Peds SAEs Flashcards
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.
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
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.
Which pulmonary parameter is predictive of mortality in a child with Duchenne muscular
dystrophy?
(a) Maximal expiratory pressure
(b) Peak flow rate
(c) Cough peak flow
(d) Forced vital capacity
Answer:(d)
Commentary: One simple method of assessing the interplay between pump function and load is
the measurement of the forced vital capacity (FVC) and fractional lung volumes. In boys with
Duchenne muscular dystrophy (DMD), the relationship between the absolute value of FVC and
age can be divided into 3 epochs: one of gradual increase coincident with their ambulatory period,
followed by a plateau phase at 10 to 12 years when they become confined to wheelchairs, and
then a gradual but persistent decline thereafter. However, when the FVC is described as a percent
of the predicted value, it is lower than normal and diverges from the normal curve over time. The
decline in FVC to a value of less than 1 liter may also predict mortality in patients who do not
receive assisted ventilation.
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.
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.
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.
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.
- Which disorder does NOT have pes cavus as a feature?
(a) Poliomyelitis
(b) Cerebral palsy
(c) Friedreich ataxia
(d) Peroneal spastic foot
Answer: (d)
Commentary: The etiology of pes cavus includes malunion of calcaneal or talar fractures, burns,
sequelae resulting from compartment syndrome, residual clubfoot, and neuromuscular disease.
The remaining cases are idiopathic and nonprogressive. Neuromuscular diseases, such as
muscular dystrophy, Charcot-Marie-Tooth (CMT) disease, spinal dysraphism, polyneuritis,
intraspinal tumors, poliomyelitis, syringomyelia, Friedreich ataxia, cerebral palsy and spinal cord
tumors can cause muscle imbalances that lead to elevated arches. Multiple theories have been
proposed for the pathogenesis of pes cavus. Duchenne described intrinsic muscle imbalances
causing an elevated arch. Whereas, peroneal spastic foot is characterized by pain in the foot,
limited subtalar motion, pes planus deformity, and shortening with spasm of the peroneal muscles
often initiated by minor trauma or unusual activity.
- A 14-year-old with severe traumatic brain injury admitted to your rehabilitation unit has no
spontaneous movement. What is the best prevention for heterotopic ossification?
(a) Passive range of motion
(b) Nonsteroidal anti-inflammatory medications
(c) Disodium etidronate (Didronel)
(d) Radiation
Answer: (a)
Commentary: Heterotopic ossification is found in a high percentage of children immobilized by
traumatic brain injury and spinal cord injury. The best prevention for the development of HO is
an aggressive program of passive range of motion. Nonsteroidal anti-inflammatory medications
and radiation are available as treatment options. Didronel is not used in pediatric patients due to
risk of rickets or rachitic syndrome.
- Which muscle group displays the earliest pattern of weakness in Duchenne muscular dystrophy?
(a) Ankle dorsiflexors
(b) Neck flexors
(c) Shoulder flexors
(d) Knee extensors
Answer: (b)
Commentary: In Duchenne muscular dystrophy, weakness is first seen in the neck flexors during
preschool years. Pelvic girdle weakness precedes shoulder girdle weakness by several years.
Ankle dorsiflexors are weaker than plantarflexors; ankle everters are weaker than inverters; knee
extensors are weaker than flexors; hip extensors are weaker than flexors.
- 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.
- Which symptom is the most predictive of cardiac disease in Duchenne muscular dystrophy?
(a) Palpitations
(b) Syncope
(c) Dyspnea
(d) Cachexia
Answer: (c)
Commentary: The most frequent predictive symptom is dyspnea. Absence of exertion dyspnea
from lack of physical activity allows myocardial impairments to remain clinically silent and
difficult to detect. A high index of suspicion is required. Electrocdardiogram abnormalities in
both Duchenne and Becker muscular dystrophy patients are attributed to progressive fibrosis of
the cardiac conduction system and impairment in the cardiac autonomous nervous system.
Palpitations and syncope will be related to the conduction abnormalities and occurs late in the
disease process. Cachexia is a late finding that occurs when feeding becomes difficult as a result
of heart failure and dyspnea.
- 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.
- 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 a
common 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
- 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%.
- 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
Answer: (c)
Commentary: Shoulder surgery, most often internal rotation contracture release, often combined
with latissimus dorsi tendon transfer to provide active external rotation, is shown to improve
shoulder function in children with birth brachial plexus palsy. The majority (60%–70%) of
infants with birth brachial plexus palsy recover spontaneously, in the first months of life. Longterm
sequelae including fixed contractures warrant intervention. Physical or occupational therapy
will be appropriate for this child postoperatively, when greater progress can likely be made.
- 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.
- 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.
- 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.
- As compared to children with severe traumatic brain injuries, children with severe anoxic encephalopathy are more likely to have
(a) rigidity and decreased rate of regaining consciousness.
(b) rigidity and increased rate of regaining consciousness.
(c) hypotonia and decreased rate of regaining consciousness.
(d) hypotonia and increased rate of regaining consciousness.
Answer: A
Commentary: Compared to children with severe traumatic brain injury, children with severe anoxic encephalopathy are less likely to regain consciousness; they also have shorter survival time, and often have profound rigidity.
- 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.
- Which statement is TRUE for children with acute inflammatory demyelinating polyneuropathy compared to adults with that disease?
(a) Both recover at the same rate.
(b) Disease course is more benign.
(c) Residual weakness is more common.
(d) Respiratory failure rates are equal.
Commentary: The natural history of acute inflammatory demyelinating polyneuropathy (AIDP) in children is more benign than AIDP in adults. Children usually recover more quickly by 3 months
on some occasions. Residual weakness is not as common for adults. The best prognostic indicator is the degree of disability at the peak of illness.
- 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.
- 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.
- 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.
An 11-year-old baseball player presents to your clinic complaining of elbow pain. X-rays of the affected side reveal an 8-mm separation of the medial epicondyle. What should be the next step in management?
(a) Relative rest for at least 6 weeks
(b) Long arm cast for at least 4 weeks
(c) Refer to pediatric orthopedic surgeon
(d) Physical therapy for strengthening
(c) “Little league elbow,” seen in throwing athletes with immature skeletons, is a conglomeration of different diagnostic entities caused by valgus and extension-overload. Medial epicondylar avulsion can frequently occur. Separation from 3–5mm can be managed nonsurgically. However, separations greater 5mm usually require surgery.
- 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.
- The family of your 15-year-old patient who had a severe traumatic brain injury 6 weeks ago asks you if they may feed their son. You observe that the patient is agitated at times, has a hoarse voice, and drools. You try to feed him applesauce and notice that he seems to swallow part of it and does not cough. The most likely finding on the videofluoroscopic swallowing study will be
(a) Silent aspiration
(b) Reflux
(c) Coughing and gagging
(d) Normal swallow
(a) The lack of coughing in a patient with neurologic impairment when presented with food may mean a normal swallow, but is more likely to mean silent aspiration. A normal videofluoroscopic swallowing study is unlikely in a patient with a TBI who is drooling and hoarse. Hoarseness may be a sign of reflux, but in a child with a TBI is more likely to mean vocal cord abnormality.
- A 3-year-old child has a high thoracic spinal cord injury. At age 10, which of the following is the most likely complication?
(a) Severe lordosis without scoliosis
(b) Scoliosis requiring surgical treatment
(c) Deep venous thrombosis
(d) Heterotopic ossification
(b) Children who sustain cervical or high thoracic spinal cord injuries at an early age are at high risk of developing progressive scoliosis that requires surgical management.
- Your 5-year-old patient with spastic tetraplegic cerebral palsy needs a wheelchair prescription. He is dependent for transfers, but cognitively normal. He is able to feed himself and uses a communication device. His family transports him in their car in an adapted car seat. On examination, he is unable to sit unsupported, but sits well with minimal support; he has no scoliosis, and his passive range of motion is full. Which elements would be best to include in his wheelchair prescription?
(a) Folding frame, sling seating
(b) Adaptive stroller, linear seating
(c) Tilt in space frame, custom seating
(d) Rigid frame, contoured seating
(d) While this child is totally dependent for transfers, he only requires minimal support to sit upright and has no fixed deformities. Custom seating should be used for those with fixed deformities. A tilt-in-space frame should be used when children need to have their position in space changed frequently because of deformities or medical problems. While it is tempting to prescribe a wheelchair with a folding frame for a family who transports a child in a car rather than a van, the child will be better positioned using contoured seating and a rigid frame. At age 5 years, the size of frame needed will be able to be transported in a car even without folding. Adaptive strollers usually position the child in a reclined position and should be used as a backup to a wheelchair, which is not easily transported in an automobile, or for a child who can walk but periodically needs dependent mobility for fatigue or following seizures or for similar reasons.
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.
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.
- 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.
- 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.
- You are called to see your 3-year-old inpatient with a C5 ASIA class A spinal cord injury. She has a
headache and complains of not feeling well. Vital signs are pulse 60, respirations 20, blood pressure
120/80. Weight 33 lbs (15kg). Physical examination is unchanged from previously. You order:
(a) Place the patient in the supine position.
(b) Administer acetaminophen (Tylenol) orally.
(c) Empty the bladder.
(d) Obtain computed tomography of the head.
(c) The child is experiencing autonomic dysreflexia. The 90th percentile for blood pressure in an
average sized 3-year-old girl is 103/62. A child with C5 tetraplegia would be expected to have
even lower average blood pressure. Initial treatment consists of positioning the patient in an
upright position and emptying the bladder. If this does not correct the problem, medications should
be considered. If medications are needed, either nitropaste 2% or nifedipine may be used. For a
child weighing 15kg the correct initial dose is 0.25 to 0.5 mg/kg/dose (3.75–7mg) of nifedipine or
½ inch of nitropaste.
- Disorders of executive functioning are common in children after severe traumatic brain injury.
Which sign indicates problems of executive function?
(a) Low intelligence quotient
(b) Attention and memory problems
(c) Aphasia
(d) Agitation
(b) Problems of executive function include impairments in attention, memory, and abstract reasoning.
While aphasia and low intelligence may be seen following traumatic brain injury (TBI), they are
not problems of executive function. Agitation is usually seen early in recovery from TBI, at the
Rancho Los Amigos stage 4. The full consequences of a TBI that occurs in a young child may not
be seen until much later, at an age when the child is expected to have that skill. For example,
problems in abstract reasoning in a child who had a TBI at age 5 may not be seen until the child
reaches 9 or 10 years of age.
- What is a reasonable long-term rehabilitation goal for a 6-year-old child with a C6 ASIA class A
spinal cord injury?
(a) Independent lower extremity dressing
(b) Bed mobility
(c) Independent bathing
(d) Independent feeding
(d) A child with C6 ASIA class A spinal cord injury would be expected ultimately to independently
self-feed, but not bathe, do lower extremity dressing, or perform bed mobility.
- 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.
- The Gross Motor Functional Measure (GMFM) is designed to measure
(a) motor changes over time.
(b) quality of motor performance.
(c) achievement of motor milestones.
(d) only walking, running, and jumping ability.
(a) The Gross Motor Functional Measure (GMFM) evaluates motor changes over time in children with
cerebral palsy. It includes activities in prone and supine positions, rolling, sitting, crawling,
kneeling, standing, walking, running, and jumping. It does not measure the quality of motor
performance.
- What percentage of American children with myelomeningocele requires a shunt to manage
hydrocephalus?
(a) 10–20
(b) 25–50
(c) 60–70
(d) 80–90
(d) Seventy-five percent of lesions in spina bifida cystica (myelomeningocele) affect the lumbosacral
spine. Ninety percent of children with spina bifida have hydrocephalus that requires a shunt for
management.
- A 9-year-old girl with C5 ASIA A spinal cord injury sustained 2 years ago is evaluated for upper
extremity splinting. Which statement regarding this scenario is TRUE?
(a) A resting hand splint should be prescribed for daytime use to preserve function.
(b) A wrist extension splint would be contraindicated for daytime use because it would interfere
with function.
(c) A mobile arm support or balanced forearm orthosis could be prescribed to make self-feeding
possible.
(d) A short hand splint should be prescribed to strengthen wrist extensor muscles.
(c) A mobile arm support or balanced forearm orthosis would enable the child with C5 ASIA A spinal
cord injury (SCI) and weak arm muscles to move the arm through useful active range of motion and
to position the hand for function. In patients with C5 SCI, these orthoses are typically combined
with wrist extension splints or a universal or palmar cuff for feeding. A resting hand splint may be
used at night to improve or maintain range of motion, but would interfere with daytime function. A
short hand splint would not strengthen wrist extensor muscles, but may be useful to improve
function.
- A 16-year-old with Duchenne muscular dystrophy presents to your office with a 3-month history of
worsening shortness of breath and pressure-like chest pain. His pulmonary function testing has not
changed significantly. The most likely cause for his complaints is
(a) decreased cardiac output.
(b) bacterial pneumonia.
(c) pulmonary embolus.
(d) aspiration pneumonia.
(a) Given that his pulmonary function has not changed, the most likely cause for his shortness of breath is
cardiac decompensation due to cardiomyopathy. Respiratory failure due to neuromuscular weakness
would typically correspond to worsening of pulmonary function testing. Bacterial and aspiration
pneumonia would both present with much more acute symptoms. Boys and men with Duchenne
muscular dystrophy rarely get deep venous thromboses or pulmonary emboli. If left untreated, the
patient will likely develop congestive heart failure from cardiomyopathy. The decreased cardiac output
usually responds to treatment with digoxin and ACE-inhibitors.
A normal 6-month-old infant may demonstrate which reflex?
(a) Rooting
(b) Automatic walking
(c) Plantar grasp
(d) Posterior protective extension
Rooting and automatic walking reflexes are present at birth, and are integrated by 4 months of age.
Posterior protective extension does not appear until 7 to 8 months of age. Plantar grasp is present at
birth and not integrated until after independent walking occurs at approximately 12 months of age.
Which statement is TRUE about swallowing in infants?
(a) Sucking and swallowing are well-coordinated for oral intake by 34 weeks of gestation.
(b) The infant’s larynx is low, about the level of the sixth cervical vertebra.
(c) The infant’s tongue fills less of the oral cavity than the adult’s tongue.
(d) Oral breathing occurs at birth and may interfere with sucking.
(a) Sucking and swallowing are well-coordinated at 34 weeks’ gestation. Because the infant’s tongue
is more anterior than the adult’s, the tongue fills more of the oral cavity than in the adult. The
larynx in the infant is high, about at the C2–3 level. Newborn infants are obligate nose breathers
and oral breathing is not observed until 3 to 4 months of age.
You are performing a consult on an 8-year-old child who has sustained a traumatic brain injury.
The child has hyperthermia, hypertension, tachycardia, and rigidity. The best management for this
child would be
(a) Propranolol (Inderal).
(b) Baclofen (Lioresal).
(c) Nonsteroidal anti-inflammatory drugs (NSAIDs).
(d) Amantadine (Symmetrel).
(a) Fever in a child with a severe traumatic brain injury should be investigated and infections treated
with appropriate antibiotics. In the absence of infection, the fever in central autonomic dysfunction
is poorly responsive to nonsteroidal anti-inflammatory drugs. Baclofen may help to control the
spasticity, but propranolol is more effective in controlling the hypertension, tachycardia, and
hyperthermia.
The Education for All Handicapped Children Act (EHA, PL 94-142), passed in 1976, and the
Individuals with Disabilities Education Act (IDEA, PL 105-17), passed in 1997, guarantee that
children with disabilities have
(a) education in special schools.
(b) medical care at school.
(c) education in the least restrictive environment.
(d) education in regular classrooms.
(c) The Education for All Handicapped Children Act and the Individuals with Disabilities Education
Act guarantee children with disabilities education in the least restrictive environment. They also
guarantee necessary health care be provided in the school environment (eg, intermittent
catheterization) but do not require medical care be provided.
- A 2-year-old patient with spinal muscular atrophy type 2 (intermediate form) presents with a 25°,
C-shaped scoliosis. What is the best treatment option at this time?
(a) Muscle strengthening
(b) Electrical stimulation
(c) Spinal fusion
(d) Spinal orthosis
(d) Muscle strengthening will not reduce the curve or prevent it from progressing and is not easily
accomplished in 2-year-old children. Posterior or anterior spinal fusion is not indicated with a
curve of this size and is to be avoided in a young child if at all possible. Spinal orthotics are used in
young children with spinal muscular atrophy to improve sitting balance and to attempt to halt curve
progression.
- A 13-year-old boy presents with waddling gait and difficulty in climbing stairs. On examination, he
demonstrates significant weakness in his proximal lower extremity muscles, especially the
quadriceps, and some calf hypertrophy. What is the genetic inheritance of this disorder?
(a) Autosomal dominant
(b) X-linked recessive
(c) Autosomal recessive
(d) No genetic linkage
(b) The abnormal gene for Duchenne and Becker muscular dystrophy (DMD, BMD, respectively) is on the
short arm of the X chromosome at position Xp21Reference. Both DMD and BMD are inherited Xlinked
recessive diseases affecting primarily skeletal and myocardial muscles. Dystrophin is a large
cytoskeletal protein in the subsarcolemmal lattice, the protein that stabilizes the plasma membrane
during muscle contractions.Mutations in the dystrophin gene that result in a complete loss of
dystrophin lead to the DMD phenotype. Mutations that cause a reduced, truncated, or dysfunctional
form of dystrophin to be produced lead to the BMD phenotype. Both DMD and BMD are progressive
myopathies, although DMD is much more severe and is universally fatal. BMD shows a similar pattern
of muscle weakness to DMD but with later onset and much slower rate of progression.
- The most common spinal problem seen with achondroplasia during childhood is
(a) kyphosis.
(b) scoliosis.
(c) spinal stenosis.
(d) low back pain.
(a) While scoliosis may occur in children with achondroplasia, it is less common than kyphosis, which
begins in infancy. Spinal stenosis occurs frequently in individuals with achondroplasia, with 38
years being the average age of symptom onset. Low back pain is extremely frequent in adults with
achondroplasia, but rare in children. Progressive kyphosis that occurs in infants and young children
with achondroplasia is treated with a spinal orthosis.
- In which activity should a 16-year-old girl with C5 ASIA class A spinal cord injury be
independent with the use of assistive devices?
(a) Self catheterization
(b) Transfers to level surfaces
(c) Self feeding
(d) Bathing
(c) While boys with C5 spinal cord injury (SCI) may learn to perform bladder self-catheterization with
assistive devices, girls do not. Level transfers require active elbow and wrist extension, which
would not be present in a person with C5 SCI. Self-feeding with assistive devices such as a palmar
band can usually be done by persons with C5 tetraplegia.
- Your 6-month-old patient had burns to his head and both arms in a house fire. What approximate
percent of his total body surface area (TBSA) was burned?
(a) 37
(b) 18
(c) 27
(d) 49
(a) An infant’s head is approximately 19% and each arm constitutes 9% of the total body surface area
(TBSA). In adults and older children the head is approximately 9% of the TBSA
- Which joints are most commonly involved in juvenile rheumatoid arthritis?
(a) Shoulder, hip, fingers
(b) Atlantoaxial, costomanubrum, hip
(c) Sternomanubrum, shoulder, sacroiliac
(d) Elbow, hip, temporomandibular
(d) The elbow is involved 90% of the time in juvenile rheumatoid arthritis (JRA), the
temporomandibular and hip 50% each. The shoulder is involved about 8% of the time in early JRA
and about 33% later
- Your 14-year-old patient with spastic diplegic cerebral palsy has increasing problems with
spasticity. He walks with ankle-foot orthoses (AFOs) and crutches and is independent in his
activities of daily living. Which medication would reduce his spasticity while minimizing
undesirable side effects?
(a) Diazepam (Valium)
(b) Baclofen (Lioresal)
(c) Dantrolene (Dantrium)
(d) Oxybutynin (Ditropan)
(b) Diazepam has lethargy and sleepiness as major side effects. Dantrolene works at the level of the
muscle and often causes weakness, which can interfere with function. Oxybutynin relaxes the
muscles of the bladder, not skeletal muscles.
- You are asked to evaluate an 8-month-old child with developmental delay. On exam you find low
tone, but brisk deep tendon reflexes at the knees and biceps, full passive range of motion, and poor
head and trunk control. This child’s diagnosis is likely
(a) myotonic dystrophy.
(b) cerebral palsy.
(c) spinal muscular atrophy.
(d) Hunter’s syndrome.
(b) This patient presents with hypotonia, weakness, and hyperreflexia, a combination most commonly
seen in central nervous system lesions such as cerebral palsy. A child with a neuromuscular
disorder would not have hyperreflexia with the hypotonia and weakness. In severe cerebral palsy it
is common to see early hypotonia with brisk reflexes that changes to hypertonia as the child gets
older.
- Which endocrine abnormality is most likely to occur 5 years after severe traumatic brain injury in a
2-year-old girl?
(a) Diabetes insipidus
(b) Precocious puberty
(c) Hypothyroidism
(d) Hyperparathyroidism
(b) Precocious puberty occurs in up to 50% of girls who sustain a severe traumatic brain injury (TBI) in
early childhood. Diabetes insipidus is an early complication of TBI. While other endocrine
abnormalities may occur, they are less common.
- A 12-year-old girl with limb girdle muscular dystrophy has trouble keeping up in school because of
slow gait and writing speed. What is the probable cause of her clinical problems?
(a) Skeletal muscle weakness
(b) Spasticity
(c) Cardiomyopathy
(d) Joint contracture
(a) The majority of clinical problems encountered in neuromuscular disease can be directly linked to
skeletal muscle weakness. Studies indicate that what causes most of the functional problems and
impairs quality of life for people with neuromuscular disease is muscle weakness.
- Parents of children with Duchenne muscular dystrophy who want them to participate in an exercise
program should be advised to
(a) encourage them to exercise to the point of fatigue.
(b) initiate a graduated, submaximal exercise program.
(c) have them participate in a competitive sports program.
(d) preclude exercise because it could make the disease progress faster.
(b) Intervention with submaximal exercise training in neuromuscular disease improves physical
performance and increases muscle efficiency, and thus helps fight fatigue and improve quality of life.
However, in children, the exercise program must be conducted in an enjoyable setting. Supervision is
necessary to make sure the children do not play to exhaustion, which could produce muscle damage
and overwork weakness.
- On physical examination an 8-year-old patient stands on his toes and has increased lumbar
lordosis. He has a Trendelenburg gait with circumduction. What else would you expect to find on
his exam?
(a) Decreased sensation in his feet
(b) Anterior tibialis weakness
(c) Quadriceps weakness
(d) Hyperreflexia at the ankle
(c) The exam describes typical findings in a boy with myopathy such as Duchenne muscular dystrophy
(DMD). The earliest weakness in DMD is proximally in the gluteus maximus. The boy assumes a
posture of lumbar lordosis to place the center of gravity posterior to the hip joint to prevent
hyperflexion of the hip and thus a fall. Toe walking is a compensatory adaptation to knee extensor
weakness.
- You are asked to evaluate a child who was born at 25 weeks gestation and had a grade 4
intraventricular hemorrhage. What type of cerebral palsy are you most likely to find?
(a) Athetoid
(b) Hemiplegic
(c) Diplegic
(d) Hypotonic
(c) Grade 4 intraventricular hemorrhages in premature infants are most commonly associated with
spastic diplegia.
- You are asked to evaluate a child with arthrogryposis and equinovarus. Which treatment strategy
would be the best to employ?
(a) Short leg braces attached to orthopedic shoes
(b) Botulinum toxin injections, stretching, and plastic ankle-foot orthoses
(c) Oral baclofen (Lioresal), range of motion, and extra depth shoes
(d) Casting followed by surgical releases
(d) Arthrogryposis multiplex congenita (AMC) may be caused by a variety of conditions, but is usually
not accompanied by spasticity. Thus botulinum toxin and baclofen are not indicated. Most club
feet (equinovarus) in patients with AMC are resistant to conservative methods and require surgical
releases.
- In children with spastic cerebral palsy, which intervention strengthens weak muscles?
(a) Ankle-foot orthotics
(b) Tendon transfer surgery
(c) Intrathecal baclofen
(d) Functional training program
(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.
- One of your 4-year-old patients exhibits the following characteristics: distress over minor changes in
environment, echolalia, lack of awareness of the existence of feelings in others, nonparticipation in
simple games. The most likely diagnosis is
(a) autism.
(b) cerebral palsy.
(c) hearing impairment.
(d) mental retardation
(a) Autism is characterized by echolalia, inability to play reciprocally, and abnormal relationships with
people. While children with mental retardation, cerebral palsy, and hearing impairment may have
some of these features, they do not have all of them in the absence of autism.
- A 17-year-old boy from India presents with a longstanding history of areflexia and asymmetric
muscular atrophy after a febrile illness as a child. The likeliest site of neurologic pathology is
(a) myelin sheath of peripheral nerves.
(b) axons of peripheral nerves.
(c) anterior horn cells.
(d) muscle membrane.
(c) Poliomyelitis involves the anterior horn cells. During an acute infection, the virus is transported to
the anterior horn cells followed by inflammation and loss of spinal and bulbar motor neurons.
- Which finding is normal in newborn infants?
(a) Extensor tone predominates
(b) Hands are kept fisted
(c) Spine is straight when held in sitting position
(d) Unable to turn head to side in prone position
(b) In normal newborn infants flexor tone predominates and hands are kept fisted. In prone position a
normal newborn is able to turn the head to either side. The newborn has a rounded spine when
placed in supported sitting
- The family of your 10-year-old patient who had a severe traumatic brain injury 6 weeks ago asks you
if they may feed their son. You observe that he is agitated at times, has a hoarse voice, and drools.
You try to feed him applesauce and notice that he seems to swallow part of it and does not cough.
The most likely finding on the videofluoroscopic feeding study will be
(a) Silent aspiration.
(b) Reflux.
(c) Coughing and gagging.
(d) Normal swallow.
(a) The lack of coughing in a patient with neurologic impairment when he/she is presented with food
may mean a normal swallow, but is more likely to mean silent aspiration. A normal
videofluoroscopic swallowing study is unlikely in a patient with a traumatic brain injury (TBI) who
is drooling and hoarse. Hoarseness may be a sign of reflux, but in a child with a TBI it is more
likely to mean vocal cord abnormality.
- Which measure is the first sign of respiratory muscle dysfunction in boys with Duchenne muscular
dystrophy?
(a) Vital capacity
(b) Oxygen saturation
(c) Maximal expiratory force
(d) Negative inspiratory force
(c) Recent studies by McDonald and by Bach showed that reduction of maximal expiratory force
(MEF) to 40%–60% of normal in the 7- to 14-year-old age group was the first sign of respiratory
muscle dysfunction in boys with Duchenne muscular dystrophy (DMD). The earlier and more
severe decreases of MEF that are greater than the decreases in maximal inspiratory force,
correspond to the clinically observed weakness of abdominal muscles, which like coughing are
important in forced expiration. Vital capacity was not found to decrease until an average of 15–16
years. Low oxygen saturation is a late manifestation in DMD, developing after hypercapnia.
- A 17-year-old person presents with frequent falls and no other complaints. On physical examination
you note unusually high arches, distal lower limb strength of 4/5, and decreased reflexes. The most
appropriate next step in the diagnosis would be
(a) nerve conduction studies.
(b) muscle biopsy.
(c) magnetic resonance imaging of the lumbosacral spine.
(d) serum heavy metal levels.
(a) The clinical picture is suggestive of a polyneuropathy. Patients with hereditary polyneuropathies
may have feet with high arches. Electrodiagnostic studies including nerve conduction studies
remain the most important first tests in the evaluation of polyneuropathy.
- A 10-year-old child with L4-5 myelodysplasia and shunted hydrocephalus develops spasticity in her
legs. The most likely cause of this spasticity is
(a) shunt malfunction.
(b) symptomatic Chiari malformation.
(c) growth.
(d) tethered cord.
(d) Tethered cord is the most common cause of new onset spasticity in patients with myelodysplasia.
Linear growth does not cause new spasticity. Symptoms of Chiari malformation include cranial
nerve disorders and respiratory problems. Shunt malfunction may be associated with headaches,
vomiting, eye muscle abnormalities, and sometimes abdominal symptoms.
- A 9-year-old girl with an L1 ASIA class A spinal cord injury that occurred at age 5 years presents in
your office with a 1-day history of a swollen left leg. History is that she woke up with the swollen
leg the day before. There is no history of trauma, fever, or shortness of breath. On examination, you
find a prepubertal girl in no distress with normal vital signs. Upper extremities are normal. Lower
extremities have moderate spasticity and no voluntary movement. Skin is normal. The left leg is
warm and swollen from the ankle to the knee. There is no sensation in the legs. Which test is most
likely to yield the correct diagnosis?
(a) Bone scan
(b) Plain radiograph
(c) Venous Doppler study
(d) White blood cell count with differential
(b) Deep venous thromboses (DVTs) which can be diagnosed by Doppler study usually occur in the
first 3 months after spinal cord injury (SCI) and are rare in prepubertal children. In lower leg DVTs
the foot and leg are usually swollen. Heterotopic ossification (HO), which can be detected by bone
scan, occurs in about 3% of children with SCI and has onset an average of 14 months after injury.
Heterotopic ossification most commonly involves the hip. Cellulitis is usually associated with skin
lesions and usually involves a discrete area. A fracture is the most likely cause of swelling in this
case and can be diagnosed by plain radiographs
- Which positive effect of ankle-foot orthotics has been proven beneficial in the treatment of children
with cerebral palsy?
(a) Improved gait efficiency as measured by gait analysis
(b) Prevention of contractures
(c) Improved knee extensor strength
(d) Decreased plantar flexor posture
(a) There are no large, randomized, controlled studies that show the long-term effects of any type of
Ankle-Foot Orthosis (AFO) on function or contracture formation. Small studies have shown that
both rigid and hinged AFOs improve gait efficiency by preventing plantar flexion.
- Juvenile rheumatoid arthritis (JRA) differs from adult onset rheumatoid arthritis: in JRA
(a) joint destruction occurs earlier.
(b) large joint involvement is less frequent.
(c) the cervical spine is involved less frequently.
(d) systemic features are more common.
(d) Children with juvenile rheumatoid arthritis are more likely to have systemic features, have large
joints involved, and have cervical spine involvement. Adults with rheumatoid arthritis have joint
destruction earlier.
- Children with which physical disorder tend to have higher verbal skills compared to overall
cognitive ability?
(a) Muscular dystrophy
(b) Myelodysplasia
(c) Cerebral palsy
(d) Autism
(b) Children with myelodysplasia have deceptively good verbal facility that creates the impression of
higher intellectual functioning than is found on formal testing (“cocktail party syndrome”).
Children with cerebral palsy, autism, and muscular dystrophy do not typically demonstrate this
finding.
- Your 10-year old patient with T6 ASIA class B paraplegia complains of right knee pain. On
examination there is no swelling of the knee or leg. Knee examination is normal. The right leg
appears shorter when the hips and knees are flexed. What is the most likely cause of these findings?
(a) Knee sprain
(b) Right hip subluxation
(c) Hip adductor spasticity
(d) Dysesthetic pain
(b) Hip subluxation is the most likely cause of knee pain in a child with T6 ASIA B SCI. Pain from
hip pathology is often referred to the knee in children. While hip adductor spasticity contributes to
subluxation or dislocation, the spasticity itself is not painful. Dysesthetic pain is usually
generalized. An abnormal knee examination is usually found in a knee injury that causes pain.
- A 16-year-old girl who had a severe traumatic brain injury 4 weeks ago with left frontal contusion
and left basilar skull fracture demonstrates worse auditory than visual attention. Which diagnostic
test would be most likely to explain this finding?
(a) Magnetic resonance imaging
(b) Electroencephalogram
(c) Audiogram
(d) Visual evoked response
(c) A basilar skull fracture is often associated with a permanent sensorineural hearing loss. Other
traumatic brain injuries may be associated with hearing loss, but this is less common. All patients
with basilar skull fracture should be assumed to have a hearing loss and have audiological
evaluation as soon as possible.
- What condition casues the typical “myopathic gait” seen in a young boy with Duchenne muscular
dystrophy with accentuated lumbar lordosis and toe walking?
(a) Hip and knee extensor weakness
(b) Hip flexion and ankle plantar flexion contractures
(c) Hip extensor weakness and plantar flexion contracture
(d) Hip flexion contracture and knee extensor weakness
(a) The typical “myopathic gait” seen in early Duchenne muscular dystrophy is caused by weakness of
the gluteus maximus and quadriceps muscles. In order to maintain upright posture the child
assumes the hyperlordotic stance. Contractures of the gastrocsoleus and iliopsoas muscles occur
later in the disorder.
- In which type of cerebral palsy is a seizure disorder most commonly seen?
(a) Tetraplegia
(b) Diplegia
(c) Athetosis
(d) Hemiplegia
(d) Approximately 70% of children with hemiplegia have seizures. About 50% of children with
tetraplegic cerebral palsy have seizures. Seizures are rare in children with diplegia or athetosis
- A 1-year-old child with a midlength transfemoral limb deficiency presents for prosthetic
management. Which component should be in the prosthetic prescription?
(a) Socket with a growth liner
(b) Single action knee joint
(c) Vertical shock pylon
(d) Dynamic foot
(a) The 1-year-old child should be fit with a simple prosthesis which suspends securely and allows for
growth. The knee joint should be added between 3 and 5 years. The SACH foot is most commonly
prescribed because of its simple design and durability. All prostheses for growing children should
incorporate a removable growth liner in the socket.
- Which reflex is typically NOT seen in a normal 4-month-old infant?
(a) Extremities extend on the face side as the head is turned to the side.
(b) Fingers flex when the palm is touched.
(c) Extremities extend to the direction of displacement when center of gravity is displaced.
(d) Shoulder abduction, and shoulder, elbow, and finger extension occur when the neck is
suddenly extended.
(c) These options all describe reflexes. (a) asymmetric tonic neck reflex, (b) palmar grasp, and (d)
Moro are seen until a baby is about 6 months old. Protective extension or parachute reaction (c)
does not appear until after 6 months.
- Which statement is true regarding spinal cord injury without obvious radiologic abnormality in
children?
(a) It most commonly occurs in lumbar rather than cervical injuries.
(b) There is a lower incidence in younger children.
(c) It is associated with larger head size and relatively weak neck muscles.
(d) Neurologic impairmen, if it occurs, is usually apparent within 2 to 4 hours post-injury.
(c) Spinal cord injury without obvious radiologic abnormality (SCIWORA) usually occurs in young
children, is thought to be due to the relatively large head size and weak neck muscles, and motor
abnormalities may not be apparent for up to several days. SCIWORA most commonly occurs in
the cervical region.
- A nonambulatory 15-year-old boy with spinal muscular atrophy is requesting a new power
wheelchair after a growth spurt. An important feature of the wheelchair prescription will be
(a) a solid seat with foam padding.
(b) extra room at each side to allow for growth.
(c) seat back below the scapular ridge.
(d) back-slanted seat with pommel.
(a) Because of growth and increasing weakness, a common sequela of motor neuron disease is
scoliosis. It is important to provide adequate pelvic support with a firm seat to avoid hip
asymmetry. If wheelchairs are too big, asymmetric spinal posture is encouraged. Slanted seats with
pommels are useful to control extensor spasticity, which should not be an issue here. Seat backs
should be high to help control spinal posture
- A 3-year-old child has a high thoracic spinal cord injury. When he reaches the age 10 years, which
complication is the child most likely to have?
(a) Isolated lumbar lordosis
(b) Thoracolumbar scoliosis
(c) Deep venous thrombosis
(d) Heterotopic ossification
(b) Scoliosis requiring surgery is a common complication seen in children who have had an spinal cord
injury (SCI) at a young age. Increased lordosis in the absence of scoliosis is rarely seen. Deep
venous thrombosis rarely occurs in young children and when it does occur it usually occurs soon
after the SCI. Heterotopic ossification tends to occur soon after the SCI.
- Acquired subluxation or dislocation of the hips in spastic cerebral palsy is usually due to muscular
imbalance and pull of the
(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) Strong hip flexor and adductor muscles can overpower weak extensors and abductors. Acquired
hip dislocation can be prevented in some cases by release of spastic hip flexors and adductors.
- Your 15-year-old patient with Duchenne muscular dystrophy complains of new onset morning
headaches. What is the most likely cause?
(a) Neck extensor tightness
(b) Hypercarbia
(c) Migraines
(d) Vision changes
(b) Migraines do not typically occur only in the morning. Neck extensor tightness usually occurs
before the loss of ambulation in boys with Duchenne muscular dystrophy, which usually occurs
before the age of 15 years. Vision changes usually do not cause morning headaches. Hypercarbia
results from hypoventilation during sleep and is an early sign of impending respiratory failure.
- Which insult is the most likely cause of spastic diplegic cerebral palsy?
(a) Intrauterine stroke
(b) Hyperbilirubinemia in the neonatal period
(c) Postnatal intraventricular hemorrhage
(d) Perinatal asphyxia
(c) Spastic diplegic cerebral palsy occurs most commonly in premature infants who have had an
intraventricular hemorrhage during the neonatal period. Intrauterine stroke causes hemiplegia.
Neonatal hyperbilirubinemia most commonly causes athetosis. Birth asphyxia is more commonly
associated with spastic quadriplegic cerebral palsy.
- The best predictor of community ambulation beyond childhood in patients with myelomeningocele is
(a) body mass index.
(b) quadriceps strength.
(c) early surgical closure of the meningocele.
(d) bowel and bladder continence.
(b) There are many studies about longterm outcomes of ambulation in children and adults with
myelomeningocele. While many factors influence outcome, including intelligence, medical
problems, and obesity, the best predictor of ambulation into adulthood is strong quadriceps
function. Bowel and bladder continence has no relationship to ambulation.
- A child with C5 ASIA A spinal cord injury should eventually become independent in which
activity?
(a) Intermittent catheterization
(b) Transfer to level surfaces
(c) Feeding
(d) Bathing
(c) A child with C5 ASIA A spinal cord injury should eventually become independent in feeding, and
in upper extremity dressing with assistive devices, in driving a power wheelchair, and in propelling
a manual wheelchair short distances on level surfaces.
- A 6-month-old child presents in your office for rehabilitation assessment. She was born at full
term. There was mild transient respiratory distress at birth. The patient was noted to be diffusely
hypotonic at birth except for normal cranial nerves. There were no feeding issues once the
respiratory distress resolved within 24 hours. The baby has remained relatively hypotonic since
birth. However, she has become very socially alert and aware and attempts to use her arms to reach
for toys and pick up lightweight objects. She doesn’t roll. She cannot sit except very briefly when
propped and bearing weight through both arms with elbows extended. On examination, head
circumference is normal, length is normal, as is weight. There is a pronounced head lag. Arms,
while in the supine position, maintain a “jug-handle” posture. Reflexes are present but diminished.
There is no spasticity. The cranial nerves are normal except for fine fasciculations of the tongue.
The most likely diagnosis is
(a) myotonic muscular dystrophy.
(b) cerebral palsy.
(c) infantile botulism.
(d) spinal muscular atrophy
(d) Spinal muscular atrophy (SMA)is a term used to describe a group of inherited disorders
characterized by weakness and muscle wasting due to degeneration of anterior horn cells of the
spinal cord and brainstem motor nuclei. Three subtypes of autosomal recessive predominantly
proximal SMA have been linked to chromosome 5q. The majority of cases of SMA type I present
within the first 2 months of life with generalized hypotonia and symmetric weakness. Children
typically sit only with support. Tongue fasciculations have been reported in 56%-61% of patients.
Proximal muscles are weaker than distal.
- Secondary injury in pediatric brain trauma is caused by
(a) hypotension, hypoxia, and hydrocephalus.
(b) growing skull fractures.
(c) coup and contrecoup cerebral contusions.
(d) diffuse axonal injuries and punctate hemorrhages.
(a) Any disorder that interferes with cerebral perfusion or oxygenation can cause further damage
following traumatic brain injury. This includes hypotension, hypoxia, increased intracranial
pressure because of cerebral edema, acute hydrocephalus, or space-occupying lesions. Midline shift
or herniation may lead to infarction because of pressure or traction on cerebral vessels. Therefore,
efforts are made to control intracranial pressure through fluid and electrolyte management,
hyperventilation, and maintenance of normal blood pressure and oxygenation. Growing skull
fractures result from the arachnoid protruding through a dural tear, producing a cyst that can
contribute to a widening skull deficit, which usually requires operative repair. This is a
complication of traumatic brain injury but not a secondary injury. Coup and contrecoup cerebral
contusions and diffuse axonal injuries are examples of primary injury.
- To prevent contractures, which position is the correct placement for children with major burns?
(a) Shoulder in external rotation
(b) Wrist in extension
(c) Hip in flexion
(d) Metacarpophalangeal joints in hyperextension
(b) Children with major burn injuries should be placed in positions that tend to prevent contractures.
These include neck extension (no pillows); shoulders at 90° abduction and neutral rotation with
elbows, wrists, hips, and knees extended; feet at neutral dorsiflexion, metacarpophalangeal joints at
70° to 90° flexion and finger interphalangeal joints in full extension.
- Which finding would indicate a poor long-term outcome in a 9-year-old child with a severe traumatic
brain injury?
(a) Bladder and bowel incontinence
(b) Agitation
(c) Dysphagia
(d) Hypertension and hyperpyrexia
(d) Most children with severe traumatic brain injury have dysphagia, incontinence, and agitation at
some time during the recovery period. Central autonomic dysfunction (hypertension, hyperpyrexia,
sweating, tachypnea, and rigidity) is associated with worse cognitive and motor outcomes a year or
more after injury.
- The most useful clinical criterion to distinguish Becker muscular dystrophy from Duchenne
muscular dystrophy is
(a) creatine kinase values at the time of diagnosis.
(b) walking ability during the teen-age years.
(c) Gowers’ sign and calf enlargement.
(d) age at onset of diagnosis.
b) The most useful clinical criterion to distinguish Becker muscular dystrophy (BMD) from Duchenne
muscular dystrophy (DMD) is the continued ability of the patient to walk into late teen-age years.
Persons with BMD will typically remain ambulatory beyond 16 years. Outlier DMD cases
generally stop ambulating between 13 and 16 years of age. Creatine kinase values cannot be used
to differentiate DMD from BMD. Calf enlargement and the presence of Gowers’ sign are a
nonspecific findings. Studies have shown significant overlap in the observed age at onset between
DMD and BMD
- The leading cause of childhood disability is
(a) traumatic brain injury.
(b) spinal muscular atrophy.
(c) spina bifida.
(d) cerebral palsy.
(d) Cerebral palsy is the leading cause of childhood disability. The reported incidence is approximately
2-3 per 1,000 live births. The incidence of spina bifida is .5 per 1,000, of spinal muscular atrophy 1
in 25,000. The annual incidence for traumatic brain injury in children is 1-2 per 1,000. However,
the great majority of cases are minor and result in no long-term disability. Approximately 15% of
brain-injured children have moderate and severe injuries resulting in permanent impairment.
- The earliest weakness seen in skeletal muscle in Duchenne muscular dystrophy is located in
(a) knee extensors.
(b) hip flexors.
(c) neck flexors.
(d) ankle plantar flexors.
(c) Neck flexor weakness occurs during preschool years. Weakness is generalized but is predominantly
proximal early in the disease course. Pelvic girdle weakness precedes shoulder girdle weakness by
several years. Weakness progresses steadily. Quantitative strength testing is more sensitive than
manual muscle testing.
- The most common complication after amputation in the immature child is
(a) phantom limb pain.
(b) diffuse edema.
(c) terminal overgrowth.
(d) painful neuroma.
(c) Terminal overgrowth at the transected end of a long bone is the most common complication after
amputation in the skeletally immature child. It occurs most frequently in the humerus, fibula, tibia,
and femur, in that order. The oppositional growth may be so vigorous that the bone pierces the
skin. The treatment of choice is surgical revision.
- In children hospitalized with acute burns, early management should include
(a) avoidance of sedation.
(b) positioning for comfort to reduce severe pain.
(c) use of a pressure garment over areas of full-thickness burns.
(d) administration of narcotics and anesthetic agents.
(d) Although opiates should be considered the most important part of acute pain management
nonopiates should be used when possible. As the needs become more chronic, other agents should
be instituted to minimize the problems seen with opiates. Behavioral management and relaxation
therapy should also be used when possible. Typically, the position of comfort for a burned child is
the position that promotes deformity and, therefore, should be avoided. Garments are fitted later in
the course of treatment.
- In considering selection of a lower limb prosthesis for a child with a congenital transfemoral
amputation, a knee joint should be included
(a) at initial fitting.
(b) between 3 to 5 years of age.
(c) when sports activities are anticipated.
(d) when the child pulls to stand.
(b) The lower limb deficient child should be fitted with a prosthesis when he or she is ready to pull up
to a standing position, usually between 9 and 12 months. A knee joint is added between 3 and 5
years.
- The earliest marker of abnormal central nervous system maturation is
(a) diffuse fasciculations.
(b) gross motor delay.
(c) delay of postural responses.
(d) persistence of primitive reflexes.
(d) In neonates and young infants, motor behavior is influenced by primitive reflexes because of the
immature central nervous system. These reflexes gradually become suppressed. Concurrently, more
sophisticated postural responses emerge. Obligatory persistent primitive reflexes are the earliest
markers of abnormal neurologic maturation.
- A parent of an 18-month-old child reports that the child babbled as an infant but became much
quieter after about 8 months of age. She has no true words, though she will wave bye-bye. She
follows no verbal commands but will follow occasional pantomime commands. Her gross and fine
motor skills have been normal. The most likely diagnosis is
(a) autism.
(b) mental retardation.
(c) hearing impairment.
(d) oral motor apraxia.
(c) A history of delay in communication development raises several diagnostic possibilities, including
true language dysfunction or a motor dysfunction or significant hearing loss. Infants with hearing
loss start to fall behind after 6-8 months of age, when learning of auditory-dependent vocalization
begins. Oral motor dysfunction is often associated with cerebral palsy, most often spastic
quadriparesis. Difficulty with drinking from a cup and difficulty with the introduction of solid food
are early symptoms of oral motor dysfunction. Autism is a spectrum disorder with qualitative
abnormalities in communication and in social and behavioral realms.
- The most severe form of mental retardation in cerebral palsy occurs in association with
(a) spastic diplegia.
(b) ataxia.
(c) spastic quadriplegia.
(d) athetosis.
(c) Mental retardation is the most common serious associated disability in cerebral palsy. The overall
incidence of mental retardation is approximately 30%-50%. Severe mental retardation is present in
about one-half of the retarded group. Approximately one-third of cases have mild cognitive
deficits. The greatest retardation is seen in rigid, atonic, and severe spastic quadriplegic cerebral
palsy.
- In traumatic brain injury in children, outcome is primarily related to
(a) severity of original injury.
(b) location of injury.
(c) age at time of injury.
(d) associated injuries.
(a) Although there is considerable variability from case to case, outcome is primarily related to the
severity of the injury.
