Lou Gehrig Disease is a term used in the United States that also refers to ALS or Amyotrophic Lateral Sclerosis. It was named after the famous Lou Gehrig, a baseball player for the New York Yankees between the 1920’s to the 1930’s. His career in baseball was cut short when he became afflicted with the disease ALS is a fatal and progressive disease which is characterized by the degeneration or deterioration of nerve cells within the brain, specifically those involved in motor functions that control the movements of voluntary muscles.
As it is a disease that involves the motor neurons, ALS can cause body and muscle weakness as well as deterioration or wasting away of the whole body due to the degeneration of motor neurons which cease to send impulses from the brain to various muscles of the body. As is the tendency of things that cease to serve their function, the body muscles gradually become weak due to inactivity and later on, begin to develop muscle twitching. An end result is muscle wastage and atrophy due to denervation so that an individual afflicted with the disease cease to have control of his own voluntary movements with the exception of the eyes.
In ALS, the individual usually retains their mental or cognitive functioning except in such cases when ALS is associated with fronto-temporal dementia. However, several studies have indicated that many individuals with ALS have slight changes cognitively in instances when neuropsychological testing is utilized. The nerves that transmit sensations as well as the autonomic nervous system is usually not affected. ALS affects all people with no preference for race or ethnicity and is regarded as one of the most common diseases that affect the brain and muscles worldwide.
Annually, 2 out of 100,000 individuals become afflicted with ALS (Ropper and Brown 2005). It commonly occurs in middle to older adulthood, with ages ranging from 40 to 60 years of age although it is not unlikely for other age groups to contract the disease and men have an increased risk for the disease as compared to women. The affected parts of the body which is usually affected by ALS depend on the motor nerve cells that damage the body. Two-thirds of ALS cases consist of individuals experiencing ALS that initially occurs in the limbs.
Individuals may notice a significant change in walking or running and may initially pass this off as clumsiness or awkwardness. Other people report onset of the disease through their arms and hands and report difficulty in going about simple tasks such as writing or buttoning a shirt. One-third of ALS cases, meanwhile, are bulbar at the beginning. Individuals may report difficulty in talking and speech may become slurred and incoherent. The first signs are commonly a decrease in the speech volume and an increased nasal tone.
Eventually, individuals experience dysphagia (Difficulty in swallowing) as well as decreased mobility of the tongue. This would eventually lead to the inability to talk and loss of protection of the airway during swallowing. The initial onset of the symptoms and the body parts which it initially affects does not really matter as this will eventually spread throughout the body during the progression of the disease. Aside from the above symptoms, this could also include spasticity of muscles, an exaggeration of reflexes and the occurrence of Babinski’s sign which normal for babies but abnormal for adults.
Furthermore, individuals may also experience labile emotions such that they may display uncontrolled bouts of laughter followed by instances of sadness or an increased frequency of mood swings due to the degeneration of bulbar motor nerve cells. There is no definitive test to diagnose ALS although many believe that the presence of motor nerve cells in a single limb may strongly indicate ALS. The diagnosis and confirmation that it is indeed ALS is commonly based on ruling out other diseases that may bring about similar symptoms produced by ALS that include Lyme disease, Syphilis, HIV as well as tick-borne Encephalitis (el Alaoui-Faris 1990).
In a research conducted by Passinetti et al (2006), it was found out that individuals with ALS have lower levels of three proteins that are found in the cerebro-spinal fluid as compared to individuals who do not have the disease. These proteins include the carboxyl-terminal fragment of neuroendocrine protein, Cystatin C, and TTR. As such, this method has provided a means to diagnose patients with ALS at a sooner time, thus allowing them to receive the necessary medical attention and provide an earlier relief of symptoms.
As of now, there is no drug or therapy that could completely cure ALS although Riluzole, the first drug for ALS has gained the approval from the Food and Drug Administration. This drug is said to decrease the release of glutamate within the body and such, is said to decrease nerve cell injury and damage. It also provides several effects that protects neurotransmitters through blocking and preventing calcium and sodium (Hubert et al 1994), the prevention of protein kinase C formation and antagonism of the N-methyl d-aspartate receptors (Beal et al 2005).
The patient’s survival is thereby lengthened and increases the time before ventilation support is needed References: Beal, MF et al. (2005). Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics. Cambridge: Cambridge University Press. el Alaoui-Faris, M et al (1990). Amyotrophic lateral sclerosis syndrome of syphilitic origin. 5 cases. Rev Neurol (Paris) 146 (1): 41–4. Hubert, JP et al (1994). Antagonism by riluzole of entry of calcium evoked by NMDA and veratridine in rate cultured granule cells: evidence for a dual mechanism of action.
Br. J. Pharmacol. 113 (1): 261–267 Phukan, et al (2007). Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol 6 (11): 994–1003. Ropper, AH and Brown, RH (2005). Syndrome of muscular weakness and wasting without sensory changes section of Degenerative diseases of the nervous system. In Adams and Victor’s Principles of Neurology, 8th ed. , pp. 938–949. New York: McGraw-Hill. Pasinetti, G et al (2006). Identification of potential CSF biomarkers in ALS. Neurology 66 (8): 1218–22.