Chapter 20: Alterations of Neurologic Function in Children

MULTIPLE CHOICE
1. The neural groove closes dorsally during which week of gestational life?
a. Second
c. Eighth
b. Fourth
d. Twelfth
ANS: B

During the fourth gestational week, 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.
PTS: 1

REF: Page 660

2. Which nutritional deficiency in a pregnant woman is associated with neural tube defect

(NTD)?
a. Iron
b. Vitamin C

c. Zinc
d. Folate

ANS: D

Maternal folate deficiency is associated with NTDs, but the specific mechanism that relates to
how folate supplements prevent these anomalies is unknown. The other options are not
thought to cause such a defect.
PTS: 1

REF: Page 663

3. Which defect of neural tube closure is most common?

a. Anterior
c. Lateral
b. Posterior
d. Midline
ANS: B

Posterior defects are most common. The other options are not the most common.
PTS: 1

REF: Page 663

4. 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

ANS: A

Anencephaly is an anomaly in which the soft, bony component of the skull and much of the
brain are missing. The other options do not represent this condition.
PTS: 1

REF: Pages 663-664

5. The most common cause of obstructive hydrocephalus in infants is:

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
ANS: B

Congenital aqueduct stenosis most commonly causes obstructive hydrocephalus. The other
options do not represent the most common cause of this condition.
PTS: 1

REF: Page 669

6. What is the term for a herniation or protrusion of brain and meninges through a defect in the

skull?
a. Encephalocele
b. Meningocele

c. Arachnoidocele
d. Cephacephalocele

ANS: A

Encephalocele refers to a herniation or protrusion of brain and meninges through a defect in

the skull, resulting in a saclike structure. The other terms are not used to describe an
encephalocele.
PTS: 1

REF: Page 664

7. 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
ANS: C

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.
PTS: 1

REF: Page 665

8. Prompt surgical repair of a myelomeningocele is critical to best prevent:

a. Infection
c. Mental retardation
b. Paralysis
d. Additional nervous system damage
ANS: D

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 other options are included as possible damage caused by the increased
leaking of CSF.
PTS: 1

REF: Page 665

9. Which body system is the largest site for human immunodeficiency virus (HIV) infection in

infants and children?

a. Central nervous system
b. Gastrointestinal system

c. Integumentary system
d. Musculoskeletal system

ANS: A

A particularly vulnerable site of HIV-1 infection in infants and children is the CNS. This
vulnerability cannot be proven regarding the other provided options.
PTS: 1

REF: Page 677

10. An infant diagnosed with hydrocephalus is observed to demonstrate:

a. Shrunken ventricles
b. Bulging fontanels
c. Retarded head growth
d. Decreased production of cerebrospinal fluid
ANS: B

During the early weeks of life, the head begins to grow at an abnormal rate. Significant
dilation of the ventricles may occur before an abnormal increase in head growth develops. The
fontanels enlarge and become full and bulging. Cerebrospinal fluid production does not
decrease.
PTS: 1

REF: Pages 668-670

11. Gait disturbances and instability are characteristic of which form of cerebral palsy?
a. Spastic
c. Ataxic
b. Dyskinetic
d. Biochemical
ANS: C

Ataxic cerebral palsy causes damage to the cerebellum and exhibits gait disturbances and
instability. This description is not accurate of the other options.
PTS: 1

REF: Page 672

12. Children with phenylketonuria (PKU) are unable to synthesize:

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
ANS: A

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 the other
options.
PTS: 1

REF: Page 673

13. Benign febrile seizures are characterized by:

a. A temperature lower than 39 C
b. Respiratory or ear infections
c. Onset after the fifth year of life
d. Episodes lasting 30 minutes or longer
ANS: B

An acute respiratory or ear infection is usually present. Simple febrile seizures are rare in
infants before 9 months of age or in children older than 5 years of age. The convulsion occurs
with a rise in temperature higher than 39 C (102.2 F). The convulsion is short (15 minutes or
less).
PTS: 1

REF: Page 679

14. What is the most common general symptom of a localized childhood brain tumor?
a. Poor bonding
b. Increased intracranial pressure
c. Delayed extinction of newborn reflexes
d. Failure to thrive
ANS: B

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. The remaining options are not the most common
symptom of a localized childhood tumor.
PTS: 1

REF: Page 685

15. The tonic neck reflex observed in a newborn should no longer be obtainable by:
a. 2 years
c. 10 months
b. 1 year
d. 5 months
ANS: D

The tonic neck reflex should be unobtainable by 5 months of age.

PTS: 1

REF: Page 663 | Table 20-1

16. What term is used to describe a hernial protrusion of a saclike cyst that contains meninges,

spinal fluid, and a portion of the spinal cord through a defect in a posterior arch of a vertebra?
a. Encephalocele
c. Spina bifida occulta
b. Meningocele
d. Myelomeningocele
ANS: D

Myelomeningocele is a hernial protrusion of a saclike cyst containing meninges, spinal fluid,

and a portion of the spinal cord with its nerves through a defect in the posterior arch of a
vertebra. The remaining options are not appropriate terms to identity the described condition.
PTS: 1

REF: Pages 664-665

17. What test is performed on amniotic fluid and maternal blood to test for neural tube defect?
a. Total protein
c. -fetoprotein
b. Culture
d. C-reactive protein
ANS: C

The presence of a neural tube defect (NTD) may result in an elevated amniotic fluid fetoprotein (AFP) level and subsequent maternal serum AFP levels. The tests presented in the
remaining options are not used to diagnose an NTD.
PTS: 1

REF: Page 666

18. The clinical manifestations of dyskinetic cerebral palsy include:

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
ANS: D

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 thalamus. The symptoms presented by the other options are not
associated with this form of cerebral palsy.
PTS: 1

REF: Page 672

MULTIPLE RESPONSE
19. Which musculoskeletal deformities are associated with myelomeningocele? (Select all that

apply.)
Extra digits on feet
Clubfoot
Dislocation of the hips
Scoliosis
Kyphosis

a.
b.
c.
d.
e.

ANS: B, C, D, E

Several musculoskeletal deformities are related to myelomeningocele, including clubfoot,

dislocation of hip or hips, and poor spinal alignment. Spinal deformities, such as scoliosis and
kyphosis, are also common. Extra digits are not associated with myelomeningocele.
PTS: 1

REF: Page 665

20. True microcephaly can 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
ANS: A, C, D

Genetic alterations, including autosomal dominant, autosomal recessive or X-linked genes, or

various chromosomal abnormalities can cause true (primary) microcephaly (present at birth).
Environmental causes include toxin exposure during the period of induction and major cell
migration. Radiation, intrauterine infection, or chemical exposure may be the initiating factor.
Secondary microcephaly (developing postnatally) is associated with a variety of causes
including infection, trauma, metabolic disorders, maternal anorexia experienced during the
third trimester of pregnancy, and the presence of other genetic syndromes.
PTS: 1

REF: Pages 667-668

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

a.
b.
c.
d.

Myelodysplasia is used to define a defect in the formation of the spinal cord.

It can be used to refer to brain anomalies involving missing brain tissue.
Myelodysplasia correctly includes encephaloceles.
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.
ANS: A, E

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.
PTS: 1

REF: Page 663

MATCHING

Match the tumor name with its site of development.

______ A. Medulloblastoma
______ B. Ependymoma
______ C. Cerebellar astrocytoma
______ D. Craniopharyngioma
______ E. Neuroblastoma
22. Originates in the neural crest that normally forms the sympathetic ganglia and adrenal
23.
24.
25.
26.

medulla.
Develops in the fourth ventricle.
Develops in the vermis of the cerebellum and may extend to the fourth ventricle.
Originates from the pituitary or hypothalamus.
Causes unilateral symptoms such as nystagmus.

22. ANS: E
PTS: 1
REF: Page 684
MSC: A neuroblastoma is an embryonal aggressive tumor that originates in the neural crest cells that
normally give rise to the sympathetic nervous system (sympathetic ganglia and the adrenal medulla).
23. ANS: B
PTS: 1
REF: Page 683
MSC: The ependymoma develops in the fourth ventricle and arises from the ependymal cells that line
the ventricular system.
24. ANS: A
PTS: 1
REF: Page 683
MSC: A medulloblastoma is an embryonal tumor and the most common childhood malignant tumor. It
occurs as an invasive tumor that develops in the vermis of the cerebellum and may extend into the
fourth ventricle.
25. ANS: D
PTS: 1
REF: Page 683
MSC: The area of the sella turcica, the structure containing the pituitary gland, is the site of several
childhood brain tumors, including craniopharyngioma (the most common). These tumors may
originate from the pituitary gland or the hypothalamus.
26. ANS: C
PTS: 1
REF: Page 683
MSC: Cerebellar astrocytomas are located on the surface of the right or left cerebellar hemisphere and
cause unilateral symptoms (occurring on the same side of the tumor), such as head tilt, limb ataxia, and
nystagmus when the eyes are turned toward the tumor.