ALS Information
ALS (amyotrophic lateral sclerosis) is a rare--yet terminal--neurological disease made famous by the immortal baseball player Lour Gehrig. ALS destroys the motor neurons that facilitate communication between the brain and the body's voluntary muscles. Its victims eventually lose movement in their legs, arms and torso.
Other People Are Reading
-
Significance
-
ALS affects approximately 5,000 Americans annually. Most of them are 40 to 60 years of age. The vast majority of its victims have no family history of ALS.
Symptoms
-
Early symptoms include muscular cramps and twitches, slurred speech and difficulty swallowing. As the disease advances, an arm or leg becomes noticeably weaker, or speech may be more difficult. Generalized muscle atrophy occurs later.
-
Diagnosis
-
ALS is diagnosed when a limb shows signs of both upper and lower motor neuron damage unrelated to other disorders. Hypersensitive reflexes indicate upper motor neuron involvement. Atrophy, cramps and twitching are signs of lower neuron damage.
Causes
-
While there's no established cause of ALS, 1993 NINDS research linked familial ALS to defects in the gene that produces the antioxidant SOD1. Inadequate SOD1 might allow accumulated free radicals to damage motor neurons.
Treatment
-
FDA-approved riluzole slows motor neuron damage by reducing the body's glutamate levels, which are higher in ALS patients. Other things that may improve the quality of life for sufferers include physical and speech therapy, respiratory aids and medications to control cramping.
-
References
- National Institute for Neurological Disorders and Stroke
- Wong PC, Pardo CA, Borchelt DR, Lee MK, Copeland NG, Jenkins NA, Sisodia SS, Cleveland DW, Price DL; An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria.; Neuron. 1995 Jun;14(6):1105-16
