Good practice in the management of amyotrophic lateral sclerosis: Clinical guidelines. An evidence‐based review with good practice points. EALSC Working Group

Abstract

The evidence base for diagnosis and management of ALS is still weak, and curative therapy is lacking. Nonetheless, early diagnosis and symptomatic therapy can profoundly influence care and quality of life of the patient and relatives, and may increase survival time. This review addresses the current optimal clinical approach to ALS. The literature search is complete to December 2006. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. We conclude that a diagnosis of ALS can be achieved by early examination by an experienced neurologist. The patient should be informed of the diagnosis by the consultant. Following diagnosis, a multi-diciplinary care team should support the patient and relatives. Medication with riluzole should be initiated as early as possible. PEG is associated with improved nutrition and should be inserted early. The operation is hazardous in patients with VC <50%: RIG may be a better alternative. Non-invasive positive pressure ventilation improves survival and quality of life but is underused in Europe. Maintaining the patient's ability to communicate is essential. During the course of the disease, every effort should be made to maintain patient autonomy. Advance directives for palliative end of life care are important and should be discussed early with the patient and relatives if they so wish.

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

“…Patient acceptance is often better when the intervention is initially used as a supplement to oral caloric intake and not the sole source of nutrition. Patient and family education on the benefits of percutaneous gastrostomy often results in an improved sense of autonomy, confidence, and quality of life [115][116][117]. Patients and families relate that there is a sense of not being able to affect any significant change in disease course heightened by a fear of choking and the caregiver burden.…”

Amyotrophic Lateral Sclerosis Presenting as Chronic Cough

“…Despite its poor prognosis, an early diagnosis can save patients from unnecessary and expensive testing, lead to interventions that improve quality of life, and give patients and family time for advanced planning. 1 This case highlights an unusual presentation of ALS.…”

“…1 The majority of ALS cases (70-80%) present initially with asymmetric limb weakness whereas respiratory symptoms are present in about 5% of patients and bulbar symptoms (such as hoarse voice and laryngospasm) are present in only about 20%. 3 Given the rarity of ALS (incidence of 2:100,000 in western countries), patients tend to undergo treatment of more common conditions prior to diagnosis of ALS as seen in our case.…”

“…Continued healthcare at a multidisciplinary center for ALS, which our patient receives, has been shown to lead to better outcomes. 1 …”

Ethics and artificial nutrition towards the end of life

“…1 • In motor neuron disease (MND), loss of swallowing is variable; when it is lost, cognitive function is usually intact. 19 However, PEG insertion is associated with significant risk if forced vital capacity is below 0.5 litre. Early PEG insertion should be discussed with the patient before there is critical decline in respiratory function.…”