Identification of Existing Resources
Behavioral distress in ALS is common and presents a number of different
signs and symptoms, including depression and anxiety. With progression of motor
neuron loss, pseudobulbar affect occurs in a group of patients with ALS. It
presents as sudden onset of excessive laughing or crying, with no relevant
trigger. The Practice Parameter reviewed the current management recommendations
for patients with onset of these issues, which usually occur prior to the end
of life. The few reports on the prevalence and management of behavioral symptoms
at the end of life are reviewed in Appendix D.
Identification of Existing Gaps
Currently, there are no standardized algorithms available that instruct how
and when health care providers should screen for behavioral comorbidities (e.g.,
anxiety, depression) at the end of life in ALS. With progression of the disease,
testing for depression is more challenging and tools adapted for end-of-life
screening for depression are lacking.
The clinical benefits of treating depression and pseudobulbar affect are not
well studied in ALS. For example, the role of psychostimulants (methylphenidate)
for depression at the end stages of other diseases (cancer, HIV/AIDS) offer
therapeutic benefit; but, possible benefits of psychostimulants in ALS at the
end of life have not been studied. Additionally, as the disease progresses
and likelihood of depression and pseudobulbar affect (or other behavioral effects)
increases, there are no protocols for preparing the patient and family for
these changes that might occur.
Recommendations to the Field
Screen for signs and symptoms of depression and anxiety
in patients with ALS; offer treatment (e.g., SSRIs) and counseling for depression and anxiety, especially at the
end of life.
- Examine the epidemiology, cause and impact of depression during the
end of life.
- Investigate and standardize the optimal approach to screening
and diagnosing depression, anxiety and pseudobulbar affect in patients
during the end of life.
- Investigate effective therapies for treatment of depression,
anxiety and pseudobulbar affect during the end of life in patients with