Nutrition Management of Amyotrophic Lateral Sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease with high risk of malnutrition. Symptoms of dysphagia, depression, cognitive impairment, difficulty with self-feeding and meal preparation, hypermetabolism, anxiety, respiratory insufficiency, and fatigue with meals increase the risk of malnutrition. Malnutrition negatively affects prognosis and quality of life, making early and frequent nutrition assessment and intervention essential. Implementation of an adequate calorie diet, dietary texture modification, use of adaptive eating utensils, and placement of a feeding tube aid in preventing malnutrition. When nutrition status is compromised by dysphagia and weight loss (5%-10% of usual body weight) or body mass index <20 kg/m(2) without weight loss and when forced vital capacity is >50%, a percutaneous endoscopic gastrostomy placement is indicated. When forced vital capacity is <50%, a radiologically inserted gastrostomy is the preferred means of enteral placement due to lessened aspiration and respiratory risk. Parenteral nutrition (PN) is indicated only when enteral nutrition (EN) is contraindicated or impossible. This article reviews the background of ALS, nutrition implications and risk of malnutrition, treatment strategies to prevent malnutrition, the role of EN and PN, and feeding tube placement methods according to disease stage.

The impact of gastrostomy in motor neurone disease: challenges and benefits from a patient and carer perspective

“…Bolus feeding is a quick method for feeding,21 although the frequency of feeding using this approach was described as disruptive by many of the participants in this study. Patients and carers reported having to plan the day around feeding times, which restricted their ability to engage in social activities outside the home.…”

Development of a new scale for dysphagia in patients with progressive neuromuscular diseases: the Neuromuscular Disease Swallowing Status Scale (NdSSS)

“…Dysphagia is one of the most critical problems in patients with progressive neuromuscular diseases such as amyotrophic lateral sclerosis (ALS) [1] and Duchenne muscular dystrophy (DMD) [2]. Patients with swallowing disorders suffer not only from dehydration and malnutrition, but also from aspiration, which may result in pneumonia and can lead to death [3].…”

Enteral feeding in neurological disorders

“…Malnutrition is a very common problem in patients with neurological disorders, such as stroke,1 motor neurone disease,2 Parkinson's disease,3 Alzheimer's disease and other dementias 4. The cause of malnutrition in these patient groups is multifactorial, including suboptimal dietary intake (because of dysphagia, lack of appetite, reduced mobility, requirement for an unpalatable diet, reliance on others and physical difficulties in preparing and consuming food); increased metabolism; physical exertion and other comorbidities affecting metabolic status 5 6.…”