SAE & Board Review > Pediatrics > Flashcards

Pediatrics Flashcards

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1
Q
  1. 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
A
  1. (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.
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2
Q
  1. 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
  1. (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.
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3
Q
  1. 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
A
  1. (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.
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4
Q
  1. 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
  1. (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.
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5
Q
  1. 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
A
  1. (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.
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6
Q
  1. 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.
A
  1. (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.
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7
Q
  1. 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
A
  1. (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.
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8
Q
  1. 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
  1. (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.
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9
Q
  1. 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.
A
  1. (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.
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10
Q
  1. 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
A
  1. (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.
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11
Q
  1. 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
A
  1. (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.
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12
Q
  1. 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
A
  1. (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.
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13
Q
  1. 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
  1. (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.
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14
Q
  1. In which type of cerebral palsy is a seizure disorder most commonly seen?
    (a) Tetraplegia
    (b) Diplegia
    (c) Athetosis
    (d) Hemiplegia
A
  1. (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.
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15
Q
  1. 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
  1. (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.
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16
Q
  1. 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
A
  1. (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.
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17
Q
  1. The most common spinal problem seen with achondroplasia during childhood is
    (a) kyphosis.
    (b) scoliosis.
    (c) spinal stenosis.
    (d) low back pain.
A
  1. (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.
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18
Q
  1. 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
A
  1. (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.
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19
Q
  1. 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
  1. (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.
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20
Q
  1. 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
A
  1. (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.
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21
Q
  1. Which of the following is part of neurodevelopmental therapy (NDT)?
    (a) Promotion of primitive reflexes
    (b) Use of taping and icing
    (c) Strengthening exercises
    (d) Facilitating automatic reactions
A
  1. (d) Neurodevelopmental therapy, developed by Bobath, emphasizes inhibition of reflex patterns,
    normalizing tone, and facilitating automatic reactions. The therapy does not include strengthening
    exercises.
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22
Q
  1. 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)
A
  1. (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.
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23
Q
  1. 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.
A
  1. (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.
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24
Q
  1. 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
A
  1. (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.
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169. 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
169. (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.
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189. 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
189. (c) Grade 4 intraventricular hemorrhages in premature infants are most commonly associated with spastic diplegia.
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199. 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
c?
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19. 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.
19. (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.
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49. 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
49. (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.
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69. 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.
``` 69. (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. ```
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99. A normal 6-month-old infant may demonstrate which reflex? (a) Rooting (b) Automatic walking (c) Plantar grasp (d) Posterior protective extension
99. (c) 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. Reflex Age of Emergence Age of Suppression (or Integration) Moro Birth 4–6 months Rooting Birth 4 months Asymmetric tonic neck reflex (ATNR) 1–3 months 6–7 months Plantar grasp Birth 12–14 months–ie, when walking well Automatic walking Birth 3–4 months Posterior protective extension 7–8 months
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109. 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.
109. (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.
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129. 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).
129. (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.
34
149. 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.
149. (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.
35
169. Once an individual becomes board-certified in the specialty of physical medicine and rehabilitation, he/she must continue to fulfill certain requirements in order to maintain certification status. Which action is NOT a requirement for maintenance of certification? (a) Continuing medical education credits (b) Maintenance of active medical licensure (c) Completion of a recertification examination every 10 years (d) Publication of at least 1 article in a scientific journal every 10 years
169. (d) Once an individual becomes board certified in the specialty of physical medicine and rehabilitation, he/she must continue to fulfill certain requirements in order to maintain their certification status. Publication of 1 article in a peer-reviewed journal every 10 years is not a requirement for maintenance of certification. All of the other options listed are required.
36
189. You have just finished admitting a 60-year-old diabetic man who has recently undergone a right below-knee amputation. The patient's son stops you in the hallway and inquires about his father's health status and prognosis for walking again. You have never met the patient's son before, and before answering the questions, you would first (a) further review the patient's medical record and determine his cardiac status. (b) perform a literature review of outcomes research in individuals with below-knee amputations. (c) ask the patient for permission to discuss his health status with his son. (d) ask the son if the patient has a living will or a health care power-of-attorney.
189. (c) Maintaining confidentiality of patient information is important even when discussing health information with family members. Before discussing the patient's health status with his son, the appropriate first step would be to ask the patient for permission. The other options listed would not be appropriate initial management strategies.
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19. 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)
19. (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.
38
59. 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.
59. (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.
39
69. 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.
69. (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.
40
89. The most common congenital limb deficiency is a (a) transverse tibial and fibular limb deficiency (below-knee limb deletion). (b) transverse transmetacarpal limb deficiency (partial hand deletion). (c) longitudinal fibular deficiency (fibular hemimelia). (d) transverse radial limb deficiency (below-elbow limb deletion).
89. (d) The left short transradial congenital limb deficiency (below-elbow limb deletion) is the most common congenital limb deficiency. It is thought to be caused by a clot which occludes the artery, resulting in resorption of the distal limb, often leaving nubbins of fingers at the end of the stump.
41
109. 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
109. (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 in a child who had a TBI at age 5 may not be seen until the child reaches 9 or 10 years of age
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129. 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
``` 129. (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. ```
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149. 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.
149. (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.
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9. According to national databases of spinal cord injury (SCI), children under the age of 6 years are more likely to have which epidemiologic pattern of spinal cord injury? (a) high tetraplegia, motor incomplete, occurred in motor vehicle accident (b) paraplegia, complete, occurred in motor vehicle accident (c) high tetraplegia, complete, caused by medical/surgical complications (d) paraplegia, motor incomplete, caused by medical/surgical complications
9. (b) According to databases of the Model SCI Systems and Shriner’s Hospital for Children, children under age 6 years are more likely than teenagers to sustain SCI in a motor vehicle accident. Their injuries are more likely to be T1 and lower, and they are more likely to have complete injuries.
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49. 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
49. (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.
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99. On examination, a 3-month-old girl still has a Moro reflex, asymmetric tonic neck reflex, and plantar grasp reflex. She does not have any protective extension. You advise her parents that (a) further diagnostic evaluation is indicated. (b) she requires a physical therapy evaluation. (c) she needs a neurology evaluation. (d) these reflexes are normal reflexes.
(d) 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. Ref: Molnar GE, Sobus KM. Growth and development. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 20.
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109. Which of the following is NOT a feature of central autonomic dysfunction in traumatic brain injury in children? (a) Hypertension (b) Tachypnea (c) Rigidity (d) Hypothermia
109. (d) Central autonomic dysfunction occurs in some children following severe brain injury. It is characterized by hypertension, hyperpyrexia, rigidity, tachypnea, tachycardia, and diaphoresis. Various medications are used to treat this dysfunction, but no studies prove the value of one medication over another.
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139. 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
139. (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.
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149. 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.
149. (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.
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9. 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
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. Ref: Molnar GE, Sobus KM. Growth and development. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 20.
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59. Acquired subluxation or dislocation of the hips in spastic cerebral palsy is usually due to muscular imbalance with excessive tone of which muscles? (a) Hip flexors and tensor fascia lata (b) Hip flexors and hip adductors (c) Rectus femoris and hip abductors (d) Tensor fascia lata and hip extensors
59. (b) Progressive changes associated with hip subluxation in patients with cerebral palsy result from the effects of neuromuscular imbalance on the growth and development of the hip joint. The primary problem is spasticity and muscular imbalance, and the musculoskeletal manifestations are secondary. Soft tissue abnormalities include a muscular imbalance between the stronger flexors and adductors, and the weaker extensors and abductors. A flexion-adduction contracture also shifts the center of rotation of the hip from the femoral head to the lesser trochanter, and the proximal femur is gradually displaced upward and outward. Ref: Spiegel DA, Flynn JM. Evaluation and treatment of hip dysplasia in cerebral palsy. Orthopedic Clinics of North America 2006;37(2):185-96.
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69. 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
69. (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. Ref: Damiano DL, Dodd K, Taylor FT. Should we be testing and training muscle strength in cerebral palsy? Dev Med Ch Neurol 2002;44:68-72.
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79. 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. Ref: Smith C, Hill J. Language development and disorders of communication and oral motor function. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999.p 72-8.
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139. 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
139. (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. Ref: Lubicky JP, Betz RR. Spinal deformity in children and adolescents after spinal cord injury. In: Betz RR, Mulcahey MJ, editors. The child with a spinal cord injury. Rosemont (IL): American Academy of Orthopedic Surgeons; 1996. p 363-70.
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149. The most common congenital limb deficiency is (a) right transtibial limb deletion. (b) right transradial limb deletion. (c) left transtibial limb deletion. (d) left transradial limb deletion.
The most common congenital limb deficiency is the left midlength transradial deficiency. Ref: Gaebler-Spira D, Uellendahl J. Pediatric limb deficiencies. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 334.
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59. 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.
59. 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. Ref: Krach LE, Kriel RL. Traumatic brain injury. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 262.
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79. Which maternal factor is associated with an increased risk of spina bifida? (a) Anticonvulsant medications during pregnancy (b) Upper socioeconomic class (c) Alcohol ingestion during pregnancy (d) Folic acid 4mg/day prior to and during pregnancy
``` Answer: A Commentary: The etiology of spina bifida is multifactorial. Both polygenic inheritance and environmental influences contribute. Several studies have shown that there the incidence of spina bifida is reduced if food is fortified with folic acid or if mothers take folic acid prior to conception and during pregnancy. Recommended doses of folic acid are 0.4 mg/day in women who are not at high risk and 4 mg /day in women at high risk (eg, those with a family history of spina bifida). Some studies have also implicated lower socioeconomic class and in utero exposure to anticonvulsant medications as being risk factors. Maternal alcohol ingestion is not related to an increased risk of spina bifida in a baby. Ref: Molnar GE, Murphy KP. Spina bifida. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 219. ```
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99. 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.
3rd ed. Philadelphia: Hanley & Belfus; 1999. p 219. 99 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. Ref: Molnar GE, Murphy KP. Spina bifida. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 222.
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109. 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.
109. 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. Ref: Molnar GE, Murphy KP. Spina bifida. In: Molnar GE, Alexander MA, editors. Pediatric rehabilitation. 3rd ed. Philadelphia: Hanley & Belfus; 1999. p 237-8.
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119. Which statement is true about the asymmetric tonic neck reflex (ATNR) or the symmetric tonic neck reflex (STNR)? (a) The STNR is present at birth and fades away by 1 year. (b) The STNR provides postural stability as the child goes from crawling to standing. (c) The ATNR appears about 6 months of age and fades away by 1 year. (d) The ATNR is obligatory in all children at certain ages.
119 Answer: B Commentary: The ATNR is also known as the fencer position and is a neonatal reflex that disappears by 6 months of age. It is never obligatory in normal children. The STNR appears about 6 months and disappears by 1 year. It provides postural stability as the child makes the transition from crawling to standing. Ref: Pidcock FS, Christensen JR, Trovato MK. General concepts of pediatric rehabilitation. In: O’Young BJ, Young MA, Stiens SA, editors. Physical medicine and rehabilitation secrets. 3rd ed,Philadelphia, Mosby Elsevier; 2007. p 657.
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129. 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
no answer
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39. 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
39. 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. Ref: Strombeck C, Krumlinde-Sundholm L, Remahl S, Sejersen T. Long-term follow-up of children with obstetric brachial plexus palsy I: functional aspects. Dev Med Child Neurol 2007;49:198-203.
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59. When should one be concerned when observing a child with an asymmetric tonic neck reflex (ATNR,“fencer” position) with neck rotation, relative extension of the limbs on the chin side and flexion of the limbs on the occiput side? (a) In a 3-month-old infant who is able to move out of the “fencer” position (b) In a 5-month-old infant who is unable to move out of the “fencer” position (c) In a 6-month-old infant whose leg response to the stimulus is greater than the arm’s (d) In an infant of any age, since the ATNR is a primitive pathological reflex.
59. Answer: (b) Commentary: The ATNR is typically present at birth and integrates between 4 and 6 months of age. An obligatory “fencer” position is abnormal at any age. A persistent or obligatory ATNR may be an early clue that a child has a disorder of motor control, most often cerebral palsy. Ref: Carpenter DL, Batley RJ, Johnson EW. Developmental evaluation of infants and children. Phys Med Rehabil Clin N Am 1996;7 561-82.
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69. 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.
69 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. Ref: Han JJ, McDonald CM. Diagnosis and clinical management
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109. 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.
109. 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. Ref: Henderson RC, Kairalla JA, Barrington JW, Abbas A, Stevenson RD. Longitudinal changes in bone density in children and adolescents with moderate to severe cerebral palsy. J Pediatr 2005;146:769-75.
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119. 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
119. 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. Ref: Reece RM, Sege R. Childhood head injuries: accidental or inflicted? Arch Pediatr Adolesc Med 2000;154:11-5.
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9. 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
9. 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.
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39. Which sign is associated with central dysautonomia following severe traumatic brain injury? (a) Flaccidity (b) Hyperthermia (c) Hypotension (d) Bradycardia
39. Answer (b) Commentary: Central dysautonomia can occur acutely after severe traumatic brain injury. It has also been called diencephalic seizures, autonomic or neuro storming or hypothalamic dysregulation syndrome. Signs include elevated temperature with a normal fever work up, tachycardia, elevated blood pressure, rapid respiratory rate and posturing. Facial flushing and diaphoresis may also be seen. Reference: Krach LE, Gormley ME, Ward M. Traumatic brain injury. In: Alexander MA, Matthews DJ. Pediatric rehabilitation: principles and practice. 4th ed. New York: Demos;2010. p 244.
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49. 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.
49. 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. Reference: a) Blasco PA, Allaire JH. Drooling in the developmentally disabled: management practices and recommendations. Consortium on Drooling. Dev Med Child Neurol 1992;34:849- 862. (b) Jongerius PH, van Limbeek J, Rotteveel JJ. Assessment of salivary flow rate: biological variation and measure error. Laryngoscope 2004;114:1801-4. (c) Mirrett PL, Riski JE, Glascott J, Johnson V. Videofluoroscopic assessment of dysphagia in children with severe spastic cerebral palsy. Dysphagia 1994;9:174-9.
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99. Risk factors for osteoporotic long bone fractures in children with cerebral palsy include (a) spastic hemiplegia. (b) adequate oral nutrition. (c) vitamin D supplementation. (d) decreased mobility.
99. Answer (d) Commentary: Fracture risk is shown to increase with decreased mobility, nutrition via gastrostomy tube and tetraplegia. Nutritional status is also linked to low bone mineral density (BMD). Deficiencies in vitamin D, calcium, folate, iron and magnesium have been found in children with cerebral palsy. Vitamin D and calcium are especially important in maintaining bone mineral density (BMD). Reference: (a) Henderson RC, Kairalla J, Abbas A, Stevenson RD. Predicting low bone mineral density in children and young adults with quadriplegic cerebral palsy. Dev Med Child Neurol 2004;46:416-419. (b) Henderson RC, Linn PP, Greene WB. Bone-mineral density in children and adolescents who have spastic cerebral palsy. J BoneJoint Surg 1995;77:1671-81. (c) Apkon SD, Kecskemethy HH. Bone
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109. The most common movement disorder associated with cerebral palsy is (a) spasticity. (b) dystonia. (c) athetosis.
109. 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. Reference: Murphy N, Such-Neibar T. Cerebral palsy diagnosis
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119. Intrathecal baclofen decreases spasticity by what mechanism? (a) It blocks Ia afferent signals through the dorsal root ganglia. (b) It acts as a GABA agonist to inhibit gamma motor neuron activity and decrease muscle spindle sensitivity to spinal reflexes. (c) It blocks acetylcholine release from neurons at the peripheral neuromuscular junction. (d) It inhibits calcium release from the sarcoplasmic reticulum during muscle contraction.
119 Answer: (b) Commentary: Intrathecal baclofen acts as a GABA agonist inhibiting the spinal reflex arc stimulated by intrafusal muscle fiber stretch. Selective dorsal rhizotomy seeks to diminish spasticity by decreasing the afferent signal by cutting rootlets in the dorsal root ganglia. Botulinum toxin blocks acetylcholine release at the neuromuscular junction. Dantrolene sodium decreases muscle contraction by inhibiting calcium release from the sarcoplasmic reticulum. All these therapeutics can be useful in the treatment of spasticity. Reference: (a) Grabb PA, Doyle JS: The contemporary surgical management of spasticity in children. Phys Med Rehabil Clin N Am 2001;12(4):907-22. (b)Yablon S. Botulinum neurotoxin intramuscular chemodenervation: role in the management of spastic hypertonia and related motor disorders. Phys Med Rehabil Clin N Am 2001;12(4):833- 74. (c) Hinderer SR, Dixon K. Physiologic and
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139. The most common etiology of cerebral palsy is (a) premature birth. (b) birth asphyxia. (c) intrauterine stroke. (d) prenatal infection.
139. 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%. Reference: McMahon M, Pruitt D, Vargus-Adams J. Cerebral palsy. In: Alexander MA, Matthews DJ. Pediatric rehabilitation: principles and practice. 4th ed. New York: Demos;2010. p 244.
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9. 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.
9. 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. Reference: Love SC, Novak I, Kentish M, Desloovere K, Heinen F, Molenaers G. Botulinum toxin assessment, intervention and after-care for lower limb spasticity in children with cerebral palsy: international consensus statement. European J Neurol 2010; 17 (Suppl 2): 9-3
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19. 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
19. Answer: (d) Commentary: Baclofen binds to GABA receptors in the spinal cord to inhibit the reflexes that lead to increased tone. Clonidine is an alpha 2 agonist, as is tizanidine. Dantrolene works in the striated muscle at the level of the sarcoplasmic reticulum. All these drugs have pediatric application. Baclofen can be used beginning at age 2 years. Reference: McMahon M, Pruitt D, Vargus-Adams J. Cerebral palsy. In: Alexander MA, Matthews DJ, editors. Pediatric rehabilitation:
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79. Which ethnicity has the highest prevalence of neural tube defects? (a) Eastern European (b) Asian (c) African American (d) Hispanic
79. 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. Reference: Pico EL, Wilson PE, Haas R. Spina bifida . In: Alexander MA, Matthews DJ, editors. Pediatric rehabilitation: principles and practice. 4th ed. New York: Demos; 2010. p 199, 201.
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99. 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)
99. 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. Reference: Pico EL, Wilson PE, Haas R. Spina bifida. In: Alexander MA, Matthews DJ, editors.
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139. 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
139. 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. Reference: Pico EL, Wilson PE, Haas R. Spina bifida . In: Alexander MA, Matthews DJ, editors. Pediatric rehabilitation: principles and practice. 4th ed. New York: Demos; 2010. p 215-216.
SAE & Board Review
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