ABSTRACT
Amyotrophic lateral sclerosis (ALS) is the most common form of degenerative motor
neuron disease in adulthood. The clinical picture was accurately described by Charcot
over 125 years ago and consists of generalized fasciculations, progressive atrophy
and weakness of the skeletal muscles, spasticity and pyramidal tract signs, dysarthria,
dysphagia, and dyspnea. Pseudobulbar affect is common. Disease-specific treatment
options are still unsatisfactory. However, therapeutic nihilism is not justified as
a large array of palliative measures is available to enhance the quality of life of
patients and their families. Palliative care in ALS is a multidisciplinary effort
requiring careful coordination. An open and frank disclosure of the diagnosis is of
paramount importance. Nutritional deficiency due to pronounced dysphagia can be relieved
by a percutaneous endoscopic gastrostomy. Respiratory insufficiency can be effectively
treated by noninvasive home mechanical ventilation. The terminal phase of the disease
should be discussed, at the latest, when symptoms of dyspnea appear in order to prevent
unwarranted fears of ``choking to death.'' Collaboration with hospice and completion
of advance directives can be of invaluable help in the terminal phase.
KEYWORD
Amyotrophic lateral sclerosis - motor neuron disease - antiglutamatergic agents -
symptom control - noninvasive ventilation - palliative care
