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25 agosto 2014 1 25 /08 /agosto /2014 16:46

 

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

 

 

ALS is the most common of the five motor neuron diseases. Amyotrophic lateral sclerosis

http://www.nature.com/nrneurol/journal/v7/n11/fig_tab/nrneurol.2011.152_F1.html

 Image not available.

Creador del reto del cubo de agua helada muere al tirarse al mar desde edificio

El Ice Bucket Challenge o "Desafío del balde de agua fría" es una campaña publicitaria solidaria que busca generar conciencia poniéndonos en el lugar de las personas que padecen la rara Esclerosis Lateral Amiotrófica ELA (Amyotrophic Lateral Sclerosis ALS ), enfermedad degenerativa progresiva que ataca las neuronas motoras, haciendo que el cuerpo se sienta como si le tiraran una cubeta de agua fría, ya que el organismo baja mucho su temperatura.

 

ice_bucket_challenge_460_312

 

El Ice Bucket Challenge o también llamado Desafío del balde de agua fría (también puede ser agua con hielo), es una campaña publicitaria solidaria que busca más que nada generar un tipo de conciencia poniéndose en el lugar de las personas que padecen una enfermedad muy extraña llamada Esclerosis Lateral Amiotrófica (ELA, o ALS por sus siglas en inglés), enfermedad degenerativa progresiva que ataca las neuronas motoras, haciendo que el cuerpo se sienta como si le tiraran una cubeta de agua fría, ya que el organismo baja mucho su temperatura.1 2 Entre las personalidades relevantes que padecen esta enfermedad se encuentra por ejemplo el físico Stephen Hawking.

El dinero se recauda a beneficio como donación para la Asociación de Esclerosis Lateral Amiotrófica que estudia la enfermedad.

La ALS Association es una organización sin fines de lucro que tiene como principal fin la protección y cuidado y sobretodo asistencia a las personas que padecen esta enfermedad, a través de una interconectada red que tiene como sede principal Estados Unidos, además de un programa de investigación que está por todo el mundo con la labor de descubrir tratamientos y una posible cura para la enfermedad.

Múltiples personalidades se han sumado al desafío para apoyar la causa y generar su aporte. Entre algunos, se destacan Niall Horan,3 George W. Bush, Lionel Messi, Bill Gates, Taylor Swift, Neymar, Javier Hernandez Balcazar, Cristiano Ronaldo, Miguel Herrera, Migbelis Castellanos, Patricia Zavala, Lady Gaga, Shakira, Juanes, Demi Lovato, Fernando Gago, Ashley Benson , Isai Jocsan Cruz Ruiz de la colonia oracio son algunos de los que han aceptado el reto.4

 

152655 600 Obama Ice Bucket Challenge cartoons

A VER SI BAJA DE SU NUBE ...

Obama Ice Bucket Challenge

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MEJOR EL CUBO DE HIELO PA´DENTRO

Ice Bucket Challenge

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152724 600 Imbalanced Ice Bucket Challenge cartoons

http://www.cagle.com/news/ice-bucket-challenge/ 

 

El periodista Ayman al-Aloul y el comediante jordano Mohammed Darwaza crearon su propia versión del desafío del balde de agua fría: el desafío del balde de escombros.

Lo que buscan, más que donaciones económicas o materiales, es crear conciencia sobre la situación política de Gaza. Como declaran en uno de los videos en los que realizan el reto: “Intentamos usar un balde de agua, pero el uso de ésta es demasiado importante como para vertirla sobre nuestras cabezas (…) cuando miramos a nuestro alrededor, lo que vimos fueron escombros.” 
Gaza crea su propio desafío del balde de agua. El momento en el que el periodista Ayman Aloul es bañado en escombros, cumpliendo el desafío que inventó.
El periodista Ayman al-Aloul y el comediante jordano Mohammed Darwaza crearon su propia versión del desafío del balde de agua fría: el desafío del balde de escombros.
En el video se puede ver también al periodista excusándose por posiblemente aparecer sucio y empolvado frente a la cámara en el futuro: “Tal vez no encuentre agua para lavarme cuando llegue a casa.”

 

The changing scene of amyotrophic lateral sclerosis

 

The changing scene of amyotrophic lateral sclerosis

2013

http://www.nature.com/nrn/journal/v14/n4/fig_tab/nrn3430_F1.html

 

 

http://www.al.com/images/specialreport/huntsvilletimes/als_graphic1.jpg

 

 

 

Amyotrophic Lateral Sclerosis

In the U.S., more than 5,600 are diagnosed every year, and up to 30,000 are currently affected. ALS is responsible for 2 deaths per 100,000 every year.[1]

Median survival time from onset to death is 39 months, and only 4% survive longer than 10 years, although rare cases survive 50 years or more. Most die from respiratory failure, usually within three to five years from onset of symptoms.[2]

 

The earliest symptoms of ALS are typically obvious weakness and/or muscle atrophy. Other presenting symptoms include trouble swallowing, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. The parts of the body affected by early symptoms of ALS depend on which motor neurons in the body are damaged first. About 75% of people contracting the disorder experience "limb onset" ALS, i.e., first symptoms in the arms or legs. Patients with the leg onset form may experience awkwardness when walking or running or notice that they are tripping or stumbling, often with a "dropped foot" which drags gently along the ground. Arm-onset patients may experience difficulty with tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock. Occasionally, the symptoms remain confined to one limb for a long period of time or for the whole length of the illness; this is known as monomelic amyotrophy.

About 25% of cases are "bulbar onset" ALS. These patients first notice difficulty speaking clearly or swallowing. Speech may become slurred, nasal in character, or quieter. Other symptoms include difficulty swallowing and loss of tongue mobility. A smaller proportion of patients experience "respiratory onset" ALS, where the intercostal muscles that support breathing are affected first. A small proportion of patients may also present with what appears to be frontotemporal dementia, but later progresses to include more typical ALS symptoms.

Over time, patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski's sign also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations). Around 15–45% of patients experience pseudobulbar affect, a neurological disorder also known as "emotional lability", which consists of uncontrollable laughter, crying or smiling, attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of motor expressions of emotion.  To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.

 

PROGRESSION

Although the order and rate of symptoms varies from person to person, eventually most patients are not able to walk or use their hands and arms. They also lose the ability to speak and swallow food, while most end up on a portable ventilator, called a BiPAP. The rate of progression can be measured using an outcome measure called the "ALS Functional Rating Scale Revised (ALSFRS-R)", a 12-item instrument administered as a clinical interview or patient-reported questionnaire that produces a score between 48 (normal function) and 0 (severe disability). Though there is a high degree of variability and a small percentage of patients have much slower disorder, on average, patients lose about 0.9 FRS point per month. A survey-based study amongst clinicians showed that they rated a 20% change in the slope of the ALSFRS-R would be clinically meaningful.[7] Regardless of the part of the body first affected by the disorder, muscle weakness and atrophy spread to other parts of the body as the disorder progresses. In limb-onset ALS, symptoms usually spread from the affected limb to the opposite limb before affecting a new body region, whereas in bulbar-onset ALS symptoms typically spread to the arms before the legs.

Disorder progression tends to be slower in patients who are younger than 40 at onset,[8][9] are mildly obese,[10] have disorder restricted primarily to one limb, and those with primarily upper motor neuron symptoms.[11] Conversely, progression is faster and prognosis poorer in patients with bulbar-onset disorder, respiratory-onset disorder, and fronto-temporal dementia.[11]

CX3CR1 allelic variants have also been shown to modify the survival time and the progression of patients.[12]

 

 

LATE STAGES

Although respiratory support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms. The median survival time from onset to death is around 39 months, and only 4% survive longer than 10 years.[2] Guitarist Jason Becker has lived since 1989 with the disorder, while physicist Stephen Hawking has survived for more than 50 years, but they're considered unusual cases.[13]

Difficulty in chewing and swallowing makes eating very difficult and increases the risk of choking or of aspirating food into the lungs. In later stages of the disorder, aspiration pneumonia can develop, and maintaining a healthy weight can become a significant problem that may require the insertion of a feeding tube. As the diaphragm and intercostal muscles of the rib cage that support breathing weaken, measures of lung function such as vital capacity and inspiratory pressure diminish. In respiratory onset ALS, this may occur before significant limb weakness is apparent. External ventilation machines that use the ventilation mode of bilevel positive airway pressure (BiPAP) are frequently used to support breathing, initially at night, and later during the daytime as well. The use of BPAP (more often referred to as non-invasive ventilation, NIV) is only a temporary remedy, however, and it is recommended that long before BPAP stops being effective, patients should decide whether to have a tracheotomy and long term mechanical ventilation. At this point, some patients choose palliative hospice care. Most people with ALS die of respiratory failure or pneumonia.

In late stages the oculomotor nerve that controls the movements of the eye, can be affected as can the extraocular muscles. The eye movements remain unaffected largely until the later stages due to differences in the extraocular muscles compared to the skeletal muscles that are initially and readily affected.

Finally, patient condition may be mimicking locked-in syndrome.[14]

 

READ MORE

Amyotrophic lateral sclerosis - Wikipedia, the free ...

en.wikipedia.org/wiki/Amyotrophic_lateral_sclerosis

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OLA DE CALOR VERANIEGO EN GRINGOLANDIA

Heat Wave

 

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LA CAMPAÑA SOLIDARIA PARA INVESTIGAR LA ELA HA GATILLADO ÉMULOS Y LOCAS CAMPAÑAS

 

 


Graphic: What is ALS?
Dulcie Teesateskie/Huntsville Times

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