is more common in bulbar ALS. This condition causes
patients to have uncontrollable episodes of laughing
and crying that are inconsistent with the patients’
mood. A randomized controlled trial found that dextromethorphan–quinidine was beneficial in managing
these emotional symptoms. Other treatments include
tricyclic antidepressants and selective serotonin reuptake inhibitors.
Reactive clinical depression occurs in 9% to 11%
of patients with ALS. Once the ALS diagnosis is confirmed, patients should be counseled about their
prognosis; their spouses and family members should
also be offered counseling. Antidepressants such as
tricyclic antidepressants, selective serotonin reuptake
inhibitors, and serotonin-norepinephrine reuptake
inhibitors should be offered to all patients. These
drugs may help to elevate mood, stimulate appetite,
and improve sleep.
Respiratory Insufficiency and Falls
Respiratory insufficiency is one of the leading causes of
death among patients with ALS. Patients with this condition may have morning headaches, vivid dreams and
nightmares, frequent nocturnal arousals, fatigue, excessive daytime somnolence, and dyspnea on exertion.
The American Academy of Neurology recommends
that patients start noninvasive ventilation if their sniff
nasal pressure is less than 40 cm, their maximal inspiratory pressure is less than –60 cm, or their forced vital
capacity is less than 50%.
Bourke et al found that noninvasive ventilation
was associated with improvement in quality of life
and survival in patients with ALS. The median survival was increased by 205 days, and quality of life
was maintained above 75% of baseline on the sleep
apnea quality of life index score. In addition, patients who have a peak cough expiratory flow of
less than 270 L/min should be offered a mechanical in-exsufflator suction device. If the patient does
not tolerate noninvasive ventilation, then palliative medicine and hospice may be appropriate, said
Evidence suggests that 2% of patients with ALS die
from fall-related complications. Risk factors for falls
in ALS include muscle weakness, deficits in gait or
balance, and cognitive impairment. Assistive devices,
wheelchairs, and physical therapy can help prevent
falls. Some patients may need a brace to help stabilize
Research suggests that 10% of patients with ALS develop frontotemporal dementia. Nearly 50% of these patients demonstrate behavioral changes such as apathy,
disinhibition, and hostility. In addition, the incidence
of frontotemporal dementia is higher in patients with
familial ALS. The treatment of frontotemporal dementia remains symptomatic.
Observational studies have indicated that antidepressant medications, particularly serotonin reuptake
inhibitors like sertraline, paroxetine, and trazadone,
decrease disinhibition, anxiety, repetitive behaviors,
and impulsivity. Although antipsychotic medications
like quetiapine or olanzapine can limit agitation, they
must be used cautiously, as they can cause extrapyramidal side effects. In addition, behavioral modification
may also be helpful for problematic behavior. Finally,
these patients require close supervision at all times to
maintain their safety.
Dysphagia and Malnourishment
Between 16% and 55% of patients with ALS become
malnourished because of dysphagia. This condition
is characterized by difficulty with chewing and swallowing, nasal regurgitation, or coughing when drinking liquids. Nutritional status must be monitored every
three months in patients with ALS, said Dr. Weiss. In
addition, their BMI must be calculated and their weight
measured. Paganoni et al found that the risk of death
increased sevenfold in patients with ALS who had a BMI
less than 18. 5. Patients with ALS also need to be queried
about the severity of choking, duration of meals, and caloric intake. A speech therapist should evaluate patients
at every visit. Dietary changes might also be necessary
and may include thickening liquids and preparing food
that forms easily into a bolus.
As this disease progresses, patients may need a percutaneous gastrostomy (PEG) tube. According to the
American Academy of Neurology practice guidelines, it
is time to discuss PEG placement when a patient with
ALS starts to lose weight. When a patient’s forced vital
capacity is over 50%, the procedure can be done safely.
When it is less than 50%, the procedure entails an increased risk of complications, including death.
Managing Dysarthria and Hospice
More than 95% of patients with ALS lose their ability to speak before they die. This condition, known as