Official websites use. Share sensitive information only on official, secure websites. Email: philip. Two systematic reviewers from Cochrane Response supported the guideline panel. The working group identified a total of 26 research questions, performed systematic reviews, assessed the quality of the available evidence, and made specific recommendations. Expert consensus statements were provided where insufficient evidence was available. The available evidence was scarce for many research questions.
Of the 26 research questions evaluated, the NICE recommendations could be adapted for 8 questions. Other recommendations required updates of existing systematic reviews or de novo reviews. The treatment landscape is changing rapidly, and further updates will be prepared when additional evidence becomes available.
The first European guideline on the diagnosis and management of amyotrophic lateral sclerosis ALS was published by a task force of the European Federation of Neurological Societies EFNS in and later updated in In ALS, the progressive loss of motor neurons in the motor cortex, brainstem, and spinal cord gives rise to progressive muscle weakness, stiffness and wasting, leading to a decline in motor function with a high impact on quality of life.
The panel therefore refers to separate publications for the diagnosis and management of cognitive and behavioural impairment in people with ALS. The cause of disease remains unknown in the majority of people with ALS. With the advent of molecular therapies for specific genetic subtypes of ALS there is a growing consensus to offer rapid gene testing to all people with ALS. Gene testing is not addressed in this guideline. For guidance on gene testing for ALS the panel refers readers to other publications.
In addition, recommendations are made on the delivery of multidisciplinary care, on the management of muscle weakness, muscle cramps, spasticity, sialorrhea, weak cough, thick mucus, respiratory insufficiency, malnutrition, emotional lability, anxiety, depression, insomnia, fatigue, deep venous thrombosis prevention, musculoskeletal pain, constipation, hoarse voice and stridor, and communication problems.
