ALS

1. ALS Samuel Awad & Osama Jamali

2. Introduction • ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. One or two out of 100,000 people develop ALS each year. ALS most commonly strikes people between 40 and 60 years of age, but younger and older people can also develop the disease. Men are affected slightly more often than women. • What is ALS? • It is a debilitating disease with varied etiology characterized by rapidly progressive weakness, muscle atrophy and fasciculation's, muscle spasticity, difficulty speaking (dysarthria), difficulty swallowing (dysphagia), and difficulty breathing (dyspnea).

3. Signs & Symptoms • Initial symptoms • The earliest symptoms of ALS are typically obvious weakness and/or muscle atrophy. Other presenting symptoms include muscle fasciculation (twitching), 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 disease experience "limb onset" ALS, i.e., first symptoms in the arms or legs. • Late stage disease symptoms • Difficulty swallowing and chewing make eating normally very difficult and increase the risk of choking or aspirating food into the lungs. In later stages of the disease, aspiration pneumonia and maintaining a healthy weight can become a significant problem and may require insertion of a feeding tube. As the diaphragm and intercostal muscles (rib cage) that support breathing weaken, measures of lung function such as forced vital capacity and inspiratory pressure diminish. In respiratory onset ALS, this may occur before significant limb weakness is apparent. External machines such as bilevel positive pressure ventilation (frequently referred to by the tradenameBiPAP) are frequently used to support breathing, first at night, and later during the daytime as well

4. New Findings • Disease progression and spread • Although the order and rate of symptoms varies from person to person, eventually most patients are not able to walk, get out of bed on their own, or use their hands and arms. The rate of progression can be measured using an outcome measure called the "ALS Functional Rating Scale (Revised)", 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). • The debilitating symptoms of amyotrophic lateral sclerosis, or ALS, appear to be increased by a lack of inflammation-reducing T cells. The researchers found that expression of the gene FoxP3 -- which helps control the production of anti-inflammatory T cells -- was an indicator of disease progression in 80 percent of the patients they studied. Low FoxP3 levels were likely in patients whose ALS would develop rapidly, and vice versa. While inflammation exacerbates disease in ALS patients, this inflammation is suppressed in some patients, The data in the article suggests that regulatory T cells can suppress this inflammation.

5. Treatment • Riluzole is the only treatment that has been found to improve survival but only to a modest extent. It lengthens survival by several months, and may have a greater survival benefit for those with a bulbar onset. It also extends the time before a person needs ventilation support. • However pharmaceutical treatment alone is not enough as a broad spectrum of health professionals should be involved giving a range of treatments, such as: • Physical therapy • Speech Therapy • Food & nutrition Therapy

6. Bibliography • http://en.wikipedia.org/wiki/Amyotrophic_lateral_sclerosis • http://www.sciencedaily.com/releases/2012/12/121207161458.htm • http://neurosciencenews.com/