Atrophy and twitching of the tongue are common. There may be muscle tremors, spasms, twitching, or muscle atrophy (loss of tissue). 5Ī neurological examination indicates weakness, often beginning in one limb or in proximal groups (such as shoulders or hips). Patients may notice problems with lifting, climbing stairs and walking.
Involvement of the limbs is characterised by weakness, stiffness, cramping, loss of co-ordination and wasting of the muscles. Patients may report difficulty in swallowing, chewing, coughing, breathing and talking if there is involvement of the higher centres. While ALS is a slowly progressive disease, a precipitous/traumatic event may occur to bring the patient to the ED. When focal limb weakness occurs, ALS is distinguished from a specific nerve problem by the lack of pain or sensory symptoms. With a complete history, ALS can be differentiated from stroke or trauma due to the subacute or chronic progression of symptoms. 9Ĭomplications include aspiration pneumonia, loss of mobility (and sequelae such as pressure sores), and loss of independence. Although the usual course is relentlessly progressive with a 50% mortality rate within three years from onset, it can sometimes be protracted. Respiratory failure is the most common cause of death in patients with ALS, usually occurring within 3 to 5 years from the onset of symptoms. When muscles in the diaphragm and chest wall fail, patients are unable to breathe without ventilatory support. Localised muscle pain over several weeks may indicate the onset of weakness and atrophy pain may be severe in later stages of disease. This often begins as a foot drop or difficulty with fine hand movements. 70% of patients present initially with progressive and asymmetric limb weakness. The motor neurons in the anterior and lateral columns of the spinal cord degenerate and are replaced by fibrous astrocytes (gliosis).
ALS is characterised by atrophy of muscle fibres, which are denervated as their corresponding anterior horn cells degenerate.
Lou garrison disease free#
Suggested contributory factors include sulfur containing toxins, excess glutamate, free radicals, viruses (enteroviruses, retroviruses) and autoimmune factors 7 In these cases, there is an associated autosomal dominant mutation (chromosome 21q22.1)
5-10% of cases are inherited or familial.In the Western Pacific region (Guam, Papua New Guinea), there is an increased incidence that in many cases is associated with Parkinsonism and dementia. Men are affected more often than women, with a male to female ratio of 1.6:1. The most common age of onset is between 40 and 60 years of age, but younger and older people also can develop the disease. 8ĪLS occurs worldwide, without predilection for races and ethnic backgrounds. The prevalence of the disease in the United States is 20,000. In the US, the disease is often called Lou Gehrig disease after the baseball legend who died from ALS in 1941.ĪLS is the most common type of adult-onset motor neuron disease. Eventually, all voluntary movement is lost and the muscles become paralysed.Ĭharcot was the first to clinically describe this condition in the 1860s, and as a result, the disease was named after him. This results in gradual weakness, atrophy and fasciculations in the muscles that lose functioning ability. When these neurons degenerate or die, they cease to send messages to muscles. In ALS, both upper and lower motor neurons are affected. The lower motor neurons relay these messages to the specific muscles that then carry out the movement. Movement occurs when upper motor neurons send messages to neurons in the spinal cord (lower motor neurons). Amyotrophic lateral sclerosis (ALS) is a type of motor neuron disease that attacks the neurons responsible for controlling voluntary muscles.
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