Chapter 28 Nueromuscular disorders Adaptive quizzing

A nurse is providing care to parents whose infant has been diagnosed with spinal muscular atrophy (SMA) type 1. The parents ask the nurse to explain what this diagnosis means for their child long term. Which statement should the nurse include in the explanation? This is an autosomal dominant disorder that affects motor and cognitive development. The slow progression of the disorder will allow the infant to have a fairly normal childhood. Intense physical therapy can aide the infant in learning to sit and walk independently. Muscular wasting results in generalized immobility and difficulty feeding and breathing.

Muscular wasting results in generalized immobility and difficulty feeding and breathing. Explanation: SMA type 1 is the most severe form of spinal muscle atrophy that results in muscle wasting, generalized immobility and difficulty feeding. This is an autosomal recessive genetic disorder that affects motor but not cognitive development. SMA type 1 has a rapid progression; these infants do not usually live past 2 years of age.

The nurse is performing an assessment of the head of a 1-month-old infant as part of the child's neurologic assessment. Which finding requires further follow up by the nurse? Child and parents' head sizes are of a similar percentile. The posterior fontanel has closed. No membranous tissue is present between parietal and frontal bones. Child's head size was within same percentile 1 month ago.

No membranous tissue is present between parietal and frontal bones. Explanation: No membranous tissue present between the parietal and frontal bones indicates closure of the anterior fontanel, which requires further follow up by the nurse, as this may indicate the presence of craniosynostosis.

The nurse is assessing the vestibulocochlear nerve function of an infant. How will the nurse proceed with the assessment? Check hearing by rubbing fingers together near the infant's ears. Observe the infant's ability to startle to loud noises. Observe the strength and quality of the infant's cry. Evaluate for the presence of the gag reflex.

Observe the infant's ability to startle to loud noises. Explanation: The nurse will observe the infant's ability to startle to loud noises as part of assessing the vestibulocochlear nerve function of an infant.

The nurse is assessing the spine of an infant. Which finding requires further follow up by the nurse? no lateral curvature mild curvature from anterior to posterior skin is free of dimples. tuft of hair present on the lower back

tuft of hair present on the lower back Explanation: A tuft of hair on the lower back is an abnormal finding that requires further follow up by the nurse, because this finding may indicate the presence of occult spina bifida

The nurse is preparing to perform a neurologic assessment on a 2-year-old child at a well-child visit. How will the nurse begin the assessment? with the child seated at rest on the examination table while the child is in the caregiver's lap offering a variety of toys of the child to play with by picking up the child from the caregiver's lap

while the child is in the caregiver's lap Explanation: The nurse will perform the assessment while the child is in the caregiver's lap, because young children are often more comfortable in this setting. Wh

Question 10 of 10 The nurse is assessing an infant with spina bifida for hydrocephalus. Which finding(s) requires further follow up by the nurse? Select all that apply. vomiting awake and alert sunset eyes widening sutures on the head flat fontanels

widening sutures on the head sunset eyes vomiting Explanation: The findings of widening sutures on the head, the border of the pupil covered by the lower eyelid (sunset eyes), and vomiting are all signs of hydrocephalus and require further follow up by the nurse.

A pediatric nurse is providing care to several children. The nurse is reviewing the assessment findings for each of the children. Which finding requires the nurse to intervene? 3-year-old preschool-aged child who goes up stairs on hands and knees 9-month-old infant who can pull self up to a standing position 24-month-old toddler who engages in parallel play 14-month-old toddler who walks with a parent's assistance

3-year-old preschool-aged child who goes up stairs on hands and knees

The nurse is reviewing the history of a 3-year-old child diagnosed with cerebral palsy as an infant. Which factor from the child's history would the nurse identify as placing this child at risk for this condition? Birth weight 7 lb 2 oz (59.2 g) Paternal history of seizure disorder Birth at 26 weeks' gestation Singleton birth

Birth at 26 weeks' gestation Explanation: The child was premature at birth, which is a risk factor for cerebral palsy. Other factors that increase the risk for cerebral palsy include low birth weight, multiple births, and maternal history of seizure disorder.

The nurse is providing education for the parents of a child with muscular dystrophy about nutrition. Which statement by the parent requires further follow up by the nurse? "A feeding tube may be required later in my child's care." "Swallowing and nutrient absorption may become impaired." "Long-term use of glucocorticoids may increase hunger." "Becoming overweight is not a concern; I should encourage high-calorie foods whenever possible."

"Becoming overweight is not a concern; I should encourage high-calorie foods whenever possible."

The nurse is assessing a 3-year-old child at a clinic visit. On reviewing the child's history, the nurse notes that a small pigmented lesion of the lower spine was identified during infancy. Which question would the nurse likely ask the parents? "Does your child's head seem to be getting bigger?" "Is your child having any trouble with urinating or bowel movements?" "Have you noticed any joint problems in your child?" "Have you seen any jerk-like leg movements?"

"Is your child having any trouble with urinating or bowel movements?" Explanation: On physical exam, an infant with a closed neural tube defect (NTD) may have a dimple or pit, patch of hair, or a pigmented lesion anywhere along the spine, but most commonly in the lower spine. Signs and symptoms suggestive of a closed NTD include leg weakness, muscular atrophy of the legs, and bowel or bladder difficulties. Therefore, the question about urinating and bowel movements would be appropriate

A 10-year-old child is brought to the emergency department by the parents. Based on the documented findings above, the nurse suspects Guillain-Barré syndrome which is later confirmed by diagnostic testing. When developing the child's plan of care, which treatment would the nurse anticipate as the priority? Institution of plasmapheresis Administration of intravenous immune globulin (IVIG) Intensive physical therapy measures Use of opioids for pain management

Administration of intravenous immune globulin (IVIG) Explanation: Therapeutic interventions for Guillain-Barré syndrome include treatment with immune globulin, plasmapheresis, pain management, and physical therapy. Treatment with intravenous immune globulin (IVIG) is the preferred clinical therapy for Guillain-Barré syndrome. If IVIG is ineffective or the child cannot receive IVIG, plasmapheresis is an alternative treatment.

The nurse receives a report on a child admitted with severe muscular dystrophy. The nurse suspects the child has been diagnosed with the most severe form of the disease, known as: Duchenne. limb-girdle. facioscapulohumeral. myotonia.

Duchenne. Explanation: Studies have shown that Duchenne is the most severe form of muscular dystrophy. Myotonia isn't a form of the disease; it's a symptom.

The nurse is conducting a physical examination of a 9-month-old infant with a suspected neuromuscular disorder. Which finding would warrant further evaluation? absence of tonic neck reflex absence of Moro reflex presence of Moro reflex presence of symmetrical spontaneous movement

Explanation: The persistence of a primitive reflex in a 9-month-old would warrant further evaluation.

What methods can a nurse use to evaluate extremity function in an 18-month-old? Select all that apply. Observe the child in developmentally appropriate play. Have the child push against resistance with both feet. Ask the child to squeeze the nurse's fingers simultaneously. Elicit from the parent a description of fine and gross motor activities. Look for symmetric motion in the arms and legs.

Observe the child in developmentally appropriate play. Elicit from the parent a description of fine and gross motor activities. Look for symmetric motion in the arms and legs.

The nurse is conducting a physical examination of a 10-year-old child. The nurse whispers the child's name from behind the child so that the child does not see the nurse's lips moving. Which cranial nerve is the nurse assessing? IV III V VIII

VIII Explanation: Testing a child's hearing by observing a response to a whisper without a visual clue assesses cranial nerve VIII, the acoustic nerve.

The nurse caring for a client diagnosed with muscular dystrophy would expect which laboratory values to be most abnormal? bilirubin creatine kinase sodium serum potassium

creatine kinase Explanation: Serum creatine kinase levels are elevated early in the disorder, when significant muscle wasting is actively occurring.

The nurse is assessing for bladder and bowel function in a newborn with spina bifida at the level of the lumbar spine. Which reflex test would the nurse use to assess this function? Achille's Cremasteric Gag Anal wink

Anal wink Explanation: Because the infant likely has neurological impairment below the level of the lesion, neurogenic bladder and/or bowel is of concern. Although it is difficult to assess for neurogenic bowel in a newborn, assessment of the anal wink is a good predictor of functional bladder control. To elicit the anal wink, the nurse would gently stoke the skin near the anus and observe for a contraction of the anal sphincter muscle. If contraction does not occur, bladder and/or bowel function is likely impaired.

The nurse is caring for a child with a spinal cord injury at T5 with corresponding paralysis. The child begins to exhibit a headache, hypertension, arrhythmias, facial flushing, and sweating. What action(s) will the nurse take? Select all that apply. Lay the child flat. Administer antihypertensives as prescribed. Administer albuterol stat. Monitor blood pressure every 5 minutes. Assist the child with voiding.

Assist the child with voiding. Monitor blood pressure every 5 minutes. Administer antihypertensives as prescribed. Explanation: The child is exhibiting signs and symptoms of autonomic dysreflexia. Priorities of care include sitting the child upright, removing the trigger stimulus, emptying the bladder, and monitoring the blood pressure every 2 to 5 minute

The nurse is assessing the moro (startle) reflex of a 2-month-old infant. Place the steps in the order in which the nurse will proceed. Use all options. Click an option, hold and drag it to the desired position, or click an option to highlight it and move it up or down in the order using the arrows to the left. 1 Observe for the arms flaring outward and abducting. 2 Gently lift the infant off the surface by the arms. 3 Place the infant in the supine position. 4 Continue lifting until the shoulders are off the bed but the majority of the head is still on the bed. 5 . Let go of the arms.

Place the infant in the supine position. Gently lift the infant off the surface by the arms. Continue lifting until the shoulders are off the bed but the majority of the head is still on the bed. . Let go of the arms. Observe for the arms flaring outward and abducting.

In caring for the child with Guillain-Barré syndrome, the nurse will provide much supportive care while watching carefully for signs of deterioration in which body system? Integumentary Respiratory Cardiovascular Urinary

Respiratory Explanation: Guillain-Barré is a life-threatening disease; the greatest risk occurs during the acute stage, when respiratory failure may occur. The child with this syndrome will be ill and will have limited mobility for an extended time

Which nursing diagnosis will the nurse prepare for the infant who is placed prone to protect the myelomeningocele repair site? Disorganized infant behavior Peripheral neurovascular dysfunction Risk for activity intolerance Risk for impaired skin integrity

Risk for impaired skin integrity

A newborn is diagnosed with spina bifida. What initial reactions might the nurse expect to observe in the parents of the newborn? Select all that apply. Denial Elation Shock Disbelief Acceptance

Shock Disbelief Denial

The nurse is assessing a 1-month-old infant's ability to feed during the neurologic assessment. What action will the nurse perform? Place a finger below the infant's toes on the plantar surface. With the infant held in an upright position with the infant's back to the nurse, quickly move the infant forward, as if falling suddenly. Stroke the infant's cheek. With the infant prone, stroke along the spine on one side.

Stroke the infant's cheek.

A nurse has provided care to several children during their well-child visits. The nurse has assessed each child's neurologic status. Which assessment finding indicates a problem requiring intervention? a 4-year-old preschool-age child who consistently walks on tiptoes a 10-month-old infant who is able to ambulate with assistance a 2-month-old infant who reaches for a rattle several times before connecting with it a 2-year-old toddler who can walk up the steps one at a time

a 4-year-old preschool-age child who consistently walks on tiptoes Explanation: At 4 years of age, a child should not consistently walk on tiptoes. This is a common manifestation of muscular dystrophy and requires intervention.

The nurse is assessing the head of a 1-year-old child during a well-child examination as part of the child's neurological assessment. What will the nurse include in the assessment? Select all that apply. measuring the head circumference assessing for symmetry and shape visually comparing the size of the head to the length of the body following the child's growth over time utilizing an appropriate growth chart

assessing for symmetry and shape measuring the head circumference utilizing an appropriate growth chart following the child's growth over time

The nurse is reviewing IV therapies that are given to treat cardiac dysfunction with the parents of a child with muscular dystrophy. Which medication(s) will the nurse include in the teaching? Select all that apply. furosemide carvedilol losartan enalapril captopril

furosemide enalapril captopril carvedilol Explanation: Furosemide, enalapril, captopril, and carvedilol are all medications that can be given by IV to treat cardiac dysfunction in muscular dystrophy. Losartan is given by mouth (PO).

The nurse is assessing the coordination of a 2-year-old child at a well-child visit. For what will the nurse observe? attempts to touch the caregiver's face ability to walk only on the heels, then only on the toes heel-to-toe walking ability gait while walking

gait while walking Explanation: The nurse will observe the gait while walking for a 2-year-old child, because this is a developmentally appropriate approach to assessing the child's coordination based on the child's age.

The nurse is teaching the parents of a child with cerebral palsy about side effects that may occur as a result of anticonvulsant therapy. What will the nurse include in the teaching? gingival hyperplasia Gower sign autonomic dysreflexia tachycardia

gingival hyperplasia Explanation: The nurse should inform the parents that gingival hyperplasia may occur as a result of anticonvulsant therapy.

Through which mechanism is Duchenne muscular dystrophy acquired? environmental toxins autoimmune factors virus heredity

heredity Explanation: Muscular dystrophy is hereditary and acquired through a recessive sex-linked trait.

A 3-month-old infant is seen in the pediatric clinic. The infant's parent expresses concern that the child has developed cerebral palsy. The nurse assesses the infant. Which assessment finding indicates to the nurse that the parent's concern is valid? unable to sit without support exhibits Gower sign hypertonia in the upper extremities turns head toward sounds

hypertonia in the upper extremities Explanation: Cerebral palsy manifests as hyper- or hypotonia, and cognitive and developmental delays

A 7-year-old child with cerebral palsy comes to the clinic for an evaluation. The child is prescribed medications to address muscle spasticity and seizures. When assessing the child's mouth, which condition would the nurse associate with the child's medication regimen? Multiple dental caries Gingival hyperplasia Malocclusion Enamel erosion

Gingival hyperplasia Explanation: Although enamel defects and malocclusion are common dental problems in children with cerebral palsy, the child is receiving anticonvulsant therapy which can lead to gingival hyperplasia

Which finding will cause the nurse to refer a 6-month-old child for further neuromuscular testing? Supporting own weight when placed in standing position Head lag when pulled from supine to sitting Bilaterally open rather than closed hands Equal withdrawal of lower extremities from pain

Head lag when pulled from supine to sitting

The parents of a infant born with an abnormality on the back are told by the neonatologist that their child has a myelomeningocele. They ask the nurse what exactly that means. Which would be the nurse's best reply? "The contents of the sac you see only has fluid in it and should cause the child no problem." "It is a herniation through the skin of the back of your child with both the spinal cord and nerve roots involved." "Your child's defect involves only the nerves to the bladder and bowel and can be easily repaired." "The sac is a very small cyst and should resolve within the first year of life."

"It is a herniation through the skin of the back of your child with both the spinal cord and nerve roots involved."

The nurse is conducting a presentation for a group of parents of adolescents at a local high school about spinal cord injury. One of the parents asks, "What is the most common cause of this type of injury?" Which response by the nurse would be most appropriate? "There is no one primary cause of this type of injury." "Firearms are highest on the list as a cause of injury." "Sports-related injuries account for most spinal cord injuries." "Motor vehicle accidents cause over 50% of these injuries."

"Motor vehicle accidents cause over 50% of these injuries."

A nurse is caring for a child with spina bifida. The child's mother asks the nurse what she did to cause the birth defect. Which statement would be the nurse's best response? "It has been linked to maternal alcohol consumption during pregnancy." "It's a common complication of amniocentesis." "Older age at conception is one of the major causes of the defect." "The cause is unknown and there are many environmental factors that may contribute to it."

"The cause is unknown and there are many environmental factors that may contribute to it.

The nurse is providing education to the parents of a child with spinal muscular atrophy (SMA) regarding the use of chest physiotherapy. Which statement by the parents indicates an understanding of the teaching? "This will slow the progression of the curvature of the spine." "This will help strengthen the swallowing muscles." "This will allow our child to maintain an upright sitting position." "This will help facilitate drainage and airway clearance."

"This will help facilitate drainage and airway clearance."

The nurse is conducting a physical examination of a 10-year-old boy with a suspected neuromuscular disorder. Which finding is a sign of Duchenne muscular dystrophy? appearance of smaller than normal calf muscles lordosis Gowers sign indications of hydrocephalus

A sign of Duchenne muscular dystrophy (DMD) is Gowers sign, or the inability of the child to rise from the floor in the standard fashion because of weakness.

The nurse is caring for an 8-year-old with Duchenne muscular dystrophy (DMD) who is experiencing cardiomyopathy. The provider has prescribed carvedilol as part of the child's treatment plan. Which assessment(s) would be important for the nurse to complete before administering this agent? Select all that apply. Radial pulse Cardiac output Blood pressure Respiratory rate Apical pulse

Apical pulse Blood pressure Explanation: Prior to administering carvedilol, a beta-1 selective adrenergic blocker, it would be important for the nurse to assess the child's apical pulse and blood pressure. If the child's apical pulse is less than 90 beats/minute, the nurse would hold the drug and notify the provider. The nurse would also monitor the child's blood pressure for hypotension, holding the drug and notifying the provider if it occurs.

The parents of a 4-year-old child bring the child to the provider's office for an evaluation. The parents are concerned about the child's weakness and problems with stair climbing. The nurse assesses the child and documents the findings. Based on the findings, the nurse suspects Duchenne muscular dystrophy (DMD). When discussing the findings with the primary care provider, the nurse anticipates which laboratory test being prescribed to provide additional information? Creatinine kinase (CK) White blood cell count Prothrombin time (PT) Alkaline phosphatase

Creatinine kinase (CK) Explanation: In light of the child's assessment findings, the nurse would anticipate the need for a creatinine kinase (CK) level. An elevated creatinine kinase (CK) level further raises the suspicion for DMD and should prompt a referral to a genetic specialist

The nurse is caring for a newborn with spina bifida and a myelomeningocele who was born approximately 1 hour ago. What action will the nurse anticipate in the plan of care for the child? Monitor for signs of autonomic dysreflexia. Administer IV furosemide as prescribed. Prepare the infant for spinal surgery. Assess the infant for Gower sign.

Prepare the infant for spinal surgery. Explanation: The nurse will anticipate preparing the infant for spinal surgery, because this is typically done for newborns with spina bifida within 24 to 36 hours of birth.

The nurse is caring for a 3-year-old child with muscular dystrophy who has a respiratory infection and is on mechanical ventilation. What intervention(s) will the nurse take to maintain effective ventilation for the child? Select all that apply. Keep the bed flat. Secure tubing and airway devices. Avoid repositioning. Perform frequent mouth care. Provide frequent suctioning as needed.

Provide frequent suctioning as needed. Perform frequent mouth care. Secure tubing and airway devices.

The nurse is performing a neurological assessment of a 6-month-old infant. Which finding requires further follow up by the nurse? With the infant supine, the nurse gently lifts the infant off the bed by the arms. When the shoulders are off the bed, but the majority of the head is still on the bed, the nurse lets go of the arms. The infant's arms flare outward and abduct. The nurse places a finger in the palm of the infant's hand. The infant closes the fingers around the nurse's finger. When the nurse attempts to pull away, the infant tightens the grip. The nurse places a finger below the infant's toes on the plantar surface. The infant's toes curl around the nurse's finger. While the infant is supine and calm, the nurse turns the infant's head to one side, causing the infant to extend the arm and leg on the side the head has been turned toward and flex the arm on the other side.

While the infant is supine and calm, the nurse turns the infant's head to one side, causing the infant to extend the arm and leg on the side the head has been turned toward and flex the arm on the other side. Explanation: Turning the infant's head to one side and causing the infant to extend the arm and leg on the side the head has been turned toward and flex the arm on the other side describes the asymmetric tonic neck reflex, which is expected to resolve at 2 to 3 months of age. This is an abnormal finding for a 6-month-old infant.

The nurse is assessing an infant's ability to move the extrinsic eye muscles. For what will the nurse observe? open and reactive pupils, and symmetric eyelids symmetric eye movements response to light touch on the face ability to regard a person's face

open and reactive pupils, and symmetric eyelids

The nurse is providing discharge teaching for the parents of a child with muscular dystrophy. What will the nurse include in the teaching? Select all that apply. nutritional guidelines signs of respiratory failure and cardiac compromise passive and active range-of-motion exercises postoperative care glucocorticoid administration and adverse effects

passive and active range-of-motion exercises glucocorticoid administration and adverse effects signs of respiratory failure and cardiac compromise nutritional guidelines

Which nursing diagnosis is most relevant in the first 12 hours of life for a neonate born with a myelomeningocele? risk for infection impaired physical mobility delayed growth and development constipation

risk for infection Explanation: All of these diagnoses are important for a child with a myelomeningocele. However, during the first 12 hours of life, the most life-threatening event would be an infection

Based on knowledge of the progression of muscular dystrophy, which activity would a nurse anticipate the client having difficulty with first? swallowing standing breathing sitting

standing