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Journal of Neurodegenerative Diseases - Volume 2014 2014, Article ID 435164, 10 pages -

Review ArticleInternal Medicine Department, University of Calabar, Calabar, Cross River State 540242, Nigeria

Received 20 June 2014; Revised 29 September 2014; Accepted 28 October 2014; Published 18 November 2014

Academic Editor: Anabela C. Pinto

Copyright © 2014 U. E. Williams et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Motor neuron disease is a neurodegenerative disease characterized by loss of upper motor neuron in the motor cortex and lower motor neurons in the brain stem and spinal cord. Death occurs 2–4 years after the onset of the disease. A complex interplay of cellular processes such as mitochondrial dysfunction, oxidative stress, excitotoxicity, and impaired axonal transport are proposed pathogenetic processes underlying neuronal cell loss. Currently evidence exists for the use of riluzole as a disease modifying drug; multidisciplinary team care approach to patient management; noninvasive ventilation for respiratory management; botulinum toxin B for sialorrhoea treatment; palliative care throughout the course of the disease; and Modafinil use for fatigue treatment. Further research is needed in management of dysphagia, bronchial secretion, pseudobulbar affect, spasticity, cramps, insomnia, cognitive impairment, and communication in motor neuron disease.

Autor: U. E. Williams, E. E. Philip-Ephraim, and S. K. Oparah



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