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Ubiquitinated inclusions are a constant feature of amyotrophic lateral sclerosis (ALS). It has been hypothesised that these inclusions reflect overload or failure of the ubiquitin‐proteasome system, and that this failure contributes to the degeneration of motor neurons. In the present study we...
INTRODUCTION: Noninvasive positive pressure ventilation (NIPPV) is associated with improved survival in amyotrophic lateral sclerosis (ALS) and has been widely recommended. The extent of NIPPV use in ALS patients and the factors associated with its use have not been studied. METHODS: A...
Literature findings on astrogliosis of the spinal cord and of the cortex of sporadic (SALS) and familial (FALS) cases of amyotrophic lateral sclerosis (ALS) and of SOD1 transgenic mice are analysed and compared with those of 50 autopsied personal cases of ALS. In the spinal cord, astrogliosis is...
OBJECTIVE: To identify and compare the primary existential, physical, and psychosocial stressors affecting patients with ALS and their caregivers. Health care providers, together with patients and their caregivers, are challenged to identify both physical and psychosocial concerns that have the...
OBJECTIVES: The study aimed to evaluate cognitive function and emotional reactivity in 18 patients with ALS, compared to19 matched controls, and assess their evolution over a 12‐month period. METHODS: 18 ALS patients and 19 matched controls were included, and assessed at inclusion, six months...
Motor fatigue, during 30 seconds of maximum voluntary isometric contraction (MVIC) was simultaneously evaluated by the decline in mechanical force output, and from the compression in the power spectrum obtained from surface electromyogram (sEMG). Measurements were performed in patients diagnosed...
Effective treatment for amyotrophic lateral sclerosis (ALS) remains elusive. Two of the primary hypotheses underlying motor neuron vulnerability are susceptibility to excitotoxicity and oxidative damage. There is rapidly emerging evidence that the cannabinoid receptor system has the potential to...
BACKGROUND: Non‐invasive ventilation (NIV) probably improves survival in ALS, but its effect on quality of life (QoL) is less clear. Most potentially useful QoL instruments have not been validated in this context. METHODS: We assessed the validity (multi‐trait multi‐method analysis),...
The care of people with MND/ALS can raise difficult ethical dilemmas. The ventilatory support of patients may involve difficult decisions and complex discussions, to allow patients and their families and carers to make truly autonomous decisions. A case is presented of a patient who asked for...
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