Amyotrophic Lateral Sclerosis: The Most Common And Lethal Form Of Motor Neuron Disease-a Case Report From Middle East
DOI:
https://doi.org/10.51985/JBUMDC2018103Keywords:
Amyotrophic Lateral Sclerosis, Motor Neuron disease, Neurodegenerative disorder, MRIAbstract
A neurodegenerative disorder which is fatal, rapidly progressive and has no effective treatment till date is amyotrophic
lateral sclerosis. Almost 90% of all cases occur in the sporadic form, with the rest occurring in the familial form. It is a
devastating disease leading to death within 3-5 years in most cases. The diagnosis of AML is difficult to made in spite of
acknowledgment for 140 years. It is diagnosed by clinical presentation which is a combination of upper and lower motor
neuron signs and electro diagnostic studies which gives information about diffuse motor axonal injury. This neurodegenerative
disorder results in degeneration of corticospinal tracts and anterior horn cells and involving motor neurons of the cerebral
cortex, brainstem, and spinal cord. There are a variable signs and symptoms of this disease, so the diagnosis is very important
for the management and better outcome of the patients. Cause of death in these patients is usually respiratory failure.
References
R. Tortelli, M. Copetti, S. Arcuti et al., “Pseudobulbar affect (PBA) in an incident ALS cohort: results from the Apulia registry (SLAP),” Journal of Neurology, 2016;263(2):316-21.
A. Ludolph, V. Drory, O. Hardiman et al., “A revision of the El Escorial criteria-2015,” Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2015;16(5-6):291-292
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitusmoto H, Rosenfeld J, Shoesmith C, Strong MJ, Wooley SC: Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009, 73: 1218-2126.10.1212/WNL.0b013e3181 bc0141.
J. Dorst, A. C. Ludolph, and A. Huebers, “Disease-modifying and symptomatic treatment of amyotrophic lateral sclerosis,” Therapeutic Advances in Neurological Disorders, vol. 11, Article ID 175628561773473, 2018
Talbot K: Motor neurone disease. Postgrad Med J 2002; 78:513–519
Shoesmith CL, Findlater K, Rowe A, et al: Prognosis of amyotrophic lateral sclerosis with respiratory onset. J Neurol Neurosurg Psychiatry 2007; 78:629 –631
Brooks BR , Miller RG , Swash M , et al.: El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000; 5:293–299
Desport JC , Preux PM , Magy L , et al.: Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis. Am J Clin Nutr 2001; 74:328–334
Ludolph AC : 135th ENMC International Workshop: nutrition in amyotrophic lateral sclerosis. Presented March 18–20, 2005, Naarden, The Netherlands. Neuromuscul Disord 2006; 16:530–538
Bourke SC , Shaw PJ , Gibson GJ : Respiratory function vs. sleep-disordered breathing as predictors of QOL in ALS. Neurology 2001; 57:2040–2044
Mitsumoto H , Rabkin JG : Palliative care for patients with amyotrophic lateral sclerosis: “prepare for the worst and hope for the best.” JAMA 2007; 298:207–216
Naveed S, Waheed N, Ghayas S (2014) Awareness regarding blastocystosis disease; a Neglected Zoonotic disease MJPMS Mintage journal of Medical and pharmaceutical Sciences. 2014;3: 15-16.
Naveed S, Ghayas S, Was HKi, Azam M, Siddiqui M, et al. (2014) Awareness of Hepatitus B in Pharm D students: Survey based study (DHR-IJPS) 214;5: 98-104.
Naveed S, Knowledge and Attitude about Crimean Congo Hemorrhagic Fever (CCHF) Amongst Local Residents Of Karachi, Pakistan, Accepted J App Pharm 2014; 6: 166-170.
Naveed S, Syed R, Zafar A, Tariq T, Wasif N, et al. Prevalence Of Oral Submucous Fibrosis And Use of Tobacco And Related Products Mintage journal of Medical and pharmaceutical Sciences2014; 3: 4-6
Naveed S, Qamar F, Zainab S, Sarwer G. A Survey Study on awareness of Hepatitis C in different groups. World journal of pharmaceutical Sciences. 2014; 2: 449-454.
Naveed S, Hashmi F, Khan A, Salahuddin N. Awareness about Thalassemia: A survey Report MJPMS Mintage journal of Medical and pharmaceutical Sciences 2014;3: 18-19.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2019 Waseem Mehmood Nizamani, Ameet Jesrani, Mujtaba Khan, Kalthoum Tlili, Nader Al Khuraish, Kashaf Anwar Arain
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Journal of Bahria University Medical & Dental College is an open access journal and is licensed under CC BY-NC 4.0. which permits unrestricted non commercial use, distribution and reproduction in any medium, provided the original work is properly cited. To view a copy of this license, visit https://creativecommons.org/licenses/by-nc/4.0