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Flashcards in Peds Rehab Deck (50):
1

What secondary condition requires the most medical management in adults with cerebral palsy?

(a) Constipation
(b) Pain
(c) Cardiovascular disease
(d) Osteoporosis

Answer: (b)
Comments: In adults with cerebral palsy (CP) The incidence of pain is high (67%-82%) across all disability groups and appears to increase with age. It is multifactorial with musculoskeletal issues being of primary concern. Constipation issues persist into adulthood and adjustments to the bowel program may be needed. Adults with CP are not reported to have a higher risk of cardiovascular disease but are less likely to be screened than the general population. Osteoporosis is most common (up to 50% in some studies) in non-ambulatory persons, (Gross Motor Function Classification System, GMFCS levels 4, 5), and it increases with age.

Reference: (a) Tosi, LL, Maher N, Moore DW, Goldstein M, Aisen ML. Adults with cerebral
palsy: a workshop to define the challenges of treating and preventing the secondary
musculoskeletal and neuromuscular complications in this rapidly growing population. Develop MedChild Neurol 2009;5:3-5. (b) Turk, MA. Health, mortality, and wellness issues in adults with cerebral palsy. Devel MedChild Neurol 2009;51:26. (c) Turk MA, Logan LR, Kanter D. Aging with pediatric onset disability and diseases. In: Alexander MA, Matthews DJ, editors. Pediatric rehabilitation: principles and practice. 4th ed. New York: Demos Medical; 2010. p428-430, 439

2013

2

What is the strongest single predictor of mortality in adults with pediatric onset disabilities?

(a) Feeding problems
(b) Presence of epilepsy
(c) Inability to walk
(d) Intellectual disability

Answer: (d)
Comment: Feeding problems, epilepsy and inability to walk are conditions associated with pediatric mortality, but intellectual disability is the strongest single predictor of mortality in adults with pediatric onset disabilities. Intellectual disability affects a person’s ability to manage health care monitoring, exercise programs, nutrition, housing, and sexuality. These items are likely to be more closely monitored by parents and pediatricians for a child than by community workers and adult physicians caring for adults with pediatric onset disabilities.

2013

3

What is the biggest barrier for participation in outdoor activities for children with physical
disabilities?

(a) Lack of an adult assistant
(b) Rejection by physically able children
(c) Need for aids such as braces or wheelchairs
(d) Inaccessible playgrounds

Answer: (d)
Comment: The environment is a significant barrier to play for children with physical disabilities.
Fencing, sand, and inaccessible playground equipment relegate children with disabilities to an
observational role and indicates they are not valued/welcomed. Younger children may see an
adult assistant as a playmate but older children see them as intrusive and a hindrance in social
situations. Physically able children are willing to include children with disabilities in their games
but readily acknowledge limitations of playgrounds in accommodating them. Children often view
their braces, walkers and wheelchairs as extensions of themselves and helpful in play situations.

2013

4

Botulinum toxin injections into the hip adductors, hamstrings and gastrocsoleus in children with
spastic cerebral palsy are shown to

(a) have greater effect in children older than 10 years.
(b) be better than serial casting in management of spastic equinus.
(c) be ineffective in management of spastic equinus gait.
(d) delay progression of hip displacemen

Answer: (d)
Commentary: Younger children with fewer physical limitations have more potential for
improvement than older children with more physical limitations. Serial casting and botulinum toxin injections appear to have similar benefit. Spastic equinus gait is effectively improved with
botulinum toxin injections into gastrocsoleus and hamstrings. Hip displacement can be delayed with botulinum toxin injections into hip adductors and hamstrings but does not affect long-term
outcome.

2012

5

Which antispasticity drug used to treat a 3-year-old child with cerebral palsy binds to GABA
receptors in the spinal cord to inhibit reflexes that lead to increased tone?

(a) Clonidine
(b) Tizanidine
(c) Dantrolene
(d) Baclofen

Answer: (d)
Commentary: Baclofen binds to GABA receptors in the spinal cord to inhibit the reflexes that
lead to increased tone. Clonidine is an alpha 2 agonist, as is tizanidine. Dantrolene works in the
striated muscle at the level of the sarcoplasmic reticulum. All these drugs have pediatric
application. Baclofen can be used beginning at age 2 years

2012

6

Pathological drooling in children with spastic quadriparetic cerebral palsy is

(a) due to excessive saliva production.
(b) unsightly, but has no medical significance.
(c) associated with inefficient, uncoordinated swallowing.
(d) associated with increased dental caries.
.


Answer: (c)
Commentary: Pathological drooling is the unintentional loss of saliva either anteriorly over the
lips or posteriorly over the back of the tongue. It is associated with an inefficient, uncoordinated
swallow. Anterior drooling is normal in infants up to 18 months of age. Recent studies have
shown that salivary production is similar to that of typical children without cerebral palsy.
Medical complications of pathological drooling include chronic aspiration, pulmonary infections
and skin irritation. Saliva is protective of dentition

2011

7

What is the biggest barrier for participation in outdoor activities for children with physical disabilities?
(a) Lack of an adult assistant
(b) Rejection by physically able children
(c) Need for aids such as braces or wheelchairs
(d) Inaccessible playgrounds

Answer: (d)
Comment: The environment is a significant barrier to play for children with physical disabilities. Fencing, sand, and inaccessible playground equipment relegate children with disabilities to an observational role and indicates they are not valued/welcomed. Younger children may see an adult assistant as a playmate but older children see them as intrusive and a hindrance in social situations. Physically able children are willing to include children with disabilities in their games but readily acknowledge limitations of playgrounds in accommodating them. Children often view their braces, walkers and wheelchairs as extensions of themselves and helpful in play situations

2013

8

A 6-year-old girl with Erb palsy since birth has an internal rotation deformity of her right
shoulder. Her shoulder external range of motion, whether passive or active, is zero degrees. Right
elbow, forearm, wrist, and hand function is good, latissimus dorsi strength is normal. Shoulder
MRI shows glenoid dysplasia, but no shoulder dislocation. She writes with her right hand but is
unable to reach the back of her head to fix her hair on the right. What is the most appropriate
management by the physiatrist at this time?

(a) Aggressive stretching of right shoulder internal rotators and strengthening of external
rotators
(b) Evaluation for compensatory strategies and assistive devices for independence in
activities of daily living
(c) Consultation with orthopedic surgeon
(d) Observation until adolescence for anticipated further improvement

A 6-year-old girl with Erb palsy since birth has an internal rotation deformity of her right
shoulder. Her shoulder external range of motion, whether passive or active, is zero degrees. Right
elbow, forearm, wrist, and hand function is good, latissimus dorsi strength is normal. Shoulder
MRI shows glenoid dysplasia, but no shoulder dislocation. She writes with her right hand but is
unable to reach the back of her head to fix her hair on the right. What is the most appropriate
management by the physiatrist at this time?

(a) Aggressive stretching of right shoulder internal rotators and strengthening of external
rotators
(b) Evaluation for compensatory strategies and assistive devices for independence in
activities of daily living
(c) Consultation with orthopedic surgeon
(d) Observation until adolescence for anticipated further improvement

2010

9

When should one be concerned when observing a child with an asymmetric tonic neck reflex
(ATNR,“fencer” position) with neck rotation, relative extension of the limbs on the chin side and
flexion of the limbs on the occiput side?

(a) In a 3-month-old infant who is able to move out of the “fencer” position
(b) In a 5-month-old infant who is unable to move out of the “fencer” position
(c) In a 6-month-old infant whose leg response to the stimulus is greater than the arm’s
(d) In an infant of any age, since the ATNR is a primitive pathological reflex.

Answer: (b)
Commentary: The ATNR is typically present at birth and integrates between 4 and 6 months of
age. An obligatory “fencer” position is abnormal at any age. A persistent or obligatory ATNR
may be an early clue that a child has a disorder of motor control, most often cerebral palsy

2010

10

A 1-year-old boy presents with marked weakness. Parents report a weak cry and cough since
birth, and the child cannot sit independently. Exam findings include a bell-shaped thorax,
hypotonia, some movement of the hands and feet but minimal movement at the hips and
shoulders, and there are tongue fasciculations. The diagnosis is best confirmed by


(a) genetic analysis.
(b) muscle biopsy.
(c) electromyography.
(d) repetitive nerve stimulation

Answer: (a)
Commentary: This child’s presentation is typical for spinal muscular atrophy (SMA) type I, also
known as Werdnig-Hoffmann disease. SMA is the second most common neuromuscular disease
of childhood, occurring with an incidence of 1:6,000. Deletion of the survival motor neuron gene
leads to degeneration of anterior horn cells and can be detected in over 90% of children with
SMA. Prior to availability of genetic diagnosis, EMG and muscle biopsies were utilized.
Repetitive nerve stimulation has been used in the investigation of botulism and congenital
myasthenia gravis but is not helpful in SMA.

2010

11

A 14-year-old girl with spastic quadriplegic cerebral palsy (CP) has driven a power wheelchair as
her primary means of mobility since age 4 years. She recently sustained a fracture of her tibia
with no known significant trauma. You anticipate that the bone mineral density (BMD) z score
for her distal femur as measured on a dual-energy x-ray absorptiometry (DEXA) scan will be

(a) increased relative to peers without spasticity.
(b) increased relative to younger nonambulatory children with CP.
(c) similar to younger ambulatory children with CP.
(d) decreased relative to age-matched peers without CP.

Answer: (d)
Commentary: Children with severe CP develop clinically significant osteopenia. Lower BMD z
scores are associated with greater severity of CP as indicated by gross motor function, and these
scores decrease with age. Rather than occurring primarily from actual losses in bone mineral, as
in aging adults, the decreasing BMD z scores seen in older youths with CP occur because they
have a slower rate of growth in bone mineral, relative to their healthy peers.

2010

12

You suspect that a 10-month-old boy’s subdural hematoma may be the result of child abuse.
Which action is LEAST likely to be helpful in further evaluation?

(a) Skeletal survey
(b) Physical examination for cutaneous injuries
(c) Hematology consultation
(d) Ophthalmology consultation

Answer: (c)
Commentary: The most common cause of serious brain injury in children younger than 1 year of
age is abuse. In very young children subdural hematoma, subarachnoid hemorrhage, retinal
hemorrhages and associated cutaneous, skeletal and visceral injuries are significantly more
common among those with inflicted brain injury than in children with unintentional injury.

2010

13

Botulinum toxin injections into the hip adductors, hamstrings and gastrocsoleus in children with
spastic cerebral palsy are shown to
(a) have greater effect in children older than 10 years.
(b) be better than serial casting in management of spastic equinus.
(c) be ineffective in management of spastic equinus gait.
(d) delay progression of hip displacement.

Answer: (d)
Commentary: Younger children with fewer physical limitations have more potential for
improvement than older children with more physical limitations. Serial casting and botulinum
toxin injections appear to have similar benefit. Spastic equinus gait is effectively improved with
botulinum toxin injections into gastrocsoleus and hamstrings. Hip displacement can be delayed
with botulinum toxin injections into hip adductors and hamstrings but does not affect long-term
outcome.

2012

14

Which antispasticity drug used to treat a 3-year-old child with cerebral palsy binds to GABA
receptors in the spinal cord to inhibit reflexes that lead to increased tone?
(a) Clonidine
(b) Tizanidine
(c) Dantrolene
(d) Baclofen

Answer: (d)
Commentary: Baclofen binds to GABA receptors in the spinal cord to inhibit the reflexes that
lead to increased tone. Clonidine is an alpha 2 agonist, as is tizanidine. Dantrolene works in the
striated muscle at the level of the sarcoplasmic reticulum. All these drugs have pediatric
application. Baclofen can be used beginning at age 2 years.

2012

15

Which ethnicity has the highest prevalence of neural tube defects?
(a) Eastern European
(b) Asian
(c) African American
(d) Hispanic

Answer: (d)
Commentary: Even though neural tube defects have declined across all ethnicities, because of
increased folate intake, the disparity between Hispanics and other ethnicities remains. While
50%-70% of neural tube defects can be prevented by adequate folic acid, genetic influences
remain.

2012

16

What neurological level of spina bifida is associated with active plantar flexion, cavus foot
deformities and neurogenic bowel and bladder?
(a) thoracic (T2-12)
(b) upper lumbar (L1-3)
(c) lower lumbar (L4-5)
(d) sacral (S1-2)

Answer: (d)
Commentary: Bowel and bladder involvement is common at all levels, even in sacral lesions.
Plantar flexion and inversion causes development of the cavus foot in sacral lesions. Lower
lumbar lesions develop unopposed ankle dorsiflexion leading to a calcaneous foot. Upper lumbar
and thoracic lesions develop ankle plantar flexion contractures due to the inability to move the
ankle at all.

2012

17

Which pulmonary function parameters would you expect to increase in a child with advanced
neuromuscular disease?
(a) Total lung capacity
(b) Vital capacity
(c) Residual volume
(d) Expiratory reserve volume

Answer:(c)
Commentary: Patients with neuromuscular diseases (NMDs) demonstrate a restrictive pattern
when fractional lung volumes are measured. There is a reduction in total lung capacity, vital
capacity, and the expiratory reserve volume. In contrast, residual volume (the volume of air that
remains in the lungs after a maximal, complete expiratory maneuver) can actually be elevated
when the respiratory muscles are too weak to deform the chest wall inward to deflate the lungs
fully. These patterns will be exaggerated in children with NMDs who also develop scoliosis.
Forced expiratory flows are typically reduced in proportion to lung volume so that the ratio of the
forced expiratory volume in the first second (FEV1) to FVC is normal or high.

2012

18

A 3-year-old boy with upper lumbar spina bifida has bilaterally dislocated hips on x-ray during a
routine follow up visit. You advise his parents that he
(a) needs surgery to relocate both his hips.
(b) should have at least one hip relocated, surgically.
(c) can still stand and walk despite the hip dislocations.
(d) will not be able to sit comfortably in a wheelchair.

Answer: (c)
Commentary: Due to the imbalance between hip flexors/adductors and hip extensor/abductors,
hip dislocation occurs in 30%-36% of children with upper lumbar spina bifida. Unilateral hip
dislocations tend to cause pelvic obliquity, difficulty with wheelchair positioning and standing,
and surgery is advocated. However, bilateral hip dislocations do not generally require surgical
intervention, because a level pelvis and good range of motion are more important for ambulation
than located hips.

2012

19

Aside from motor vehicle crashes (MVC), which etiology is the most common cause of spinal cord injury in children younger than 5 years?
Medical/surgical complications
a.Medical/surgical complications
b.Violence
c.Sports
d.Falls

a medical/surgical complications

Motor vehicle crashes are the most common cause of spinal cord injury (SCI) in all pediatric age groups, accounting for 65% of SCI in children younger than 5 years. The second most common cause in of SCI in this age group is medical/surgical complications, accounting for 11.6% of spinal cord injuries, while the rate among older pediatric patients (age 16-21 years) is only 0.6%. More common in the older age groups are violence, sports injuries, and falls. For example, among children younger than 5 years, 8.7% experience SCI from violence, while 21.6% in the 16- to 21-year range fall victim to SCI for that reason. Sports account for 0.2 % of SCI in children below age 5, while the percentage in youngsters between the ages of 16 and 21 is 18.3%. Falls account for 6.5% of SCI in children younger than 5 years; 8.1 % of spinal cord injuries in the 16- to 21-year age group are attributed to falls, and 24% of adults over age 22 sustained their spinal cord injury in a fall. Because both physiology and behavior change with age in the pediatric population, the pediatric-onset SCI population exhibits distinct epidemiologic characteristics among different age groups and when compared to the adult-onset SCI population

2014

20

The mother of a 7-year-old ambulatory child with cerebral palsy (CP) has just visited with the pediatric orthopedic surgeon. He has suggested a single-event multilevel surgery (SEMLS) for her child. This surgery would consist of tendon Achilles lengthenings (TAL), femoral varus derotation osteotomies (VDO) and rectus femoris transfers. She is worried that this is too much to do at once and wants your opinion. Your advice is that?

a. he should have serial Tendo Achilles lengthenings (TAL) surgeries, not a single-event multilevel surgery.
b femoralvarus derotation osteotomies (VDO) should not be performed until after puberty
c single event mulitlevel surgery (SEMLS) is the most helpful orthopedic procedure for ambulatory children with CP older than 6 years
d. split posterior tibial tendon transfers would be the surgery of choice in this case.

C is correct

Multiple procedures during a single surgical event is shown to have less morbidity and better long-term outcomes than single procedures done over multiple, separate, surgical events often spanning many years. Single-event multilevel surgery (SEMLS) is defined as 2 or more bony or soft tissue procedures at 2 or more anatomic levels performed during a single surgical procedure. Multiple muscles and joints are targeted when function is the primary goal, because all these structures are interrelated for walking.

2014

21

A pattern of burns on the feet, buttocks and perineum of a 1 year old child is suggestive of

a. sunburn
b playing with matches
c a house fire
d non-accidental trauma

d is correct

Non-accidental trauma accounts for an estimated 10% to 20% of pediatric burns and is more common in children younger than 2 years. Children with nonaccidental trauma are less likely to attend outpatient therapy and follow-up clinics

2014.

22

A 6-month-old boy exhibits hypotonia and generalized weakness. What is the most likely diagnosis?

a. Myelomeningocele
b. Duchenne muscular dystrophy
c. Spinal muscular atrophy
d. Neuroblastoma

Option c is correct.

Spinal muscular atrophy presents within the first 2 months of life with generalized hypotonia and symmetric weakness. Myelomeningocele usually involves weakness of the legs, but not generalized hypotonia. Duchenne muscular dystrophy usually presents after age 2 years with weakness, but attainment of motor milestones is usually normal to mildly delayed. Neuroblastoma may present with movement disorder, but not hypotonia. The differential diagnosis of hypotonia and weakness in an infant is brain lesion (e.g., severe encephalopathy), spinal cord disorders (e.g., spinal cord injury or spinal muscular atrophy), and myopathy.

2014

23

Non-accidental traumatic brain injury in young children is characterized by the clinical triad of subdural hematoma, retinal hemorrhages, and encephalopathy. As the child’s history is taken, which finding is classically noted?

a. The history given is consistent with the severity of injuries.
b. The injuries are reported as unwitnessed.
c. Multiple care providers were present at time of injury.
d Medical attention was sought promptly after the injury.

Option b is correct.

The classic history for nonaccidental traumatic brain injury (shaken baby syndrome) is that of a history incompatible with the severity of injuries, an unwitnessed event, so detail is missing, and the injury is inflicted by a solitary care provider. There is often a delay in seeking medical attention. Recent studies indicate that the child usually experiences a physical impact, in addition to the shaking.

2014

24

Compared to adults, children with burn injuries have a higher frequency of

a. heterotopic ossification.
b. contractures requiring surgical releases.
c. psychological or behavioral problems.
d. long-term physical deficits.

Option b is correct.

Children with burns have good physical and psychological outcomes. Heterotopic ossification is rare in children. Contractures around head, hands, and axilla more commonly require surgical releases in children

2014

25

. Your 3-year-old patient with cerebral palsy has a Gross Motor Function Classification System
(GMFCS) Level IV. Family is asking you what to expect she will be able to do when she is a
teenager. Which activity is the highest level she is most likely to attain as a teenager?

(a) Independent ambulation in the household
(b) Independent ambulation in the community
(c) No independent mobility in the household
(d) Wheelchair use in the community

Answer: D
Commentary: The Gross Motor Function Classification System (GMFCS) classifies mobility of
people with cerebral palsy from I to V. Level I is independent ambulation indoors and outdoors
with no assistive device. Level IV requires wheelchair for household and community mobility

2009

26

Which statement is true about the asymmetric tonic neck reflex (ATNR) or the symmetric tonic
neck reflex (STNR)?

(a) The STNR is present at birth and fades away by 1 year.
(b) The STNR provides postural stability as the child goes from crawling to standing.
(c) The ATNR appears about 6 months of age and fades away by 1 year.
(d) The ATNR is obligatory in all children at certain ages

Answer: B
Commentary: The ATNR is also known as the fencer position and is a neonatal reflex that
disappears by 6 months of age. It is never obligatory in normal children. The STNR appears
about 6 months and disappears by 1 year. It provides postural stability as the child makes the
transition from crawling to standing


2009

27

A 6-month-old child with L4 spina bifida presents to your clinic. He also has shunted
hydrocephalus. Other than repair of his back and shunt placement, his past medical history has
been negative. On examination, you find that he has full hip flexion against gravity and knee
extension strength is at least 4/5. The infant has no movement around the ankle. Feet are in
neutral position. Hip examination is symmetric. Which prediction is most accurate in this
patient?

(a) The child is likely to be a functional community ambulator by age 5 years.
(b) The child is likely to be only a household ambulator.
(c) The child is likely to learn to crutch walk by 18 months.
(d) The child is likely to be only a wheelchair user.

Answer: A
Commentary: This child has a strong quadriceps muscle and no deformities noted at 6 months of
age. He is reported to be healthy. The best early predictor of ambulation in children with spina
bifida is a strong quadriceps muscle. Negative predictors are spine and lower extremity
deformities and obesity. Children do not typically learn to use crutches until 3 to 5 years of age
or older

2009

28

Children with L4-5 spina bifida are most likely to have

(a) equinus foot.
(b) cavus foot.
(c) knee flexion contractures.
(d) knee extension contractures.

Answer: D
Commentary: The knee extensors (quadriceps) are innervated at the L3-4 level, while the knee
flexors (hamstrings) are innervated at the L5-S1 level. A child with L4-5 preserved level would
have quadriceps muscles that work, while hamstrings will either be weak or absent. Foot muscles
are innervated at the L5-S2 levels. Equinus and cavus feet result from asymmetric pull of foot
muscles, which would be seen in sacral levels of spina bifida.


2009

29

Which maternal factor is associated with an increased risk of spina bifida?

(a) Anticonvulsant medications during pregnancy
(b) Upper socioeconomic class
(c) Alcohol ingestion during pregnancy
(d) Folic acid 4mg/day prior to and during pregnancy

Answer: A
Commentary: The etiology of spina bifida is multifactorial. Both polygenic inheritance and
environmental influences contribute. Several studies have shown that there the incidence of spina bifida is reduced if food is fortified with folic acid or if mothers take folic acid prior to conception and during pregnancy. Recommended doses of folic acid are 0.4 mg/day in women who are not at high risk and 4 mg /day in women at high risk (eg, those with a family history of spina bifida). Some studies have also implicated lower socioeconomic class and in utero exposure to
anticonvulsant medications as being risk factors. Maternal alcohol ingestion is not related to an increased risk of spina bifida in a baby.

2009

30

You are asked to evaluate a 1-year-old patient who is not yet walking but is developmentally appropriate. Which reflex would you expect to find?
(a) Asymmetric tonic neck
(b) Symmetric tonic neck
(c) Palmar grasp
(d) Plantar grasp

D

The asymmetric tonic neck reflex (ATNR) and symmetric tonic neck reflex (STNR) are usually integrated by 6 to 7 months. Palmar grasp disappears by 5 to 6 months. Plantar grasp is integrated when walking is achieved. The normal age of walking varies, but may be as late as 18 months.

2008

31

Acquired subluxation or dislocation of the hips in spastic cerebral palsy is usually due to muscular imbalance with excessive tone of which muscles?
(a) Hip flexors and tensor fascia lata
(b) Hip flexors and hip adductors
(c) Rectus femoris and hip abductors
(d) Tensor fascia lata and hip extensors

(b)
Progressive changes associated with hip subluxation in patients with cerebral palsy result from the effects of neuromuscular imbalance on the growth and development of the hip joint. The primary problem is spasticity and muscular imbalance, and the musculoskeletal manifestations are secondary. Soft tissue abnormalities include a muscular imbalance between the stronger flexors and adductors, and the weaker extensors and abductors. A flexion-adduction contracture also shifts the center of rotation of the hip from the femoral head to the lesser trochanter, and the proximal femur is gradually displaced upward and outward.
Ref: Spiegel DA, Flynn

2008

32

In children with spastic cerebral palsy, which approach strengthens weak muscles?
(a) Ankle-foot orthotics
(b) Tendon transfer surgery
(c) Intrathecal baclofen
(d) Functional training

(d)
Children with cerebral palsy often have weakness as part of their disorder. Treatments such as bracing, tendon lengthening or transfers, and medications such as botulinum toxin or intrathecal baclofen add to this weakness. Strengthening programs or functional training programs can help to strengthen weak muscles.

2008

33

The most common etiology of cerebral palsy is
(a) premature birth.
(b) birth asphyxia.
(c) intrauterine stroke.
(d) prenatal infection

Answer: (a)
Commentary: There is a common misperception that cerebral palsy is caused by injury at birth;
however, the greatest risk factor for cerebral palsy is prematurity, accounting for nearly half of
the cases. Low birth weight, infection and stroke are also risk factors. Birth asphyxia accounts for
about 10%.

2011

34

The most common movement disorder associated with cerebral palsy is
(a) spasticity.
(b) dystonia.
(c) athetosis.
(d) rigidity.

Answer: (a)
Commentary: While all of the listed movement disorders can be seen in cerebral palsy, spasticity -- defined as velocity-dependent resistance to passive movement -- is the most common. Dystonia
is described as involuntary sustained muscle contractions that result in twisting and abnormal posturing of the extremities. Athetosis is a slow, nearly continuous writhing movement, once acommon result of kernicterus, coupled with chorea. Rigidity is resistance to movement of a joint because agonist and antagonist muscles are both contracting. It is not velocity dependent and is
rarely found in cerebral palsy.

2011

35

What is your next course of action in a 7-year-old boy newly diagnosed with Duchenne muscular
dystrophy?
(a) Evaluate for a wheelchair
(b) Recommend spinal orthosis to correct scoliosis
(c) Order an echocardiogram
(d) Order physical therapy for contracture reduction

Answer: (c)
Commentary: Duchenne muscular dystrophy is an X-linked recessive disorder caused by the
absence of dystrophin. Prognosis is poor because of cardiac involvement leading to severe heart
failure. Baseline echocardiograms and electrocardiograms should be obtained after establishment
of the diagnosis. Contractures are common in DMD, but typically are seen after 13 years of age.
Scoliosis incidence is between 33% to 100% and presents between ages 12 and 15 years
corresponding to the adolescent growth spurt. Orthotics and a wheelchair will be necessary in the
course of the disease, but not at age 7.

2011

36

According to national databases of spinal cord injury (SCI), children under the age of 6 years are more likely to have which epidemiologic pattern of spinal cord injury?
(a) high tetraplegia, motor incomplete, occurred in motor vehicle accident
(b) paraplegia, complete, occurred in motor vehicle accident
(c) high tetraplegia, complete, caused by medical/surgical complications
(d) paraplegia, motor incomplete, caused by medical/surgical complications

(b) According to databases of the Model SCI Systems and Shriner’s Hospital for Children, children under age 6 years are more likely than teenagers to sustain SCI in a motor vehicle accident. Their injuries are more likely to be T1 and lower, and they are more likely to have complete injuries

2007

37

On examination, a 3-month-old girl still has a Moro reflex, asymmetric tonic neck reflex, and plantar grasp reflex. She does not have any protective extension. You advise her parents that
(a) further diagnostic evaluation is indicated.
(b) she requires a physical therapy evaluation.
(c) she needs a neurology evaluation.
(d) these reflexes are normal reflexes.

(d) These are normal reflexes in a 3-month-old child. The Moro and asymmetric tonic neck reflexes (ATNR) usually are integrated by approximately 6 months. The plantar grasp reflex is integrated by 12 to 14 months after walking has begun. Protective extension in sitting is seen anteriorly at 5 to 7 months, lateral at 6 to 8 months, and posterior at 7 to 8 months.

2007

38

A 2-month-old infant presents to you for evaluation of delayed development. He was the product of a normal term pregnancy, labor, and delivery. Birth weight was 3500 grams. He has had difficulty feeding since birth. Family history is negative for developmental problems. On physical examination, he is awake, but not alert. Weight is 3600 grams. Respiration is unlabored. He has poor head control and decreased tone throughout. Deep tendon reflexes are absent. What is the most likely diagnosis?
(a) Kugelberg Welander syndrome
(b) Duchenne muscular dystrophy
(c) Infantile botulism
(d) Tetraplegic cerebral palsy

(d) This patient illustrates the diagnostic dilemma of the floppy infant. Causes of this problem include central nervous system lesions (both brain and spinal cord), myopathies, neuropathies, and neuromuscular junction problems. This infant has had abnormalities since birth, which argues against infantile botulism. Kugelberg Welander syndrome (also known as spinal muscular atrophy type 3) has onset during childhood, as does Duchenne muscular dystrophy. Tetraplegic cerebral palsy often presents in infancy with floppiness and hyporeflexia, which later change to spasticity and hyperreflexia

2007

39

The most common musculoskeletal abnormalities seen in a child with L5 myelodysplasia with sparing of the L5 segment and above are
(a) cavus foot, early hip dislocation, hip and knee flexion contractures.
(b) calcaneus foot, late hip dislocation, hip and knee flexion contractures.
(c) cavus foot, late hip dislocation, hip adduction contractures.
(d) calcaneus foot, early hip dislocation, hip adduction contractures

(b) The child with L5 myelodysplasia typically has late hip dislocation, calcaneus foot, hip flexion contractures, and may have either knee extension or flexion contractures, depending on whether quadriceps (L2-4) or hamstrings (L4-S1) are stronger. Gluteus medius (hip abductor, L4-S1) and hip adductors (L1-3) are innervated higher than L5 and are typically balanced in L5 myelodysplasia. Late hip dislocation is due to either unbalanced hip musculature or spinal deformities.

2007

40

A 10-year-old girl presents with scoliosis 5 years after sustaining a severe traumatic brain injury.
Radiographic studies reveal a 25° levoconvex curve from C8 to T12 with the apex at T4. After
consultation with the orthopedic surgeon, you prescribe a spinal orthosis. Which type of orthosis
should be used in this patient?
(a) Cervicothoracolumbosacral orthosis (CTLSO)
(b) Thoracolumbosacral orthosis (TLSO)
(c) Thermoplastic Minerva body jacket (TMBJ)
(d) Sterno-occipital mandibular orthosis (SOMI)

(a) A thoracolumbosacral orthosis is used for scoliosis having an apex at T9 or lower. A sternooccipital
mandibular orthosis immobilizes the neck. A thermoplastic Minerva body jacket is also
used for cervical immobilization. A cervicothoracolumbosacral orthosis such as the Milwaukee
brace extends from the pelvic section to the neck ring and has been shown to correct scoliotic
curves throughout that area

2006

41

Spinal instrumentation in neuromuscular scoliosis is indicated when
(a) onset of scoliosis is before skeletal maturity.
(b) primary curve exceeds 25° and forced vital capacity (FVC) is greater than 35% of normal.
(c) ambulation abilities are lost due to severe back pain.
(d) primary curve exceeds 50° and FVC is less than 25% of normal

(b) The indication for spinal instrumentation in neuromuscular scoliosis is that the primary curve
exceeds 25° and forced vital capacity has not dropped below 35% of normal.

2006

42

Randomized controlled trials examining intrathecal baclofen (ITB) use in children with cerebral
palsy show that children who receive ITB have
(a) improved upper extremity function.
(b) reduced spasticity in lower extremities.
(c) improved walking and transfers.
(d) improved knee range of motion

(b) A comprehensive review of published English language studies on intrathecal baclofen (ITB) showed evidence of statistically significant improvement in upper and lower extremity tone with ITB use in children with cerebral palsy (CP). Other reported improvements with ITB in children with CP are either anecdotal or not substantiated by randomized controlled trials.

2006

43

The most common congenital limb deficiency is a
(a) transverse tibial and fibular limb deficiency (below-knee limb deletion).
(b) transverse transmetacarpal limb deficiency (partial hand deletion).
(c) longitudinal fibular deficiency (fibular hemimelia).
(d) transverse radial limb deficiency (below-elbow limb deletion).

(d) The left short transradial congenital limb deficiency (below-elbow limb deletion) is the mostcommon congenital limb deficiency. It is thought to be caused by a clot which occludes the artery,resulting in resorption of the distal limb, often leaving nubbins of fingers at the end of the stump.

2006

44

You are covering for your partner who is a pediatric physiatrist. Today, you are admitting a 2-yearold
girl who sustained a burn. You suspect that this girl is a victim of a non-accidental injury (ie,
child abuse) because you see
(a) non-symmetrical burns.
(b) splash marks in a scald-type injury.
(c) injuries in various stages of healing.
(d) non-uniform burn depth.

(c) Non-accidental injuries account for approximately 10% to 28% of pediatric burns. Of all nonaccidental
injuries experienced by children, 10% are due to burns. Among abused children 30% to
70% suffer a repeat injury. Signs of abuse include injury that does not correlate with the type and
location of injury observed on examination; uniform burn depth; sharp lines of demarcation
between burned and non-burned areas; symmetrical wounds (eg, in a stocking or glove pattern); an
absence of splash marks in scald injuries; the presence of other injuries in various stages of healing

2006

45

A 56-year-old man with cerebral palsy is evaluated in your clinic. He is noted to have a slow,
writhing, worm-like movement in his arms. Which term correctly describes this movement?
(a) Dystonia
(b) Chorea
(c) Myoclonus
(d) Athetosis

(d) Athetosis is a continuous, slow writhing movement of the limbs or other body parts. It is important to differentiate athetosis from spasticity, because athetosis does not respond to antispasticity treatment. Chorea is characterized by brief, irregular contractions, which affect individual muscles
as random events that seem to flow from one muscle to the other. Myoclonus manifests as rapid, shock-like or lightning-like jerks. Dystonia is a syndrome of sustained muscle contraction that frequently causes twisting and repetitive movements or abnormal postures.

2006

46

A 6-month-old infant presents to you with hypotonia. You perform an electrodiagnostic study which
shows normal motor conduction velocity, normal sensory conduction velocity and amplitude, normal
motor units, and occasional fibrillations and positive waves. The most likely cause of these findings
is
(a) congenital myotonic dystrophy.
(b) spinal muscular atrophy.
(c) metachromatic leukodystrophy.
(d) infantile botulism.

(a) Hypotonia in infants can be caused by many abnormalities, including cerebral lesions, spinal cord
pathology, polyneuropathies, and myopathies. These electrodiagnostic findings are most consistent
with congenital myotonic dystrophy

2006

47

A 17 year old patient with cerebral palsy presents at rest as shown in the photo. On exam, he has a repetitive twisting movement and full passive range of motion with varying resistance felt during velocity-dependent testing in the joints of the right upper limb. This presentation is best described as:
a. Clonus
b. hypertonic rigidity
c. contracture
d. spastic dystonia

d

spastic dystonia is muscle overactivity present at rest; it is spontaneous, has no primary triggering factor and causes deformation of joints and body postures. It frequently produces twisting or repetitive movements. In contrast to spastic dystonia, hypertonic rigidity is characterized by consistent resistance being felt throughout a passive range of motion, regardless of the speed or direction of movement. Clonus is a rhythmic, often self-sustaining contraction evoked by rapid muscle stretch. Contracture is fixed resistance to passive stretching of muscles due to shortening or wasting (atrophy) of muscle fibers or the development of scar tissue (fibrosis) over joints.

2015

48

What is the most commonly reported health concern of adults with cerebral palsy?

a. depression
b. seizures
c. falls
d. pain

d

Pain is the most reported complaint in adults with cerebral palsy; seizures and falls are less commonly reported concerns, followed by contracture and osteoarthritis. Depression is more likely to be a complaint of adults with Down syndrome.

2015.

49

a pediatrician requests your assessment of a 9-month old infant. The child's parents feel something is wrong because he is not keeping up with a cousin of similar age. You are concerned because the child is not yet able to:

a. cruise along furniture
b. hold objects with his fingertips.
c. transfer toys from one hand to another
d. point to nose on request

c

By 18 months a child should have a 10-25 word vocabulary using single words at a time. By 24 months this should have increased to atleast 50 words and they should be putting 2 words together, for example, "go now". By age 3 a child should be able to state his or her full name, count to 3 and recognize basic colors

2015

50

A pediatrician asks your advice about ordering a speech therapy referral for a 2 year old child whose parents feel he isn't speaking as well as his older sibling did at the same age. By what age should a child be able to use 2-word phrases?

a. 18 months
b. 24 months
c. 30 months
d. 36 months

b

By 18 months a child should have a 10-25 word vocabulary using single words at at time. By 24 months, this should have increased to at least 50 words and they should be putting 2 words together, for example, "go now". By age 3 child should be able to state his or her full name, count to 3 and recognize basic colors

2015.