A REVIEW OF CAUSES, SYMPTOMS, DIAGNOSIS, AND TREATMENT OF MOTOR NEURON DISEASE (MND)

Authors

  • Eftikhaar Hasan Kadhum Department of pharmaceutical science, College of Pharmacy, University of Thi-Qar, Thi-Qar, 64001, Iraq

Keywords:

MND, ALS, neurons, PMA

Abstract

Motor neuron disease (MND) also called amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative defect characterized by loss of upper motor neurons (UMNs) and lower motor neuron (LMNs, anterior horn cells of the spinal cord and brainstem nuclei). The term MND is widely synonymous with ALS, reflecting the observation that most patients reveal combined LMN-related loss of muscle because of denervation (amyotrophy), and UMN degeneration of the lateral corticospinal tract and its cortical origins establishing as gliosis, or hardening (sclerosis). The average survival of patients is 3–5 years. However, there is several causes of this disease includes: genetics, mitochondrial dysfunction, apoptosis, oxidative stress, protein aggregation, reduced axonal transport… etc.

The symptoms of MND are includes the following: dysphagia, bulbar featuresdysarthria, excessive salivation and tongue fasciculations, muscle wasting, limb or muscle features focal weakness, muscle twitching, sensory impairment and muscle stiffness; respiratory features orthopnoea, rapidity of breath on exertion, early morning headache and fatigue or tiredness, depression, cognitive features behavioural change, emotional lability…. etc. Riluzole is the only drug licensed to treat MND.

References

1. Bäumer D, Talbot K, Turner MR. Advances in motor neurone disease. Journal of the Royal Society of Medicine. 2014 Jan;107(1):14-21. DOI: 10.1177/0141076813511451.

2. Furuta N, Makioka K, Fujita Y, Okamoto K. Changes in the clinical features of amyotrophic lateral sclerosis in rural Japan. Internal Medicine. 2013;52(15):1691-6. DOI:

10.2169/internalmedicine.52.0195.

3. Hardiman O, Van Den Berg LH, Kiernan MC. Clinical diagnosis and management of amyotrophic lateral sclerosis. Nature reviews neurology. 2011 Nov;7(11):639-49.doi: 10.1038/nrneurol.2011.153.

4. Crone C, Krarup C. Peripheral Nerve Disorders: Chapter 6. Neurophysiological approach to disorders of peripheral nerve. Elsevier Inc. Chapters; 2013 Aug 17.

5. Huisman MH, de Jong SW, van Doormaal PT, Weinreich SS, Schelhaas HJ, van der Kooi AJ, de Visser M, Veldink JH, van den Berg LH. Population based epidemiology of amyotrophic lateral sclerosis using capture–recapture methodology. Journal of Neurology, Neurosurgery & Psychiatry. 2011 Oct 1;82(10):1165-70. doi: 10.1136/jnnp.2011.244939.

6. Logroscino G, Traynor BJ, Hardiman O, Chiò A, Mitchell D, Swingler RJ, Millul A, Benn E, Beghi E. Incidence of amyotrophic lateral sclerosis in Europe. Journal of Neurology, Neurosurgery & Psychiatry. 2010 Apr 1;81(4):385-90. doi: 10.1136/jnnp.2009.183525.

7. Kumar DR, Aslinia F, Yale SH, Mazza JJ. Jean-Martin Charcot: the father of neurology. Clinical medicine & research. 2011 Mar 1;9(1):46-9.

8. Miller RG, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane database of systematic reviews. 2012(3).doi: 10.1002/14651858.CD001447.

9. Raaphorst J. Cognition and behavior in motor neuron disease. Universiteit van Amsterdam [Host]; 2015. doi: 10.1097/01376517-200110000-00005.

10. Martin LJ, Price AC, Kaiser A, Shaikh AY, Liu Z. Mechanisms for neuronal degeneration in amyotrophic lateral sclerosis and in models of motor neuron death. International journal of molecular medicine. 2000 Jan 1;5(1):3-16.doi: 10.3892/ijmm.5.1.3.

11. Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. 2005 Aug 23;65(4):586-90. doi: 10.1212/01.

12. Chiò A, Calvo A, Moglia C, Mazzini L, Mora G. Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. Journal of Neurology, Neurosurgery & Psychiatry. 2011 Jul 1;82(7):740-6. doi: 10.1136/jnnp.

13. Wicks P, Abrahams S, Leigh PN, Williams T, Goldstein LH. Absence of cognitive, behavioral, or emotional dysfunction in progressive muscular atrophy. Neurology. 2006 Nov 14;67(9):1718-9.doi: 10.1212/01.wnl.0000242726.36625.f3.

14. Visser J, van den Berg-Vos RM, Franssen H, van den Berg LH, Wokke JH, de Jong JV, Holman R, de Haan RJ, de Visser M. Disease course and prognostic factors of progressive muscular atrophy. Archives of neurology. 2007 Apr 1;64(4):522-8.doi: 10.1001/archneur.64.4.522.

15. Kabashi E, Valdmanis PN, Dion P, Spiegelman D, McConkey BJ, Velde CV, Bouchard JP, Lacomblez L, Pochigaeva K, Salachas F, Pradat PF. TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nature genetics. 2008 May;40(5):572-4. doi: 10.1038/ng.132.

16. Ince P.G., Evans J., Knopp M., Forster G., Hamdalla H.H., Wharton S.B. and Shaw Ince PG, Evans J, Knopp M, Forster G, Hamdalla HH, Wharton SB, Shaw PJ. Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS. Neurology. 2003 Apr 22;60(8):1252-8. doi: 10.1212/01.wnl.0000058901.75728.4e.

17. Mateen FJ, Sorenson EJ, Daube JR. Strength, physical activity, and fasciculations in patients with ALS. Amyotrophic lateral sclerosis. 2008 Jan 1;9(2):120-1. doi: 10.1080/17482960701855864.

18. Krarup C. Lower motor neuron involvement examined by quantitative electromyography in amyotrophic lateral sclerosis. Clinical neurophysiology. 2011 Feb 1;122(2):414-22. doi:

10.1016/j.clinph.2010.06.027.

19. Shimizu T, Fujimaki Y, Nakatani-Enomoto S, Matsubara S, Watabe K, Rossini PM, Ugawa Y. Complex fasciculation potentials and survival in amyotrophic lateral sclerosis. Clinical Neurophysiology. 2014 May 1;125(5):1059-64. doi: 10.1016/j.clinph.2013.10.052.

20. Wijesekera LC, Nigel Leigh P. Amyotrophic lateral sclerosis. Orphanet journal of rare diseases. 2009 Dec;4:1-22.doi: 10.1186/1750-1172-4-3.

21. Shaw PJ. Molecular and cellular pathways of neurodegeneration in motor neurone disease. Journal of Neurology, Neurosurgery & Psychiatry. 2005 Aug 1;76(8):1046-57. doi: 10.1136/jnnp.2004.048652.

22. Yoshida M. Amyotrophic lateral sclerosis with dementia: the clinicopathological spectrum. Neuropathology. 2004 Mar;24(1):87-102. Doi: 10.1111/j.1440-1789.2003.00544.x.

23. Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC. Amyotrophic lateral sclerosis. The lancet. 2011 Mar 12; 377(9769):942-55.

doi: 10.1016/S0140-6736(10)61156-7.

24. McDermott CJ, Shaw PJ. Diagnosis and management of motor neurone disease. Bmj. 2008 Mar 20; 336(7645):658-62. doi: 10.1136/bmj.39493.511759.BE.

25. Bäumer D, Talbot K, Turner MR. Advances in motor neurone disease. Journal of the Royal Society of Medicine. 2014 Jan; 107(1):14-21.

26. Richards D, Morren J, Pioro E. Time to diagnosis and factors affecting diagnostic delay in amyotrophic lateral sclerosis. Exon Publications. 2021 Jul 25:15-33. doi:10.1016/j.jns.2020.117054.

27. Filippi M, Agosta F, Abrahams S, Fazekas F, Grosskreutz J, Kalra S, Kassubek J, Silani V, Turner MR, Masdeu JC. EFNS guidelines on the use of neuroimaging in the management of motor neuron diseases. European journal of neurology. 2010 Apr;17(4):526-e20. doi: 10.1111/j.1468-1331.2010.02951.

28. Kwan JY, Jeong SY, Van Gelderen P, Deng HX, Quezado MM, Danielian LE, Butman JA, Chen L, Bayat E, Russell J, Siddique T. Iron accumulation in deep cortical layers accounts for MRI signal abnormalities in ALS: correlating 7 tesla MRI and pathology. PloS one. 2012 Apr 17;7(4):e35241. doi: 10.1371/journal.pone.0035241.

29. Roeben B, Wilke C, Bender B, Ziemann U, Synofzik M. The motor band sign in ALS: presentations and frequencies in a consecutive series of ALS patients. Journal of the Neurological Sciences. 2019 Nov 15; 406:116440. Doi: 10.1016/j.jns.2019.116440.

30. Statland JM, Barohn RJ, McVey AL, Katz JS, Dimachkie MM. Patterns of weakness, classification of motor neuron disease, and clinical diagnosis of sporadic amyotrophic lateral sclerosis. Neurologic clinics. 2015 Nov 1; 33(4):735-48. -62.

Published

2024-09-03

How to Cite

Kadhum, E. H. (2024). A REVIEW OF CAUSES, SYMPTOMS, DIAGNOSIS, AND TREATMENT OF MOTOR NEURON DISEASE (MND). EUROPEAN JOURNAL OF MODERN MEDICINE AND PRACTICE, 4(9), 66–70. Retrieved from https://inovatus.es/index.php/ejmmp/article/view/3950

Similar Articles

You may also start an advanced similarity search for this article.