Neurofibromatosis Essay

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Introduction

According to Gutmann et al. (2017) neurofibromatosis is a group of three conditions whereby tumors grow in the nervous system. These conditions are neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and schwannomatosis. It is considered to be a genetic disorder of the nervous system. Neurofibromatosis mainly affects the development and growth of nerve cell tissue. The tumors can develop anywhere in the nervous systems including spinal cord, brain, and nerves. These tumors are mostly noncancerous, however, there have been instances when they do become cancerous. The most common condition is NF1. Schwannomatosis is the most recent and it is a rare type of neurofibromatosis. Little is known about schwannomatosis.

NF1 manifests at birth or in early childhood. It is characterized by multiple café-au-lait (light brown) spots that are concentrated in the groin and underarms (Gutmann et al., 2017). It is also manifested by benign tumors under the skin. Scoliosis may be present. NF2 might appear during childhood, adolescence, or in early adulthood. NF2 is portrayed by benign tumors of the nerves that will transmit sound impulses and balance signals from the inner ears to the brain. The tumors will mostly affect both the right and left auditory nerves.

Normal Anatomy of The Nervous System

The central nervous system (CNS) consists of the brain and spinal cord. The brain is charged with controlling most of the body functions including movements, awareness, thoughts, sensations, memory, and speech. The spinal cord is connected to the brain at the brainstem. It is covered by vertebrae of the spine. The nerves exit the spinal cord on to both sides of the body. The spinal cord is charged with carrying signals back and forth from between the nerves in the body and the brain (Anderson & Gutmann, 2015).

The part of the nervous system that is outside the CNS is referred to as the peripheral nervous systems (PNS). PNS is made up of nerves that send and receive signals from the CNS. It is divided into the autonomic nervous system and the somatic nervous system. The autonomic nervous system is charged with controlling involuntary functions that the body does by itself like breathing. The somatic nervous system controls body movements that are under the individual's control like walking.


Normal Physiology of The Nervous System

The nervous system is charged with receiving information regarding the environment and generating responses to the information received (Kruyt, Verheul, Hanssens, & Kunst, 2018). It is divided into regions that are responsible for sensation and for a response. Sensory functions are for sensation and motor functions are for a response. Sensation is involved in receiving information from the environment in order to gain input regarding what is happening outside the body. The sensory…

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…Relevance

It is vital that this disorder is well understood. With proper understanding, a cure and prevention for the disorder can be developed. Research is still ongoing on how best to prevent and treat the disorder. Considering the prevalence of the disorder, primary prevention should be encouraged. This will work towards reducing the number of cases being reported. People should be encouraged to get diagnosed or seek treatment if they have more than six café-au-lait spots. Even without treatment, it is possible to manage the disorder. Therefore, there is a need for education both for nursing staff and patients on how best to live with the disorder.

Conclusion

Neurofibromatosis is a genetic disorder that affects the nervous system. The disorder is genetically transmitted from a parent or through genetic mutation. The nervous system is charged with receiving signals from the environment and transmitting the signals to the brain for interpretation. once interpreted they are sent to the relevant body organ through the nerves for action. When there is a disruption in this system, signals will be received and transmission will fail since the nerves have tumors that prevent any signal from being sent or received. This results in the failure of particular bodily functions or senses like sight or movement. This disruption is not preventable and there is no cure for the disorder. However, there are ways for treating….....

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References

Anderson, J. L., & Gutmann, D. H. (2015). Neurofibromatosis type 1 Handbook of clinical neurology (Vol. 132, pp. 75-86): Elsevier.

de Blank, P. M., Fisher, M. J., Liu, G. T., Gutmann, D. H., Listernick, R., Ferner, R. E., & Avery, R. A. (2017). Optic Pathway Gliomas in Neurofibromatosis Type 1: An Update Surveillance, Treatment Indications, and Biomarkers of Vision. Journal of Neuro-Ophthalmology, 37, S23-S32.

Gutmann, D. H., Ferner, R. E., Listernick, R. H., Korf, B. R., Wolters, P. L., & Johnson, K. J. (2017). Neurofibromatosis type 1. Nature Reviews Disease Primers, 3, 17004.

Kruyt, I. J., Verheul, J. B., Hanssens, P. E., & Kunst, H. P. (2018). Gamma Knife radiosurgery for treatment of growing vestibular schwannomas in patients with neurofibromatosis Type 2: a matched cohort study with sporadic vestibular schwannomas. Journal of neurosurgery, 128(1), 49-59.

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