Als- Amyotrophic Lateral Sclerosis
Introduction
Amyotrophic Lateral Sclerosis (ALS) is a fast progressing, degenerative, neurological disease. The disease is marked by weakness, atrophy and paralysis of the body’s voluntary muscles as a result of motor neuron degeneration and death (Wijesekera & Leigh, 2009). ALS gets its name from Greek origin: Amyotrophic- “A” meaning without, “myo” meaning muscle, “trophic” meaning nourishment, literally translated this term means “without muscle nourishment”. “Lateral Sclerosis” refers to the hardening and scarring of the anterior and lateral corticospinal tracts as the disease progresses (Wijesekera & Leigh, 2009).
In the US, ALS is commonly referred to as “Lou Gehrig’s Disease” for the famous Yankees first baseman, who died from the disease in 1941. In the UK, ALS is known as “Motor Neuron Disease” (MND) and in France it is known as “Charcot’s Disease”, for the French doctor Jean-Marie Charcot who first wrote about the disease in 1869. (NINDS, 2003; MFMER 2010).
ALS occurs in 1-3 people per 100,000 worldwide. The onset of ALS generally occurs in people between 40 and 60 years of age, however it can develop at any age (NINDS, 2003). The disease shows a slight preference for men. However, in diagnoses after the age of seventy, the preference disappears. There are two forms of ALS:
“In 5% to 10% of people with ALS, the disease is inherited as an autosomal dominant trait and is referred to as familial amyotrophic lateral sclerosis. In 90% to 95% of people with ALS, there is no family history of the disease, and these people are said to have sporadic amyotrophic lateral sclerosis.” (Bello-Haas, Kloos & Mitsumoto, 1998, p. 1313).
The two forms of ALS will be discussed further in the Etiology section of this paper. ALS is incurable and fatal. The majority of people with ALS die within 3-5 years of their diagnosis, usually due to respiratory failure. Treatments are generally focused on relieving symptoms enough to improve a patient’s quality of
Amyotrophic Lateral Sclerosis (ALS) is a fast progressing, degenerative, neurological disease. The disease is marked by weakness, atrophy and paralysis of the body’s voluntary muscles as a result of motor neuron degeneration and death (Wijesekera & Leigh, 2009). ALS gets its name from Greek origin: Amyotrophic- “A” meaning without, “myo” meaning muscle, “trophic” meaning nourishment, literally translated this term means “without muscle nourishment”. “Lateral Sclerosis” refers to the hardening and scarring of the anterior and lateral corticospinal tracts as the disease progresses (Wijesekera & Leigh, 2009).
In the US, ALS is commonly referred to as “Lou Gehrig’s Disease” for the famous Yankees first baseman, who died from the disease in 1941. In the UK, ALS is known as “Motor Neuron Disease” (MND) and in France it is known as “Charcot’s Disease”, for the French doctor Jean-Marie Charcot who first wrote about the disease in 1869. (NINDS, 2003; MFMER 2010).
ALS occurs in 1-3 people per 100,000 worldwide. The onset of ALS generally occurs in people between 40 and 60 years of age, however it can develop at any age (NINDS, 2003). The disease shows a slight preference for men. However, in diagnoses after the age of seventy, the preference disappears. There are two forms of ALS:
“In 5% to 10% of people with ALS, the disease is inherited as an autosomal dominant trait and is referred to as familial amyotrophic lateral sclerosis. In 90% to 95% of people with ALS, there is no family history of the disease, and these people are said to have sporadic amyotrophic lateral sclerosis.” (Bello-Haas, Kloos & Mitsumoto, 1998, p. 1313).
The two forms of ALS will be discussed further in the Etiology section of this paper. ALS is incurable and fatal. The majority of people with ALS die within 3-5 years of their diagnosis, usually due to respiratory failure. Treatments are generally focused on relieving symptoms enough to improve a patient’s quality of
References: Dal Bello-Haas, V, Kloos, AD, & Mitsumoto, H. (1998). Physical therapy for a patient through the six stages of amyotrophic lateral sclerosis. Physical Therapy, 78(12), 1312-1323. NINDS.(2003,April). NINDS amyotrophic lateral sclerosis fact sheet. Retrieved from http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis MFMER. (2010). Amyotrophic Lateral Sclerosis. Retrieved from http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/ Wijesekera, LC., & Leigh, PN. (2009). Review: Amyotrophic lateral sclerosis. Orphanet Journal of Rare Diseases, 4(3).