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PM&R Pediatric Boards > A2 Neurologic Disorders: Brain 0-100 > Flashcards

A2 Neurologic Disorders: Brain 0-100 Flashcards

1
Q

Neuroimaging in CP

A
  1. 90% abnormal
  2. Most commonly prenatal
  3. Destructive 90% (normal brain insulted with PVL or deep grey matter injury)
  4. Brain Mal-development 10%
  5. Normal Neuroimaging 10%
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2
Q

When do babies sit

A

7 - 8 months

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3
Q

What are the symptoms of ADEM (Acute Disseminated Encephalomyelitis)?

A

Weakness
Loss of vision
Numbness
Loss of Balance

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4
Q

When does a child run, do stairs, and demonstrate hand dominance

A

2 years

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5
Q

What is the largest group of CNS pediatric tumors

A

Low grade astrocytoma

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6
Q

with neurologic structure degenerates in SMA?

A

Anterior Horn Cells

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7
Q

When does a baby walk with help

A

12 months

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8
Q

Complications of spina bifida

A
  1. Hydrocephalus
  2. Tethered cord
  3. Syrinx
  4. Arnold-Chiari II
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9
Q

What is GMPM?

A

The GMPM is an observational instrument used to evaluate gross motor performance (i.e., quality of movement) in children with cerebral palsy. The instrument’s primary purpose is to evaluate change over time in specific qualitative features or attributes of gross motor behaviour. The measure was found to be responsive to change over time.

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10
Q

3 Postnatal risk factors for CP

A

Trauma
Infection
Coagulopathies (sickle cell, clotting deficiencies, etc.)

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11
Q

Differential diagnosis of ADEM

A 
Tumors
MS
Direct infection
LP shows lymphocytes
No oliclonal bands (Seen in. MS)
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12
Q

List 3 types of neural tube defects:

A
  1. Encaphalocele
  2. Anencephaly
    3,. Spina Bifida
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13
Q

Symptoms of pediatric CNS tumors

A 
Sudden onset headache
Nausea and vomiting
Ataxia
Cranial Nerve Palsy
Impaired vision
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14
Q

What is the GMFM

A

The Gross Motor Function Measure (GMFM) is an assessment tool designed and evaluated to measure changes in gross motor function over time or with intervention in children with cerebral palsy. It was first developed in the late 1980s for use in both clinical and research settings and has evolved through advanced analytic techniques and in response to requests for more efficient testing. The GMFM user’s manual is available for purchase at the Mac Keith Press website.

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15
Q

4 Perinatal risk factors for CP

A

Prematurity
Breech
Birth complications (placental detachment, cord, uterine rupture)
Birth asphyxia (<10%)

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16
Q

What is used for DXA scan measure for children

A

z-score
Not T-score
Distal Femur

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17
Q

What is the life expectancy of a child with CP who can’t lift his head up from supine

A

<20% norm

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18
Q

Describe clubfoot with spina bifida

A

Common
Early casting helpful
Surgery helpful

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19
Q

What is the life expectancy for independently ambulatory children with CP compared to their peers?

A

Same

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20
Q

What is the trend in Prevalence of CP

A

Decreasing in Preterm
Stable in term
Combined stable

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21
Q

How does baclofen work?

A

Chemically, baclofen is a derivative of the neurotransmitter γ-aminobutyric acid (GABA). It is believed to work by activating (or agonizing) GABA receptors, specifically the GABAB receptors. Its beneficial effects in spasticity result from its actions in the brain and spinal cord

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22
Q

4 factors of hip dislocation in spina bifida

A

FILL OUT

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23
Q

When can a child ride a trike, be toilet trained, speak a 3 work sentence, and draw a circle

A

3 years

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24
Q

Pediatric CNS tumors

A

Most common solid tumors in children. Leading cause of cancer death ages 0-14.
Rate 5-6.2/100,000 0-14
Higher in males

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25
Q

Name 3 types of Spina Bifida

A
  1. Myelomeningocele
  2. Meningocele
  3. Occulta
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26
Q

Name 4 lower extremity functional measures for CP

A
  1. Instrumented and clinical gait parameters
  2. Gross Motor Function Measure (GMFM)
  3. GMPM
  4. 6 minutes walk test
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27
Q

What is the z-score in DXA scans for Osteoporosis in children

A

z-score <2.0

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28
Q

When do babies babble

A

6 - 10 months

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29
Q

Risk factors for poor bone health in CP

A
  • nutrition and absorption
  • decreased exposure to sun
  • non-ambulatory
  • anti-seizure meds
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30
Q

Etiology of pediatric stroke`

A 
Cardiac
Vasculoar
Sickle Cell
Infection
Trauma
Genetics
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31
Q

what gene diminishes neuronal functional survival in SMA?

A

SMN1 (Survival Motor Neuron 1)
On chromosome 5q13
SMN protein essential for anterior horn cell survival

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32
Q

When can a child use roller skates and recite the alphabet

A

6 years

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33
Q

When does head control develop

A

4 months

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34
Q

What is the PEDI test/scale?

A

The PEDI (Pediatric Evaluation of Disability Inventory), is an interview-based assessment that can be used to monitor the self-care, mobility and social abilities of a person with cerebral palsy. In this assessment, the parent or care giver answers questions about the person’s performance in these aspects of life. There are two versions of this assessment widely used in clinical practice today

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35
Q

Pediatric Glascow coma scale

A

Less than 5 is the cut-off for pediatric scale (compares for 8 with adults)

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36
Q

Risk factors for hip migration in CP

A 
Malalignment (valgus neck shaft angle)
Femoral anteversion
Decreased weight-bearing
Spasticity
Weakness of hip abductors and extensors
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37
Q

When can a child hop, use a pencil, use scissors, and dresses

A

4 years

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38
Q

What is more predictable of function in Spina Bifida

a. Surgical anatomy
b. Exam and motor capacity

A

b,

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39
Q

Prenatal screen for spina bifida

A

increased alphafetoprotein at 16-18 months

Ultrasound findings at 18-22 weeks

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40
Q

Dose of folate for prevention of spina bifida in general

A

4 mg/day

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41
Q

What is the most malignant pediatric CNS tumor?

A

Meduloblastoma

42
Q

CP Prevalence

A

1 - 5/1,000 births worldwide
2/1,000 Europe
3/1,000 US

43
Q

When does a baby talk

44
Q

Optimal use of DEXA in CP

  1. scan hip or L-spine
  2. avoid scan in fracture area
  3. Low bone density is Z-score -2 or below
  4. low bone density T-score 2.5 or below
A
  1. Low bone density CP defined at Z-score < 2
45
Q

Which of the following is TRUE:
Pediatric TBI pathophysiology differs from adult because
A. Pedi brains have less myelination
b. Pedi brains have decreased cerebral blood flow
c. Pedi brains have fewer synapses
d. Pedi brains favor less excitability

A

A. Only A is true

46
Q

Locations of pediatric CNS tumors

A 
Supratentorial:  28/%
Infratentorial: 26%
Pituitary/pineal:  17%
Non-specific:  14%
Cranial nerves:  6%
Spinal cord/cauda eq:  5%
Meninges:  3%
47
Q

What is Arnold Chiari II?

A

Herniation of fetal cerebellum and medulla downward into spinal canal and up into the middle fossa

48
Q

4 prenatal risk factors for CP

A

Major birth defects
Fetal growth restriction
Maternal Infection
Multiparous Births

49
Q

What is the other gene for SMA?

A

SMN 2
Also codes for the SMN protein. Quantities are not absent but inadequate.
Absence is fatal. 4-8 copies more milder disease

50
Q

Modified Ashworth Scale

A 
0 - normal
1 - slight catch
1+ - slight catch with continued increased in resistance
2 - resistance through full ROM
3 - considerable increase in resistance
4 - rigid
51
Q

How does diazepam work?

A

Diazepam is a benzodiazepine that exerts anxiolytic, sedative, musclerelaxant, anticonvulsant and amnestic effects. Most of these effects are thought to result from a facilitation of the action of gamma aminobutyric acid (GABA)-A , an inhibitory neurotransmitter in the central nervous system.

52
Q

Who is at highest risk for scoliosis in spina bifida?

A

Higher (Thoracic) level has 100% incidence

53
Q

How does Dantrolene sodium work?

A

Inhibition skeletal muscle contraction by suppressing release of calcium

54
Q

What is the Melbourne (Upper Extremity) Assessment?

A

The MA2 can be used with children aged 2.5 to 15 years who have either a congenital (for example, cerebral palsy) or an acquired neurological condition.
The MA2 has been developed for children with a range of different movement disorders including spasticity, dystonia, choreoathetosis and ataxia

55
Q

Definition of Tics

A

Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement

56
Q

Signs of Syrinx on spina bifida

A
  1. Weakness
  2. Spasticyt
  3. Irritability
57
Q

Symptoms, association, and treatment of Autononomic instability (Paroxysmal sympathetic hyperactivity)

A
  1. High temp
  2. High BP
  3. High respiratory rate
    Associated with increased duration of coma. Treat with beta blockers and ITB
58
Q

When does a baby stand, creep on all fours, have a pincer grasp, and finger feeds

59
Q

When do children with spina bifida have their maximum ambulation

A

9 years of age

60
Q

Name 2 global functionality pediatric systems

A
  1. PEDI

2. weeFIM

61
Q

When does a baby walk alone with a wide base

62
Q

Complication of anoxic brain injury in children

A
  1. Higher seizure rates
  2. Heterotopic ossification
  3. GI complications
  4. Pneumonia
  5. High risk of autonomic dysfunction and storm
63
Q

2 classification systems for CNS pediatric tumors

A

WHO: tumor histology

International Classification of Childhood cancer: Site and morphology

64
Q

Signs of physical abuse of a child

A
  1. Shaking or impact
  2. Skull fracture
  3. Subdural
  4. Brain contusions
  5. Retinal hemorrhages
  6. Skeletal survey findings
65
Q

Simple demographics of pediatric TBI

A

Agest 0-4 (falls and 15-19 (MVA) are highest.

Males higher than females

66
Q

CP Low Bone Density Fractures

A

Prevalence: 20% for non-ambulatory Patients
7-9.7% per year incidence for severe CP
Most common distal femur

67
Q

Definition of Myoclonus

A

Myoclonus is a sequence of repeated, often nonrhythmic, brief shock-like jerks due to sudden involuntary contraction or relaxation of one or more muscles

68
Q

Definition of Athetosis

A

Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture

69
Q

Name 8 treatments for dystonia

A
  1. Baclofen
  2. Artane
  3. Benzodiazepinews
  4. Clonidine
  5. Gabapentin
  6. DBS
  7. ITB
  8. Botox
70
Q

Classification: Motor CP

A

Spastic: 80%
Dyskinetic: 10% (Athetosis, Chorea, Dystonia)
Ataxic/hypotonic: 10%

71
Q

Which is true regarding CP

  1. Prenatal factors major
  2. Labor causes high %
  3. Premature no longer a problem
  4. Most CP children premature
A
  1. Prenatal is major contributor
  2. Labor <10%
  3. Premature strongest risk factor
  4. Most children with CP are born at or near term
72
Q

Factors of the pediatric brain that worsen or affect TBI injury

A
  1. Increased brain water
  2. Less myelin
  3. increased blood flow
73
Q

What does SEMLS stand for?

A

Single Event MultiLevel Surgery

74
Q

Describe hips in Spina Bifida

A

Often dislocated
Most problematic with 1 hip out
Often do not need surgery

75
Q

Babies at higher risk of spina bifida

A 
Female
Children of mom with spina bifida
Maternal febrile
Lack of folic acid
Depakote
Maternal obesity
Solvents
76
Q

Definition of Chorea

A

Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments

77
Q

What Cobb angle is cut-off for scoliosis fusion

A

> 40 to 50 degrees

78
Q

Definition of Tremor

A

Tremor is a rhythmic back-and-forth or oscillating involuntary movement about a joint axis

79
Q

Cut offs to look diligently for Neuromuscular diseases

A
  1. Can’t sit by 9 months
  2. Can’t walk by 18 months
  3. Can’t run by 2 1/2
  4. Can’t stand on one leg by 4
80
Q

Determinants of TBI pediatric outcome

A
  1. Duration of post-traumatic amnesia

2. Measure with Children’s Outcome and Amnesia Test (COAT)

81
Q

Definition of Dystonia

A

Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both

82
Q

3 Means of prevention of low bone density in CP

A
  • proper nutrition and calcium intake
  • vitamin D
  • Increase weight bearing
83
Q

Causes of anoxic brain injury in children

A
  1. Drowning
  2. Seizures
  3. Illnesses
84
Q

Hypertonia Assessment Tool (HAT)

A

7 item tool to differentiate spasticity, dystonia, and rigidity

85
Q

Describe factors in pediatric Transverse Myelitis

A
  1. Immune mediated CNS inflammation
  2. MRI has high T2
  3. Children do better than adults
  4. 1/2 make full recovery by 2 years
86
Q

Safe dosing for Botox includes all the follow factors EXCEPT:

a. Body weight
b. Previous Orthopedic Surgery
c. Functional mobility level
d. Comorbidities

A

b. Previous orthopedic surgeries

Lower dosing for GMFCS IV and V, prior swallowing or respiratory difficulties, and adjusted for body weight

87
Q

Which of the following contributes to hip subluxation in CP:

  1. Congenital hip dysplasia
  2. Overactive gluteus medius and minimus
  3. Weakness of adductors
  4. Femoral anteversion and coxa valgus
A
  1. Femoral anteversion
88
Q

Inheritance of Spinal Muscular Atrophy (SMA)

A

90% Autosomal Recessive

89
Q

When can a child skip, speak fluently, and tie shoes

90
Q

What is a calcium risk in pediatric SCI?

A

Hypercalcemia

Lethargy and Nausea, esp with adolescent boys

91
Q

Treatments for ADEM

A
  1. IV steroids
  2. IVIG
  3. Plasmapheresis
  4. Clyclophosphamide
  5. Mitoxantrone
92
Q

Risk of early mortality for CP

A
  1. Seizures
  2. Cognitive impairment
  3. Severe vision and hearing loss
  4. Non-verbal
93
Q

Spasticity

A

Increased resistance to velocity dependent stretch

94
Q

Another name for rocker bottom

A

Vertical talus. 10% children with Sina bifida

95
Q

Incidence of HO in children

A
  1. UNCERTAIN %

2. Hip, knee, shoulder, and elbow most common

96
Q

Contrast 2 definitions CP

A

Earlier: Disorder of movement and posture, non-progressive from early brain abnormality

2007: Problems with sensation, perception, cognition, communication, behavior, seizures, and musculoskeletal symptoms change with time

97
Q

What testing does the American Academy of Pediatrics recommend for all children with low tone and developmental delay?

a. No testing unless delays persist past two years
b. MRI of the brain alone
c. MRI of the brain and complete spinal cord
d. CPK and TSH

A

d. CPK and TSH

The AAP recommends neuroimaging if developmental delays are associated with high tone. For children with low tone, the AAP recommends CPK and TSH as a first step

98
Q

Describe the ideal SDR candidate

A
  • spastic diplegia
  • premature birth
  • PVL
  • GMFCS 1-3
  • GMFM greater than 60

Contraindications: spasticity of spinal cord origin, other movements like dystonia, weakness

99
Q

Tardieu Scale

A

R1 - first catch

R2 - end range

100
Q

What does SCWORA stand for?

A 
Spinal
Cord 
Injury
WithOut
RAdiographic
These are 30-40% of all pediatric SCI
101
Q

Barry-Albright Dystonia Scale (BADS)

A

5 point dystonia scale

specific areas of the body covered.

PM&R Pediatric Boards (14 decks)

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