Free Essay - Charcot Marie Tooth Syndrome

Charcot-Marie Tooth Syndrome (CMT) disease refers to an inherited set of disorders that affects motor and sensory peripheral nerves causing sensory loss, atrophy, and muscle weakness. Peripheral nerves form part of the peripheral nervous system, which includes sensory and motor nerves that link the central nervous system to other parts of the body (Corrado et al., 2016). The condition causes a wide range of mutations in about 45 genes. The gene mutations result in axon abnormalities and demyelination neuropathies, which explain the different symptoms of CMT. This condition affects 1 in 2500 people in the US and affects more than 3 million people globally (Corrado et al., 2016). CMT is one of the most commonly inherited neurological disorders and has several phenotypes founded on inheritance and severity.

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Symptoms of CMT are evident in various sections of the body. The weaknesses of leg and foot muscles cause foot drop and multiple deformities, for instance, hammertoes and high arches Corrado et al., 2016). The condition also causes difficulties with small tasks that involve manual dexterity, such as manipulating buttons and writing due to muscular wasting in the hands (Ekins et al., 2015). In severe cases, which are seldom, CMT causes difficulties in performing voluntary muscle activity, which could render individuals unable to speak, swallow, or walk. Additionally, CMT leads to poor tolerance for hot, cool, and cold temperatures. Many people suffering from the condition have chronically cold feet and hands.

Causes/Origins of CMT

CMT is an inherited genetic condition that occurs in the event of mutations in the genes that affect the nerves of a human’s hands, arms, legs, and feet. Such variations either damage the nerves or destroy the myelin sheath, which forms a protective coating for the nerves. Consequently, such damages result in weaker communication between the brain and extreme regions such as feet and arms Corrado et al., 2016). Therefore, the senses and muscles are inadequately stimulated. Brain signals are not effectively transmitted, which accounts for the various symptoms. Generally, people whose families have a history of CMT are at higher risk of developing the disorder. Diseases such as diabetes, which are linked to neuropathies, may also cause symptoms resembling CMT.

Drugs Used to Treat CMT

Although there is no cure for CMT, several medicines can be used to alleviate the associated symptoms. These drugs include Vinca alkaloids, Taxols, and arsenic trioxides.

Vinca Alkaloids

Examples of Vinca alkaloids include vincristine, vinorelbine, and vinblastine. However, this type of drug is toxic and is associated with various side effects. Vincristine is linked with neurotoxicity and could result in several central nervous system effects (Moudi et al., 2013). Its intake may cause confusion, depression, agitation, insomnia, and depression. Besides, vinca alkaloids may lead to gastrointestinal toxicities which manifest themselves by constipation, bloating, and abdominal pains (Moudi et al., 2013). Other associated effects include vomiting and diarrhea. Another side effect is weakening one’s immunity hence making them more susceptible to illnesses. Moreover, these drugs rank among effective vesicants and could cause significant tissue damage (Moudi et al., 2013). Such side effects necessitate medical doctors’ guidance to determine the right threshold and subsequently minimize the potential adverse impacts.

Taxols

Examples of taxols include paclitaxel, docetaxel, cabazitaxel. Although they fall under high-risk drugs, they are recommended for some patients with CMT. Nonetheless, these drugs have several side effects. Taxols are irritants and could result in tissue damage; hence patients are required to take additional medication to regulate such reactions. The medicines may cause variations in blood pressure levels, which manifest by irregular heartbeats, headaches, and dizziness (Moudi et al., 2013). They could lead to temporary hair loss with healthy growth returning after ending the treatment. Taxols could cause temporary reductions in white and red blood cell count, which may place one at risk of anemia or infections (Moudi et al., 2013). Moreover, mild vomiting and nausea may occur after the drugs’ intake. Taxols are commended when doctors judge that the associated benefits will be higher than the side effects.

Arsenic Trioxides

An example of arsenic trioxide is trisenox. It is given via injection, and the dosage depends on factors such as one’s weight and response to treatment. Nevertheless, although the drug helps treat CMT, some potential side effects could result. Patients could have edema, which is characterized by the swelling of feet and hand due to the accumulation of fluids. A person is likely to feel tired and weak after the injection, and the feeling could prolong even after treatment (Ekins et al., 2015). Arsenic trioxides may cause skin changes, such as itching and rash. However, such changes are temporal and end once the treatment finishes. Additionally, the drugs could cause variations in potassium and magnesium levels in the blood, which could result in shortness of breath and chest pains (Corrado et al., 2016). As such, despite its helpfulness, patients have to bear with arsenic trioxide’s side effects when undergoing CMT treatment.

Overall, Charcot Marie Tooth Syndrome (CMT) disease is a hereditary condition whose symptoms are more pronounced in the legs, feet, arms, and hands. It affects the nervous system, rendering nerves unable to send signals to a human body’s extreme areas effectively. Although the condition has no cure, some drugs, including Vinca alkaloids, Taxols, and arsenic trioxides, can be used to manage it. Nonetheless, the medicines have various side effects, and doctors must evaluate a patient’s condition to determine the suitability and threshold of particular medications. There are no potential dental-related complications that may arise due to the nature of this disease.

References

Corrado, B., Ciardi, G., & Bargigli, C. (2016). Rehabilitation Management of the Charcot–Marie–Tooth Syndrome: A Systematic Review of the Literature. Medicine, 95(17). https://pubmed.ncbi.nlm.nih.gov/27124017/

Ekins, S., Litterman, N. K., Arnold, R. J., Burgess, R. W., Freundlich, J. S., Gray, S. J., ... & Pleasure, D. (2015). A brief review of recent Charcot-Marie-Tooth research and priorities. F1000Research, 4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4392824/

Moudi, M., Go, R., Yien, C. Y. S. & Nazre, M. (2013). Vinca alkaloids. International journal of preventive medicine, 4(11), 1231. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3883245/