What better way to board review? Jeopardy Template

Rheumatology

SCI/Spina Bifida

TBI

Potpourri

100

Which of the following juvenile rheumatoid arthritis (JRA) diseases carries the worst prognosis?

A Polyarticular, RF+

B Polyarticular, RF-

C Pauciarticular, RF+

D Pauciarticular, RF-

Polyarticular, RF+ JRA carries the worst prognosis and should be treated early.

100

A child with C5 ASIA A spinal cord injury should eventually become independent in which activity?

(a) Intermittent catheterization

(b) Transfer to level surfaces

(d) Bathing

A child with C5 ASIA A spinal cord injury should eventually become independent in feeding, and in upper extremity dressing with assistive devices, in driving a power wheelchair, and in propelling a manual wheelchair short distances on level surfaces.

100

Which of the following is NOT a feature of central autonomic dysfunction in traumatic brain injury in children?

(a) Hypertension

(b) Tachypnea

(d) Hypothermia

(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.

100

The most common movement disorder associated with cerebral palsy is:

(a) spasticity

(b) dystonia

(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.

100

A patient with a history of coarctation of the aorta presents to your clinic with the chief complaint of shoulder pain. On exam you detect a webbed neck and broad chest. This patient’s karyotype is most likely which of the following?

Turner syndrome involves a 45,X karyotype, webbed neck, shield chest, and coarctation of the aorta. Down syndrome is trisomy 21 with upward slanting eyes, “simian” crease, cardiac disease (e.g. endocardial cushion defects). Klinefelter syndrome involves a male presenting with tall stature, gynecomastia, and small testicles, with an 47,XXY genotype.

200

A patient with sickle cell disease who has experienced chronic episodic left hip/groin pain will most likely suffer from which of the following pathologies later in his life?

A Femoroacetabular impingement

B Greater trochanteric bursitis

C Hip avascular necrosis (AVN)

D Hip labral tear

AVN is a common sequela of chronic sickle cell disease, notably in the hip joint.

200

A 13 year-old female with a history of myelomeningocele at L3-L4 presents for routine checkup. She notes worsening back pain over the past few weeks, and more frequent episodes of urinary incontinence. She denies trauma, fever, or chills. On exam you notice abnormal posture (not documented previously) and knee extension strength is 2/5, but the previous note documents antigravity strength. Which of the following is the most reasonable next step?

This patient with spina bifida presents with acute neurologic worsening (progressive weakness, worsening bowel/bladder function, worsening pain), suspicious for tethered cord. MRI of lumbar spine is necessary along with a neurosurgery consultation for cord detethering surgery. A urinary tract infection would not explain worsened leg strength, worsened posture/scoliosis, and worsened bowel/bladder function (typically bladder function alone, possibly with flank pain, would be suspected in urinary tract infection, along with potential fever/chills).

200

What is the most common cause of traumatic brain injury in a child under the age of 1 year?

(a) Motor vehicle crash

(b) Near drowning in bath

(d) Fall from changing table

For infants, more than two-thirds of all traumatic brain injuries result from falls; only 8% of these result in moderate or severe injuries.

For preschool children, falls account for 51% of TBI and motor vehicle crashes for 22%.

For children 8 to 9 years of age, etiology of TBI is evenly divided between falls, sports, and recreational activities, and motor vehicle crashes.

*Leading cause of severe TBI ages 0-4 is inflicted injuries

200

Which anti-spasticity 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: Clonidine is an alpha 2 agonist, as is tizanidine

(b) Tizanidine

(d) Baclofen: binds to GABA receptors in the spinal cord to inhibit the reflexes that lead to increased tone.

Baclofen can be used beginning at age 2 years.

200

A 15 year-old male with a history of obesity presents with sudden-onset left hip pain. He denies trauma. Hip x-rays demonstrate a widened growth plate and femoral head sliding off the growth plate by approximately 20%. Which of the following is the most appropriate treatment for this problem?

Slipped capital femoral epiphysis (SCFE) involves sudden-onset hip/groin pain due to the femoral head sliding off the growth plate, typically in obese adolescent males. X-ray grading is defined by the percentage of slippage of the femoral head off the growth plate.

Grade 1: 0-33% sliding

Grade 2: 34-50% sliding

Grade 3: >50% sliding

There is no grade 4. Treatment is orthopedic surgery.

300

Juvenile rheumatoid arthritis (JRA) differs from adult onset rheumatoid arthritis: in JRA

(a) joint destruction occurs earlier
(b) large joint involvement is less frequent
(c) the cervical spine is involved less frequently
(d) systemic features are more common

(d) Children with juvenile rheumatoid arthritis are more likely to have systemic features, have large joints involved, and have cervical spine involvement. Adults with rheumatoid arthritis have joint destruction earlier.

300

A 9-year-old girl with an L1 ASIA class A spinal cord injury that occurred at age 5 years presents in your office with a 1-day history of a swollen left leg. History is that she woke up with the swollen leg the day before. There is no history of trauma, fever, or shortness of breath. On examination, you find a prepubertal girl in no distress with normal vital signs. Upper extremities are normal. Lower extremities have moderate spasticity and no voluntary movement. Skin is normal. The left leg is
warm and swollen from the ankle to the knee. There is no sensation in the legs. Which test is most likely to yield the correct diagnosis?

(a) Bone scan
(b) Plain radiograph
(c) Venous Doppler study
(d) White blood cell count with differential

(b) Deep venous thromboses (DVTs) which can be diagnosed by Doppler study usually occur in the first 3 months after spinal cord injury (SCI) and are rare in prepubertal children. In lower leg DVTs
the foot and leg are usually swollen. Heterotopic ossification (HO), which can be detected by bone scan, occurs in about 3% of children with SCI and has onset an average of 14 months after injury.
Heterotopic ossification most commonly involves the hip. Cellulitis is usually associated with skin lesions and usually involves a discrete area. A fracture is the most likely cause of swelling in this case and can be diagnosed by plain radiographs.

300

In traumatic brain injury in children, outcome is primarily related to:

(a) severity of original injury

(b) location of injury

(d) associated injuries

(a) Although there is considerable variability from case to case, outcome is primarily related to the severity of the injury

300

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?

Grade 4 intraventricular hemorrhages in premature infants are most commonly associated with spastic diplegia

(a) Athetoid - kernicterus

(b) Hemiplegic - intrauterine stroke

(d) Hypotonic - genetics?

(e) Quadriplegic - HIE

300

Which muscle group displays the earliest pattern of weakness in Duchenne muscular dystrophy?

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.

400

A 33 year-old male presents with right ankle pain and heel pain over the achilles tendon. He also endorses pain with urination. Which of the following etiologies is most likely responsible?

A Chlamydia

B Calcium pyrophosphate deposition

C Uric acid deposition

D Neisseria gonorrhea

Reactive Arthritis presents with asymmetric arthritis affecting especially the feet and ankles as well as enthesitis (e.g. achilles tendon pain). It also presents with urethritis and uveitis. This constellation of symptoms is “reactive” to an infection, typically chlamydia, campylobacter, or salmonella. Treatment involves antibiotics, NSAIDs, and physical therapy.

400

A 6 month-old female is found to have myelomeningocele at the S1 level. Which of the following is most likely to be true?

This patient will theoretically have normal myotomal function through L5, but disrupted function at the S1-S5 levels. The S1-S2 myotomes control plantarflexion and intrinsic foot muscle function, but spares the quadriceps, hamstrings, and other L2-L5 muscles. Thus, KAFO is not needed, but AFO would be useful to stabilize the ankle and allow for modified independent ambulation. Bowel/bladder dysfunction is expected to occur; for example, the parasympathetic and somatic control of the bladder arises from the sacral nerve roots.

400

Which endocrine abnormality is most likely to occur 5 years after severe traumatic brain injury in a 2-year-old girl?

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.

400

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

(d) Wheelchair use in the community

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

400

A 13 year-old male presents with back pain. He denies trauma. On exam you notice a forward-flexed posture. Spine x-rays demonstrate thoracic kyphosis of 50 degrees with anterior vertebral body wedging and Schmorl nodes in multiple segments. Which of the following is the most likely diagnosis?

A Friedreich ataxia

B Becker muscular dystrophy

C Scheuermann disease

D Idiopathic scoliosis

This patient presents with Scheuermann disease, involving idiopathic juvenile kyphosis which can lead to a restrictive lung pattern. X-rays show kyphosis, vertebral body wedging, and Schmorl nodes. Treatment is physical therapy, bracing, and surgery. Scoliosis would show a coronal plane curvature, not simply kyphosis. Becker muscular dystrophy would also present with gradual onset weakness and disability. Friedreich ataxia would also demonstrate weakness, disability, cardiomyopathy, and vision and hearing dysfunction.

500

Which of the following juvenile rheumatoid arthritis (JRA) subtypes requires ophthalmology referral?

A Systemic

B Pauciarticular

C Polyarticular, RF-

D Polyarticular, RF+

Pauciarticular JRA requires ophthalmology referral, as this subtype is associated with iridocyclitis/uveitis.

500

Urinary incontinence occurs in 95% of persons with spina bifida. Hydronephrosis with reflux is most likely to occur with:

a. disturbed bladder sensations

b. inadequate outflow resistance

c. over distended hypotonic bladder

d. high-pressure bladder

Hydronephrosis occurs with reflux more often in the high-pressure bladder. Intravesical pressure over 40 cm H2O was found to occur in upper tract dilation in 81% of children with spina bifida. Deteriorating kidney function is the eventual consequence of hydronephrosis and infection. Renal function is further complicated in the presence of malformed or solitary kidneys, which are common in children with spina bifida.

500

Secondary injury in pediatric brain trauma is caused by

(a) hypotension, hypoxia, and hydrocephalus.

(b) growing skull fractures

(d) diffuse axonal injuries and punctate hemorrhages

Any disorder that interferes with cerebral perfusion or oxygenation can cause further damage following traumatic brain injury. This includes hypotension, hypoxia, increased intracranial pressure because of cerebral edema, acute hydrocephalus, or space-occupying lesions. Midline shift or herniation may lead to infarction because of pressure or traction on cerebral vessels. Therefore, efforts are made to control intracranial pressure through fluid and electrolyte management, hyperventilation, and maintenance of normal blood pressure and oxygenation.

Growing skull fractures result from the arachnoid protruding through a dural tear, producing a cyst that can contribute to a widening skull deficit, which usually requires operative repair. This is a complication of traumatic brain injury but not a secondary injury.

Coup and contra-coup cerebral contusions and diffuse axonal injuries are examples of primary injury.

500

Which of the following is the best indicator of good ambulation prognosis in a child with cerebral palsy?

The ability to sit independently by 2 years of age is the best indicator of good ambulatory prognosis in a patient with cerebral palsy, and is high-yield to know regarding ambulation prognosis in patients with cerebral palsy.

500

Which pulmonary parameter is predictive of mortality in a child with Duchenne muscular dystrophy?

(a) Maximal expiratory pressure

(b) Peak flow rate

(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.