Question 1

A baby is born with a myelomeningocele and needs urgent surgery to repair the defect. The parents want to take the baby home instead. What does the healthcare professional tell the parents about the purpose of this surgery?
A)"Surgery is much easier on a tiny infant than on a larger, older baby."
B)"If your baby has surgery this young, he/she cannot feel pain."
C)"Additional nervous system damage will occur the longer we wait."
D)"Prompt surgery is needed to prevent total paralysis later on."

Accepted Answer

Until the myelomeningocele is surgically closed, cerebrospinal fluid (CSF) may accumulate, resulting in further dilation and enlargement of the sac, which may risk more damage to the nervous system. The surgery may or may not be easier on a smaller infant, but that is not the rationale for having surgery as soon as possible. Pain can be felt by newborns. A child with a myelomeningocele will have motor, sensory, reflex, and autonomic function below the level of the lesion and will not have total paralysis.

Question 2

A healthcare professional teaches a parenting class that benign febrile seizures are characterized by what?
A)A temperature higher than 38.5°C (101.3°F)
B)Concurrent respiratory or ear infections
C)Onset after the fifth year of life
D)Episodes lasting 30 min or longer

Accepted Answer

An acute respiratory or ear infection is usually present when a child has a febrile seizure. Simple febrile seizures are rare in infants before 9 months of age or in children older than 5 years of age. The seizure occurs with a rise in temperature higher than 39°C (102.2°F). The seizure is short (15 min or less).

Question 3

Gait disturbances and instability are characteristic of which form of cerebral palsy?
A)Spastic
B)Dystonic
C)Ataxic
D)Biochemical

Accepted Answer

Ataxic cerebral palsy causes damage to the cerebellum and exhibits gait disturbances and instability. Spastic cerebral palsy is associated with increased muscle tone, persistent primitive reflexes, hyperactive deep tendon reflexes, clonus, rigidity of the extremities, scoliosis, and contractures. Dystonic cerebral palsy is a result of injury to the basal ganglia or extrapyramidal tracts. Movements are stiff, uncontrolled, and abrupt, resulting in extreme difficulty in fine motor coordination and purposeful movements. Biochemical is not a type of CP.

Question 4

A pregnant woman is seen for the first time at 6 months' gestation and has not taken prenatal vitamins. The healthcare professional educates the woman on the need for a blood test specifically to assess what substance?
A)Total protein
B)Culture
C)a-Fetoprotein
D)C-reactive protein

Accepted Answer

Pregnant women are advised to take folic acid supplements to prevent the development of neural tube defects. The presence of a neural tube defect (NTD) may result in an elevated amniotic fluid a-fetoprotein (AFP) level and subsequent maternal serum AFP levels. Total protein would provide information on nutritional status. A culture would be done to isolate microorganisms in a suspected infection. C-reactive protein would be drawn to evaluate inflammation.

Question 5

A healthcare professional advises a pregnant woman to add supplements of which nutrient to her diet to prevent birth defects?
A)Iron
B)Vitamin C
C)Zinc
D)Folate

Accepted Answer

Maternal folate deficiency is associated with neural tube defects such as meningocele. All the listed substances are part of a healthy diet, but only folate is associated with a specific birth defect.

Question 6

Which defect of neural tube closure is most common?
A)Anterior
B)Posterior
C)Lateral
D)Midline

Accepted Answer

Posterior defects are most common.

Question 7

A child has phenylketonuria (PKU). The healthcare professional educates the parents on the special diet needed, telling them that children with PKU are unable to synthesize what?
A)Essential amino acid, phenylalanine, to tyrosine
B)Renin, erythropoietin, and antidiuretic hormone
C)Aldosterone, cortisol, and androgens
D)Neurotransmitters gamma-aminobutyric acid (GABA) and acetylcholine

Accepted Answer

PKU is an inborn error of metabolism characterized by the inability of the body to convert the essential amino acid, phenylalanine, to tyrosine. PKU does not affect synthesis of renin, erythropoietin, antidiuretic hormone, aldosterone, cortisol, androgens, GABA, or acetylcholine.

Question 8

Which statements regarding the term myelodysplasia are true? (Select all that apply.)
A)Myelodysplasia is used to define a defect in the formation of the spinal cord.
B)It can be used to refer to brain anomalies involving missing brain tissue.
C)Myelodysplasia correctly includes encephaloceles.
D)It is used to describe a herniation of brain and meninges through a defect in the skull.
E)Myelodysplasia can be used to refer to a form of spina bifida.

Accepted Answer

Although myelodysplasia is defined as a defect in the formation of the spinal cord, the term is also used to refer to anomalies of both the vertebral column and the spinal cord. Birth defects in which the vertebrae fail to close are known as spina bifida (split spine). Anencephaly is an anomaly in which the soft, bony component of the skull and much of the brain are missing. Encephalocele refers to a herniation or protrusion of various amounts of brain and meninges through a defect in the skull, resulting in a saclike structure.

Question 9

The neural groove closes dorsally during which week of gestational life?
A)Second
B)Fourth
C)Eighth
D)Twelfth

Accepted Answer

During the fourth week of gestation, the neural groove deepens, its folds develop laterally, and it closes dorsally to form the neural tube, epithelial tissue that ultimately becomes the central nervous system (CNS). The second week is too early, and the other options represent times periods after the groove closes.

Question 10

A student reads in a chart that a baby was born with an encephalocele. The student asks the healthcare professional to explain this condition. What explanation by the healthcare professional is best?
A)A herniation or protrusion of brain and meninges through a defect in the skull
B)A protruding saclike cyst of meninges filled with spinal fluid and is a mild form of spina bifida
C)Protrusion of a saclike cyst containing meninges, spinal fluid, and a portion of the spinal cord through a defect in the posterior arch of a vertebra
D)Premature closure of one or more of the cranial sutures during the first 18 to 20 months of an infant's life

Accepted Answer

Encephalocele refers to a herniation or protrusion of brain and meninges through a defect in the skull, resulting in a saclike structure. A meningocele is a saclike cyst of meninges filled with spinal fluid. A myelomeningocele contains meninges, spinal fluid, and a portion of the spinal cord with nerves. Craniosynostosis causes malformations due to premature closure of sutures.

Question 11

What is the most common cause of obstructive hydrocephalus in infants?
A)Obstructed arachnoid villi
B)Stenosis of the aqueduct of Sylvius
C)Excessive production of cerebrospinal fluid
D)Impaired cerebrospinal fluid circulation in the subarachnoid space

Accepted Answer

Congenital aqueduct stenosis most commonly causes obstructive hydrocephalus. The other options do not represent the most common cause of this condition.

Question 12

A baby demonstrates asymmetric pathological reflexes and microcephaly at a 9-month checkup. The baby appeared normal at birth. What action by the healthcare professional is best?
A)Determine the baby's risk for developing an HIV infection since birth.
B)Assess the baby for other signs of child abuse or neglect.
C)Ask the mother if she uses drugs or alcohol in excess.
D)Perform spinal cord imaging and other diagnostic studies.

Accepted Answer

A particularly vulnerable site of HIV-1 infection in infants and children is the CNS. This baby demonstrates manifestations of HIV infection, which include failure to attain or loss of previously developed milestones or intellectual functioning, impaired brain growth or acquired microcephaly, and acquired symmetric motor deficits manifested by two or more of these: paresis, pathologic reflexes, ataxia, or gait disturbances. The healthcare professional should assess the baby for his or her risks of contracting HIV infection. Part of the assessment would include the mother's drug history, but many other factors must be considered. Since these manifestations are specific to HIV infection, there is no reason to suspect child abuse or perform imaging specifically on the spinal cord.

Question 13

Parents bring their 1-year-old child to the emergency department, reporting that the child has been irritable and pounding on her head, has projectile vomiting, and seems very sleepy for most of the last 3 days. What diagnostic testing does the healthcare professional prepare the child and parents for as the priority?
A)Brain scanning with either CT or MRI
B)Lumbar puncture with CSF cultures
C)Developmental assessment
D)Ophthalmologic assessment

Accepted Answer

Symptoms of brain tumors may be generalized or localized. The most common general symptom is increased intracranial pressure, which may cause headaches, irritability, vomiting, somnolence, and bulging of fontanels. This young child may not be able to verbalize symptoms so pounding on the head may indicate pain. The most critical test to undertake first is a brain scan. Developmental and ophthalmologic assessments will be part of the workup but do not take priority because of the threat of increased ICP. A lumbar puncture would be done if infection is suspected.

Question 14

What is the anomaly in which the soft bony component of the skull and much of the brain is missing?
A)Anencephaly
B)Myelodysplasia
C)Cranial meningocele
D)Hydrocephaly

Accepted Answer

Anencephaly is an anomaly in which the soft, bony component of the skull and much of the brain are missing. Myelodysplasia is a defect in the formation of the spinal cord. Meningocele is a saclike cyst of meninges filled with spinal fluid and is a mild form of spina bifida. Hydrocephalus is a common cause of accelerating head growth and macrocephaly. Increased intracranial pressure results in enlargement of the cerebrospinal fluid (CSF) compartment (ventricles).

Question 15

What is the result of a Chiari type II malformation associated with a myelomeningocele?
A)Upward displacement of the cerebellum into the diencephalon
B)Motor and sensory lesions below the level of the myelomeningocele
C)Downward displacement of the cerebellum, brainstem, and fourth ventricle
D)Generalized cerebral edema and hydrocephalus

Accepted Answer

One serious, potentially life-threatening problem associated with myelomeningocele is the Chiari type II malformation. This deformity involves the downward displacement of the cerebellum, cerebellar tonsils, brainstem, and fourth ventricle. The other options do not appropriately describe the pathologic characteristics resulting from a Chiari type II malformation.

Question 16

The clinical manifestations of dyskinetic cerebral palsy include what?
A)Increased muscle tone and prolonged primitive reflexes
B)Exaggerated deep tendon reflexes, clonus, and rigidity of extremities
C)Scoliosis, contractures, and stiffness of trunk muscles
D)Jerky uncontrolled and abrupt fine musculoskeletal movements

Accepted Answer

Dyskinetic cerebral palsy is associated with extreme difficulty in fine motor coordination and purposeful movements. Movements are jerky, uncontrolled, and abrupt, resulting from injury to the basal ganglia or extrapyramidal tracts.

Question 17

A 7 months old in the clinic displays an intact tonic neck reflex. What action by the healthcare professional is most appropriate?
A)Chart the finding as normal in the child's chart.
B)Assess the baby for signs of malnutrition.
C)Educate the parents on how to improve mobility.
D)Assess the child's development further.

Accepted Answer

The tonic neck reflex should be unobtainable by 5 months of age, so the professional needs to do a complete developmental and medical history on the child. Malnutrition and mobility are not issues at this time.

Question 18

An infant is brought to the emergency department by parents who report that the baby's fontanels seem to be bulging outward. What action by the healthcare provider is most appropriate?
A)Assess the baby for recent trauma to the head.
B)Measure the head circumference and plot it on a growth chart.
C)Prepare the baby for a lumbar puncture and blood cultures.
D)Determine how much fluid the baby had in the last 24 hours.

Accepted Answer

A bulging fontanel and macrocephaly are common findings in hydrocephalus. The healthcare professional should measure the baby's head circumference and plot it against a standard growth chart to determine if the baby's head is larger than expected. The baby may or may not need a trauma assessment depending on other findings, but this would not be the first action. A lumbar puncture and blood cultures would be done for suspected infection. Fluid intake probably would not have this dramatic an effect on the fontanels.

Question 19

What can microcephaly be caused by? (Select all that apply.)
A)Autosomal gene alterations
B)Prenatal physical abuse of the mother
C)X-linked gene alterations
D)Toxic-induced chromosomal defects
E)Maternal anorexia

Accepted Answer

Autosomal recessive genetic defects in any 1 of 16 chromosomes can cause primary microcephaly (present at birth). Environmental causes include toxin exposure, radiation, intrauterine infection, or chemical exposure may be the initiating factor in secondary microcephaly. Other causes can include infection, trauma, metabolic disorders, maternal anorexia, and the presence of other genetic syndromes.

Quiz 20: Alterations of Neurologic Function in Children

A baby is born with a myelomeningocele and needs urgent surgery to repair the defect. The parents want to take the baby home instead. What does the healthcare professional tell the parents about the purpose of this surgery?

A healthcare professional teaches a parenting class that benign febrile seizures are characterized by what?

Gait disturbances and instability are characteristic of which form of cerebral palsy?

A pregnant woman is seen for the first time at 6 months' gestation and has not taken prenatal vitamins. The healthcare professional educates the woman on the need for a blood test specifically to assess what substance?

A healthcare professional advises a pregnant woman to add supplements of which nutrient to her diet to prevent birth defects?

Which defect of neural tube closure is most common?

A child has phenylketonuria (PKU) . The healthcare professional educates the parents on the special diet needed, telling them that children with PKU are unable to synthesize what?

Which statements regarding the term myelodysplasia are true? (Select all that apply.)

The neural groove closes dorsally during which week of gestational life?

A student reads in a chart that a baby was born with an encephalocele. The student asks the healthcare professional to explain this condition. What explanation by the healthcare professional is best?

What is the most common cause of obstructive hydrocephalus in infants?

A baby demonstrates asymmetric pathological reflexes and microcephaly at a 9-month checkup. The baby appeared normal at birth. What action by the healthcare professional is best?

What is the anomaly in which the soft bony component of the skull and much of the brain is missing?

What is the result of a Chiari type II malformation associated with a myelomeningocele?

The clinical manifestations of dyskinetic cerebral palsy include what?

A 7 months old in the clinic displays an intact tonic neck reflex. What action by the healthcare professional is most appropriate?

An infant is brought to the emergency department by parents who report that the baby's fontanels seem to be bulging outward. What action by the healthcare provider is most appropriate?

What can microcephaly be caused by? (Select all that apply.)