Amyotrophic Lateral Sclerosis Info & Sources (Anatomy Paper) [Again and Sorry]

17 Jul

Excuse me for posting this one again.  And no pictures even–what a gyp for you!  I’m sort of at a stopping point on this paper until I go to the campus library.  And who knows when that will be.  It seems something (tiredness, weather, holidays, other fun stuff to do, running, laziness, everything) is always stopping me.  But I am determined to get it finished BEFORE the semester begins.  I want to get some legit (physical) journals in their entirety and use those.  Then, once I have those sources–the easy part–writing the facts in a cohesive order.  It’s the works-cited page that I think is the most difficult.  That, and the in-text citations.  USING the information in the paper will be easy.  So this is more for me, then you, my readers.  If you’re totally bored and tired of my Anatomy class ALS paper’s sources–you can always check out my blog archives.  There is plenty of interesting material there. . .

ALS (9,620 results) {note to self:  type out full name to get more and better results}

bilateral degeneration of the upper motor neuron in the primary motor areas also impairs further adjusted motor areas, which leads to a strong reduction of ‘swallowing related’ cortical activation. While both hemispheres are affected by the degeneration a relatively stronger activation is seen in the right hemisphere.

Source 1:  http://www.springerlink.com/content/w7fd9x9b1a2v1ew1/

-Dysphagia severity tended to be particularly influenced by dysfunction of the posterior tongue.

-early stage of dysphagia in ALS was mainly caused by oral dysfunction, and the oral phase disorders began in some cases with a decreased function of bolus transport at the anterior part of the tongue, and in other cases with a deteriorated function of holding the bolus at the posterior part of the tongue.

Source 2:  http://www.ncbi.nlm.nih.gov/pubmed/11681400

-abnormal lingual movement may result in pharyngeal residue that is aspirated after the swallow is completed and respiration is resumed. ALS patients with bulbar involvement demonstrate more severe swallowing problems (such as aspiration)

Source 3:  http://jama.jamanetwork.com/article.aspx?volume=234&issue=7&page=715

-Until a definitive treatment is found for amyotrophic lateral sclerosis, there will continue to be a need to provide symptomatic care.

Using amyotrophic lateral sclerosis as the search term:

Source 4:  http://www.ajcn.org/content/63/1/130.short

-fatal neurodegenerative disease characterized by progressive muscle atrophy and weakness.

-Although dysphagia is a universal feature of this illness, the nutritional and metabolic status of ALS patients has received little attention.  Regression analysis demonstrated progressive decreases in body fat, lean body mass, muscle power, and nitrogen balance and an increase in resting energy expenditure as death approached.

-We conclude that ALS patients have a chronically deficient intake of energy and recommended augmentation of energy intake rather than the consumption of high-protein nutritional supplements.

Source 5:  http://www.nejm.org/doi/full/10.1056/NEJM200105313442207

–  no cut & paste allowed

-good overview/history

Source 6:  http://ukpmc.ac.uk/abstract/MED/11854102

Amyotrophic lateral sclerosis (ALS) is the most common degenerative motor neuron disease in adults.

-The clinical picture consists of generalized fasciculations, progressive atrophy and weakness of the skeletal muscles, spasticity and pyramidal tract signs, dysarthria, dysphagia, and dyspnea. Pseudobulbar affect is common.

Nutritional deficiency caused by dysphagia can be relieved by a percutaneous endoscopic gastrostomy. Respiratory insufficiency can be effectively treated by non-invasive home mechanical ventilation. The terminal phase of the disease should be discussed at the latest when symptoms of dyspnea appear, in order to prevent unwarranted fears of “choking to death.” Psychological and spiritual care of patients and families are important. Collaboration with hospice institutions and completion of advance directives can be of invaluable help in the terminal phase.

Source 7:  http://www.ojrd.com/content/4/1/3/abstract

-whole abstract is good info for entire overview of DZ

Source 8:  http://www.ncbi.nlm.nih.gov/pubmed/9562665

-Amyotrophic lateral sclerosis (ALS) is a motor neuron disease with evidence of both anterior horn cell and corticospinal tract degeneration. The incidence of ALS is 1 to 2.5 cases per 100,000 population and the disease occurs primarily in adult life. The etiology of sporadic ALS remains unknown, although 5 to 10% of cases are familial. The diagnosis of ALS requires the presence of both upper and lower motor neuron findings and progressive motor dysfunction. Several theories regarding the pathogenesis of ALS have emerged including glutamate excitotoxicity, free radical oxidative stress, neurofilament accumulation, and autoimmunity. Clinical trials involving antiglutamate agents, antioxidants, immunosuppressants, and growth factors have shown no substantial benefit in slowing progression, with death usually occurring 2 to 5 years following the onset of symptoms. The management of ALS patients requires a multidisciplinary team that can provide the numerous medical and physical interventions necessary to treat weakness and fatigue, bulbar dysfunction, spasticity and pain, depression, and respiratory failure.

Source 9:  http://ukpmc.ac.uk/abstract/MED/7952238

-Therapeutic trials for amyotrophic lateral sclerosis have attracted much attention, but no drug tested has been effective yet. Three major theories of pathogenesis form the basis for these trials: autoimmunity, chronic excitotoxic stimulation due to accumulation of glutamate, and, in the familial form, peroxidation due to subnormal activity of superoxide dismutase. In striking contrast to the negative results of all of the other drug trials, riluzole (a glutamate antagonist) was said to benefit patients with bulbar onset but not those with spinal onset. Problems with the original trial may be clarified by other studies now in progress.

Source 10:  http://www.sciencedirect.com/science/article/pii/S0140673696916803

-In an initial study, riluzole decreased mortality and slowed muscle-strength deterioration in ALS patients. We have carried out a double-blind, placebo-controlled, multicentre study to confirm those findings and to assess drug efficacy at different doses.

-959 patients with clinically probable or definite ALS of less than 5 years’ duration were randomly assigned treatment with placebo or 50 mg, 100 mg, or 200 mg riluzole daily; randomisation was stratified by centre and site of disease onset (bulbar or limb).

-primary outcome was survival without a tracheostomy. Secondary outcomes were rates of change in functional measures (muscle strength, functional status, respiratory function, patient’s assessments of fasciculation, cramps, stiffness, and tiredness).

-The most common adverse reactions were asthenia, dizziness, gastrointestinal disorders, and rises in liver enzyme activities; they were commonest with the 200 mg dose

-Overall, efficacy and safety results suggest that the 100 mg dose of riluzole has the best benefit-to-risk ratio. This study confirms that riluzole is well tolerated and lengthens survival of patients with ALS.

Source 11:  http://www.sciencedirect.com/science/article/pii/S0140673610611567

-Substantial wasting of the tongue muscles in bulbar-onset ALS. Note the absence of palatal elevation present on vocalisation. Difficulty with mouth opening and dysphagia might require supplementary feeding through a percutaneous endoscopic gastrostomy. In further support of a corticomotoneuronal hypothesis, the tongue is often disproportionately affected in comparison to other oropharyngeal musculature in patients with bulbar-onset ALS. As with the thenar muscles in the hand, the tongue receives more extensive cortical input than other muscle groups in the oropharyngeal area.

-neurodegeneration in ALS might result from a complex interaction of glutamate excitoxicity, generation of free radicals, cytoplasmic protein aggregates, SOD1 enzymes, combined with mitochondrial dysfunction, and disruption of axonal transport processes through accumulation of neurofilament intracellular aggregates. Mutations in TARDBP andFUS result in formation of intracellular aggregates, which are harmful to neurons. Activation of microglia results in secretion of proinflammatory cytokines, resulting in further toxicity. Ultimately, motor neuron degeneration occurs through activation of calcium-dependent enzymatic pathways

 

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