Palliative care in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is the most common degenerative motor n euron disease in adults. The clinical picture consists of generalized fasci culations, progressive atrophy and weakness of the skeletal muscles, spasti city and pyramidal tract signs, dysarthria, dysphagia, and dyspnea. Pseudob ulbar affect is common. Diseasespecific treatment options are still unsatis factory Therapeutic nihilism is not justified as a large array of palliativ e measures available to enhance the quality of life of patients and their f amilies. Because of its clinical characteristics, ALS represents a paradigm for palliative care in neurological diseases. Numerous projects are being undertaken worldwide in an effort to enlarge the evidence base for palliati ve interventions in ALS. Palliative care in ALS is a multidisciplinary effo rt requiring careful coordination. An open and empathic disclosure of the d iagnosis is essential. Nutritional deficiency caused by dyshagia can be rel ieved by a percutaneous endoscopic gastrostomy. Respiratory insufficiency c an be effectively treated by non-invasive home mechanical ventilation. The terminal phase of the disease should be discussed at the latest when sympto ms of dyspnea appear, in order to prevent unwarranted fears of "choking to death." Psychological and spiritual care of patients and families are impor tant. Collaboration with hospice institutions and completion of advance dir ectives can be of invaluable help in the terminal phase.