Amyotrophic Lateral Sclerosis: A Predominant Form of Degenerative Disease of the Motor Neuron System

Amyotrophic Lateral Sclerosis: A Predominant Form of Degenerative Disease of the Motor Neuron System

Newman Osafo (Kwame Nkrumah University of Science and Technology, Ghana), David Darko Obiri (Kwame Nkrumah University of Science and Technology, Ghana), Oduro Kofi Yeboah (Kwame Nkrumah University of Science and Technology, Ghana) and Prince Amankwah Baffour Minkah (Kwame Nkrumah University of Science and Technology, Ghana)
DOI: 10.4018/978-1-5225-5282-6.ch015

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder (ND) that primarily comprises the neurons responsible for controlling voluntary muscle movement. The unique neuropathologic findings include anterior horn cell degeneration producing muscle atrophy or amyotrophy, degeneration, and sclerosis of the corticospinal tracts. It is a common neuromuscular disease worldwide and has been identified in people of all races. There seems to be neither identified risk factors nor family history associated with most of the documented ALS cases. There exists no treatment for ALS that can prevent neither its progression nor reverse its development. However, there are treatments available that can help control symptoms, prevent unnecessary complications, and make living with the disease easier. This chapter extensively discusses this neurodegenerative disorder based on the currently available knowledge on the condition.
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Background

ALS is a clinically and genetically heterogeneous, devastating, rapidly progressive neurodegenerative motor disorder with cognitive and behavioral impairments as core features (Schmidt et al, 2016). Alongside behavioral features, executive dysfunction is present in up to 50% of ALS patients (Beeldman et al, 2016). Anatomical connectivity studies have revealed clear white matter impairments, mostly affecting tracts directly linked to the motor cortex (Schmidt et al, 2014).

Improvements in our knowledge of the glutamate neurotransmitter system coupled with the detection of causal genes associated with the progression of familial ALS (fALS) have inspired research interest (Kiernan et al, 2011). Whereas these research findings allow for vital comprehensions regarding the ultimate consequences of ALS on the brain, the causal pathogenic mechanism of ALS remains largely unknown (Schmidt et al, 2016).

Cronin et al (2007) reported that there is a low incidence of ALS in people with mixed ancestral origin than in people of Spanish origin. About 5–10% of ALS is familial, with a Mendelian pattern of inheritance. The remaining 90% of the ALS population is identified as having sporadic disease for which results from family aggregation studies have realized a connection between ALS and common NDs, indicative of the presence of susceptibility genes that might elevate the overall risk of neurodegeneration among relatives (Fallis & Hardiman, 2009). As such, attempts to identify susceptibility genes have had little influence on the establishment of a complex genetic basis for sporadic ALS.

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