Question 1

Which of the following diagnostic evaluations is/are appropriate for children with neuromuscular dysfunction who exhibit progressive carbon dioxide retention,increasing muscle weakness,and advancing concerns regarding nighttime hypoventilation?

A)Spirometry studies
B)Complete pulmonary function studies
C)Home overnight oximetry
D)Arterial blood gas analysis and pulse oximetry

Accepted Answer

With further progression of carbon dioxide retention and muscle weakness and advancing concerns regarding nighttime hypoventilation,home overnight oximetry or polysomnography may be useful in determining the early need for assisted respiratory support.

Question 2

What are the names of the separate pathways in the spinal cord that support both voluntary and involuntary ventilation and transmit signals through descending pathways to motor neurons?&#10;A)Voluntary,reticulospinal;involuntary,corticospinal&#10;B)Voluntary,cervicothoracic;involuntary,reticulospinal&#10;C)Voluntary,corticospinal;involuntary,reticulospinal&#10;D)Voluntary,corticospinal;involuntary,cervicothoracic

Accepted Answer

Separate pathways in the spinal cord support both voluntary (corticospinal)and involuntary (reticulospinal)ventilation and transmit signals through descending pathways to motor neurons in the cervicothoracic portion of the spinal cord.These motor neurons transmit signals through peripheral nerves and across the neuromuscular junctions to the muscles of respiration.Dysfunction in any part of this control system,from brainstem to respiratory muscles,can result in respiratory failure.

Question 3

Which of the following glycogen storage diseases has the most severe respiratory symptoms?&#10;A)Pompe disease&#10;B)Diabetes&#10;C)Von Gierke&#10;D)Forbes-Cori disease

Accepted Answer

In terms of respiratory involvement,glycogen storage disease type II (also called Pompe disease or acid maltase deficiency)has the most severe symptoms.

Question 4

Which of the following neuromuscular diseases is characterized in early childhood by progressive muscle weakness,followed by skeletal and respiratory muscle weakness,and in adolescence by confinement to a wheelchair and requirement for ventilatory assistance?

A)Amyotrophic lateral sclerosis
B)Duchenne muscular dystrophy
C)Glycogen storage disease
D)Becker muscular dystrophy

Accepted Answer

The muscle weakness is progressive and eventually leads to profound skeletal and respiratory muscle weakness in all cases.By adolescence,all patients with DMD are wheelchair bound and require assistance with ventilation.

Question 5

Ondine's curse,or congenital central hypoventilation syndrome,occurs during which activity?&#10;A)Laughing&#10;B)Sleeping&#10;C)Eating&#10;D)Crying

Accepted Answer

One of the best examples of a CNS disorder that affects breathing is congenital central hypoventilation syndrome.Also known as Ondine's curse,this represents a condition involving hypoventilation associated with sleep.

Question 6

Which of the following muscles is considered a primary muscle of inspiration?&#10;A)Internal intercostal muscles&#10;B)Transversus abdominis&#10;C)Sternocleidomastoid muscles&#10;D)Diaphragm

Accepted Answer

The main inspiratory muscle is the diaphragm,which contributes almost three quarters of the inspiratory capacity and increases the vertical dimension of the thorax.Cervical nerves 3 to 5 contribute to form the phrenic nerve,which drives the diaphragm.Additional inspiratory force is provided by the external intercostal muscles,which contract to expand the rib cage during inspiration.The innervation of the intercostal muscles occurs via the intercostal nerves,which come off the thoracic spinal nerve roots.

Question 7

The therapist is asked to evaluate a young child with a diagnosis of Chiari malformation.Which of the following signs and symptoms should the therapist expect to see?
I)Irregular breathing
II)Difficulty swallowing
III)Vomiting
IV)Choking

A)II and IV only
B)III and IV only
C)I,II,and III only
D)I,II,and IV only

Accepted Answer

Younger children with Chiari malformations may present with difficulty swallowing,choking,irregular breathing patterns,or apnea.

Question 8

Which of the following diseases is characterized by chronic,slowly progressive muscle wasting and weakness,cataracts,heart conduction defects,and endocrine disorders?&#10;A)Glycogen storage disease&#10;B)Spinal muscular atrophy&#10;C)Myotonic dystrophy&#10;D)Becker muscular dystrophy

Accepted Answer

Myotonic dystrophy is a highly variable inherited disease characterized by chronic,slowly progressive muscle wasting and weakness,cataracts,heart conduction defects,and endocrine disorders.

Question 9

Which of the following therapeutic strategies should the therapist deem as the most important in patients with neuromuscular disease?&#10;A)Airway clearance techniques&#10;B)Oxygen therapy&#10;C)Ventilatory support&#10;D)Hydration

Accepted Answer

Children with neuromuscular weakness may have trouble with each phase of coughing.Inspiratory muscle weakness reduces vital capacity and maximal inhaled volume,bulbar muscle weakness can lead to impaired glottic closure,and expiratory muscle weakness reduces the maximal intrathoracic pressure and expulsive force.The significance of this cannot be overstated: in patients with neuromuscular disease,most episodes of acute respiratory failure result from the inability to eliminate airway secretions and mucus during otherwise benign chest infections.A peak cough flow less than 160 L/minute is associated with impaired secretion clearance,but early intervention at 250 to 270 L/minute is recommended for beginning cough assistance.

Question 10

Which of the following measurements is the main determinant of respiratory compromise in pediatric patients with neuromuscular disease?&#10;A)Forced vital capacity&#10;B)Forced expiratory volume in 1 second&#10;C)Forced inspiratory volume&#10;D)Flows during coughing

Accepted Answer

The use of pulmonary function tests (PFTs)in diagnosing and monitoring the progression of neuromuscular disease has been well established in adults,but pediatric testing remains a challenge.Although newer techniques for respiratory muscle strength testing are being introduced,the majority of school-age children and adolescents are best monitored by standard spirometry with maximal inspiratory and expiratory pressure monitoring.Additional testing includes assessment of cough flows because they are the main determinant of respiratory compromise.

Question 11

Which of the following forms of poliomyelitis affects the nerves in the cervical spine region that control diaphragmatic function?&#10;A)Spinal&#10;B)Bulbar&#10;C)Bulbospinal&#10;D)Bulbosacral

Accepted Answer

The answer of Which of the following forms of poliomyelitis...

Question 12

A therapist is administering incentive spirometry to a patient who demonstrates ptosis,diplopia,and a weak cough.The patient has a history of a minimal chronic respiratory disease.Which of the following disorders is likely affecting this patient?

A)Guillain-Barré syndrome
B)Becker muscular dystrophy
C)Duchenne muscular dystrophy
D)Myasthenia gravis

Accepted Answer

The answer of A therapist is administering incentive spirometry to...

Question 13

Which of the following diseases is characterized by demyelination of sheaths that coat peripheral nerves?&#10;A)Poliomyelitis&#10;B)Guillain-Barr&#233; syndrome&#10;C)Chiari malformations&#10;D)Infantile botulism

Accepted Answer

The answer of Which of the following diseases is characterized...

Question 14

What are the consequences of bulbar muscle dysfunction?
I)Dysphagia
II)Impaired coughing
III)Dysarthria
IV)Impaired inspiration

A)II and IV only
B)I and III only
C)I,II,and III only
D)II,III,and IV only

Accepted Answer

The answer of What are the consequences of bulbar muscle...

Question 15

The therapist is performing an airway clearance technique to a 5-month-old child with a diagnosis of aspiration pneumonia.The child has a very weak cough and,although he appears alert,he cannot move.Which of the following disorders of the motor nerve should the therapist suspect?

A)Polio
B)Myasthenia gravis
C)Spinal muscular atrophy
D)Guillian-Barré

Accepted Answer

The answer of The therapist is performing an airway clearance...

Deck 33: Neurologic and Neuromuscular Disorders