in mesio-temporal lobe structures – especially in amygdalar, thalamic and hippocampal circuits – is a sensitive instrument for eliciting correlates of memory deficits [8,9,10,11,12,13,14], as these structures are a crucial part of the memory-related centers in the brain [15].…”

Section: Introductionsupporting

confidence: 81%

Amygdala Size Reduction Is Associated with Memory Deficits in Complicated Hereditary Spastic Paraparesis: An MRI Study

Pinkhardt

Sperfeld²,

Uttner³

et al. 2010

Eur Neurol

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Neuroimaging findings in hereditary spastic paraparesis (HSP), especially in complicated HSP (cHSP), are heterogenous. This study aimed to investigate possible in vivo morphological alterations of the whole brain and the amygdala in cHSP as a correlate of cognitive impairment in contrast to pure variants (pHSP). Amygdalar and whole brain volumes of 33 HSP patients (21 pHSP and 12 cHSP) were measured using three-dimensional magnetic resonance imaging (MRI) data sets by region of interest-based volumetry. A neuropsychological test battery was also performed. A significant reduction in whole brain volume compared with the controls, as well as significant correlations with reduced amygdalar volume and a worse neuropsychological test performance was observed only in cHSP patients. Our findings of disproportional amygdalar atrophy only in cHSP substantiate the association of morphologically assessable cerebral degeneration with cognitive impairment in cHSP.

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Section: Introductionsupporting

confidence: 81%

Amygdala Size Reduction Is Associated with Memory Deficits in Complicated Hereditary Spastic Paraparesis: An MRI Study

Pinkhardt

Sperfeld²,

Uttner³

et al. 2010

Eur Neurol

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“…The first symptoms of ALS are usually muscle weakness and increased dependency on others [12]. Psychosocial suffering in ALS therefore may not be restricted to the terminal phase.…”

Section: Discussionmentioning

confidence: 99%

Requests for euthanasia: origin of suffering in ALS, heart failure, and cancer patients

et al. 2010

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In The Netherlands, relatively more patients (20%) with amyotrophic lateral sclerosis (ALS) die due to euthanasia or physician-assisted suicide (EAS) compared with patients with cancer (5%) or heart failure (0.5%). We wanted to gain insight into the reasons for ALS patients requesting EAS and compare these with the reasons of cancer and heart failure patients. Knowing disease-specific reasons for requesting EAS may improve palliative care in these vulnerable patients. The data used in the present study were derived from the Support and Consultation in Euthanasia in The Netherlands (SCEN) evaluation study. This study provided consultation reports and questionnaires filled out by the attending physicians from 3,337 consultations conducted by SCEN physicians in situations where a patient requested EAS. For this study we selected data on all ALS patients (n = 51), all heart failure patients (n = 61), and a random sample of 73 cancer patients. The most frequently reported reasons for unbearable suffering were: fear of suffocation (45%) and dependency (29%) in ALS patients, pain (46%) and fatigue (28%) in cancer patients, and dyspnea (52%) and dependency (37%) in heart failure patients. Somatic complaints were reported more frequently as a reason for EAS by cancer patients [odds ratio (OR) 0.20, 95% confidence interval (CI) 0.09-0.46] and heart failure patients [OR 0.16, 95% CI 0.05-0.46] than by ALS patients. ALS patients should be helped in a timely fashion to cope with psychosocial symptoms, e.g., by informing them about the low risk of suffocation in the terminal phase and the possible means of preventing this.

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“…Diagnosis is based on careful clinical examination, appropriate neurophysiologic studies and frequent follow up [1]. The true incidence of ALS worldwide is unknown-there is evolving evidence that there may be variations in disease frequency across populations, with lowest rates in those of mixed ancestral origin [2,3].…”

Section: Introductionmentioning

confidence: 99%

Validating population-based registers for ALS: how accurate is death certification?

2010

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The purpose of this study was to determine the accuracy of death certification for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND) as recorded by the Central Statistics Office in the Republic of Ireland, and to examine its utility in capture-recapture analysis. The database of the Irish Central Statistics Office (CSO) was searched for death certificates of individuals over 15 years of age for whom ALS/MND (ICD9) was listed as a primary, secondary or tertiary cause of death from 2002 to 2006. This dataset was compared with mortality data from the Irish Register for ALS/MND for the same period. Three hundred ninety-eight cases with a diagnosis of ALS were identified through the CSO. The cause of death as identified by the CSO was 318 out of 398 (80%) cases known to the Register during the study period. ALS/MND was listed as a cause of death by the CSO in 79 (20%) cases that were unknown to the ALS/MND Register. Of these, ALS/MND was listed as a secondary/tertiary cause of death in 76, and 75% of all such deaths occurred in rural areas. Capture-recapture analysis using the CSO and ALS Register as independent datasets generated a likely over-estimation of the crude incidence of ALS from 2.1/100,000 to 3.45/100,000 in Ireland. Population-based registers are most accurate when ascertaining from a wide network of inter-dependent sources. Death certification for ALS/MND lacks both sensitivity and specificity. Capture-recapture exercises using such data sources are likely to over-estimate the true incidence of disease.

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The management of amyotrophic lateral sclerosis

Cited by 42 publications

References 77 publications

Amygdala Size Reduction Is Associated with Memory Deficits in Complicated Hereditary Spastic Paraparesis: An MRI Study

Amygdala Size Reduction Is Associated with Memory Deficits in Complicated Hereditary Spastic Paraparesis: An MRI Study

Requests for euthanasia: origin of suffering in ALS, heart failure, and cancer patients

Validating population-based registers for ALS: how accurate is death certification?

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