Deck 20: Amyotrophic Lateral Sclerosis

Question 1

Pseudobulbar affect,which is commonly seen in individuals with spastic bulbar palsy,refers to

A)deficits in speaking and swallowing due to weakness in bulbar muscles.
B)fluctuating pathological emotional control.
C)deficits that mimic bulbar weakness.
D)slowness of speech and decreased articulation.

Answer 1

B

Question 2

Which of the following is true with regard to exercise in patients with ALS?

A)When prescribing exercises,therapists must strive to attain a balance between promoting optimal use of intact muscles and preventing overuse fatigue.
B)Strengthening exercises are most effective in the weakest muscles,as these have the greatest room for improvement.
C)Exercise should be prescribed at moderate-to-high intensities; otherwise it is not effective for improving strength and endurance.
D)Exercise is contraindicated,regardless of intensity,because it causes overwork damage.

Answer 2

A

Question 3

Pain in patients with ALS

A)is typically a sharp and shooting pain as a result of demyelination.
B)typically occurs as a secondary or composite impairment as a result of immobility or malalignment.
C)is one of the first signs/symptoms of the disease.
D)is uncommon.

Answer 3

B

Question 4

Which of the following is true with regard to pharmacological management of ALS?

A)Drugs such as Copaxone,which are commonly used in the treatment of multiple sclerosis,are also effective in slowing the progression of ALS.
B)The glutamate inhibitor Riluzole (Rilutek)is approved for the treatment of ALS and may extend survival by several months.
C)Anticonvulsant medications such as Dilantin are often prescribed because risk of seizures increase as the disease progresses.
D)There are currently no medications available for the treatment of ALS.

Question 5

Which of the following is true with regard to prognosis in patients with ALS?

A)Older age at onset is a positive prognostic indicator; people diagnosed after age 55 have better 5-year survival rates.
B)Patients with familial ALS have a better prognosis that those with sporadic ALS.
C)Those with limb-onset ALS have a better prognosis than those with bulbar-onset ALS.
D)Disease-modifying medications,if taken early after diagnosis,can increase life expectancy by 10 years or more.

Question 6

Based on research on individuals with neuromuscular disease,exercise in patients with ALS

A)is contraindicated,as it may cause overuse weakness.
B)may improve the strength of muscles with a strength grade less than 3/5.
C)may improve the strength of muscles with a strength grade of at least 3/5.
D)should be utilized in any weak muscle regardless of the grade.

Question 7

Which of the following impairments is relatively rare in patients with ALS?

A)Sensory deficits
B)Contracture
C)Weakness/paralysis of the spinal muscles
D)Dysphagia

Question 8

Which is considered the cardinal sign of ALS?

A)Pain
B)Sensory loss
C)Ataxia
D)Muscle weakness

Question 9

Appropriate physical therapy interventions to manage respiratory muscle weakness in patient with ALS may include

A)supine positioning to optimize ventilation.
B)assisted coughing techniques and breathing exercises.
C)UE ergometry at 70%-85% target heart rate.
D)locomotor training using body weight support and a motorized treadmill.

Question 10

Amyotrophic lateral sclerosis is characterized by

A)upper motor neuron signs and symptoms.
B)lower motor neuron signs and symptoms.
C)both upper and lower motor neuron signs and symptoms.
D)peripheral nerve injury signs and symptoms.

Question 11

Which of the following is NOT true with regard to ALS?

A)In the majority of cases the disease is inherited.
B)Average age at onset is mid to late 50s.
C)Initial symptoms most often occur in the extremities.
D)It is a neurodegenerative disease.

Question 12

For controlling ankle and knee involvement,the most appropriate LE orthosis for patients with ALS is typically

A)a hip-knee-ankle-foot orthosis as patients have both trunk and LE involvement.
B)a knee-ankle-foot orthosis because of the extreme hyperextension that often occurs with LE weakness.
C)an ankle-foot orthosis because it allows some control of both the ankle and the knee,but it is still relatively lightweight.
D)a foot orthosis because excessive pronation often results in significant pain if not addressed.

Question 13

In patients with MS,which of the following is true with regard to the management of dysphagia?

A)For most patients with ALS,dysphagia is not an issue.
B)Initially,dysphagia can be managed by dietary modifications,patient education,and swallowing adaptations and strategies.
C)Most patients with ALS are started early on total parenteral nutrition through a central line to prevent malnutrition.
D)Even in patients with dysphagia,malnutrition and dehydration are not common.

Question 14

Which of the following mobility devices would be most appropriate to purchase for a patient with ALS?

A)A manual wheelchair with reclining back
B)A power wheelchair with tilt-in-space seating
C)A motorized scooter
D)A wheeled standing frame

Question 15

A patient with a diagnosis of ALS has sialorrhea.The patient will experience

A)excessive sweating.
B)excessive salivation and drooling.
C)ocular paralysis.
D)unexplained limb pain.

Question 16

The primary goal of medical intervention in patients with ALS is

A)to cure the disease and restore the patient to health.
B)to prevent progression of the disease and when possible reverse the pathological process.
C)to achieve the best quality of life for patients through palliative care.
D)to prepare the patient for inevitable early death.

Question 17

In patients with ALS,positive pressure noninvasive ventilation

A)is recommended once vital capacity falls below 50% of predicted values.
B)is recommended at diagnosis to prevent vital capacity from decreasing.
C)is not recommended,as supplemental oxygen is usually all that is needed.
D)is not recommended,as it cannot stop progression of disease and will extend the patient's survival only by a few months.

Question 18

Which of the following interventions is not appropriate for managing cervical muscle weakness in patients with ALS?

A)Cervical collars for provide external support
B)Reclining or tilt-in-space wheelchair with head support
C)Environmental/ergonomic changes such as elevating reading material
D)Halotraction

Question 19

The treatment of patients with early-stage ALS should include

A)restorative intervention.
B)preventive intervention.
C)compensatory intervention.
D)all of the above.