**1. Introduction**

594 Amyotrophic Lateral Sclerosis

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Amyotrophic lateral sclerosis (ALS) is characterized by progressive paralysis secondary to the impairment of the motor neurons, upper motor neuron and lower motor neuron. The most common symptoms and signs are atrophy and muscle weakness, fasciculations, cramps, hypertonia and hyperreflexia. In more advanced stages, decreased respiratory muscle strength, progressive loss of body weight and changes in food intake are observed (Nelson et al., 2000).

Several factors are inherent to the food intake in ALS, such as: lack of appetite, dysphagia, weakness, dyspnoea, and depression (Stanich et al., 2004; Nelson et al., 2000; Kasarskis et al., 1996; Welnetz, 1990; Slowie et al., 1983). ALS patients usually have rapid weight loss associated with reduced food intake, increased feeding time, fatigue, dehydration and depression (Wright et al., 2005). The combination of these factors may result in increased energy expenditure and therefore hypercatabolism.

Different studies confirm the correlation between the reduced Body Mass Index (BMI) and the decreased survival in subjects with ALS and the decreased food intake and decrease in tricipital skinfold (TSF) (Kasarskis et al., 1996; Desport et al., 2003; Desport et al., 1999; Desport et al., 2001; Heffernan et al., 2004; Slowie et al. 1983). They also show a high percentage of weight loss, greater than 15% (Mazzini et al. 1995), and prevalence of malnutrition.

Dysphagia, a common symptom with the disease progression, is a factor that makes difficult the maintenance of oral feeding, increasing the respiratory complications, with initiation of invasive ventilation, difficulty to move the patient for the outpatient care and depression (Mazzini et al., 1995).

In this context, taking the nutritiontal impairment experienced by ALS subjects into account, this chapter aims to discuss the key strategies of nutritional care of patients with ALS, with a tool for maximizing the nutritional status.
