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.
