A2 Neurologic Disorders: Brain 0-100 Flashcards
1
Q
Neuroimaging in CP
A
- 90% abnormal
- Most commonly prenatal
- Destructive 90% (normal brain insulted with PVL or deep grey matter injury)
- Brain Mal-development 10%
- Normal Neuroimaging 10%
2
Q
When do babies sit
A
7 - 8 months
3
Q
What are the symptoms of ADEM (Acute Disseminated Encephalomyelitis)?
A
Weakness
Loss of vision
Numbness
Loss of Balance
4
Q
When does a child run, do stairs, and demonstrate hand dominance
A
2 years
5
Q
What is the largest group of CNS pediatric tumors
A
Low grade astrocytoma
6
Q
with neurologic structure degenerates in SMA?
A
Anterior Horn Cells
7
Q
When does a baby walk with help
A
12 months
8
Q
Complications of spina bifida
A
- Hydrocephalus
- Tethered cord
- Syrinx
- Arnold-Chiari II
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.
10
Q
3 Postnatal risk factors for CP
A
Trauma
Infection
Coagulopathies (sickle cell, clotting deficiencies, etc.)
11
Q
Differential diagnosis of ADEM
A
Tumors MS Direct infection LP shows lymphocytes No oliclonal bands (Seen in. MS)
12
Q
List 3 types of neural tube defects:
A
- Encaphalocele
- Anencephaly
3,. Spina Bifida
13
Q
Symptoms of pediatric CNS tumors
A
Sudden onset headache Nausea and vomiting Ataxia Cranial Nerve Palsy Impaired vision
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.
15
Q
4 Perinatal risk factors for CP
A
Prematurity
Breech
Birth complications (placental detachment, cord, uterine rupture)
Birth asphyxia (<10%)
16
Q
What is used for DXA scan measure for children
A
z-score
Not T-score
Distal Femur
17
Q
What is the life expectancy of a child with CP who can’t lift his head up from supine
A
<20% norm
18
Q
Describe clubfoot with spina bifida
A
Common
Early casting helpful
Surgery helpful
19
Q
What is the life expectancy for independently ambulatory children with CP compared to their peers?
A
Same
20
Q
What is the trend in Prevalence of CP
A
Decreasing in Preterm
Stable in term
Combined stable
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
22
Q
4 factors of hip dislocation in spina bifida
A
FILL OUT
23
Q
When can a child ride a trike, be toilet trained, speak a 3 work sentence, and draw a circle
A
3 years
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
25
Name 3 types of Spina Bifida
1. Myelomeningocele
2. Meningocele
3. Occulta
26
Name 4 lower extremity functional measures for CP
1. Instrumented and clinical gait parameters
2. Gross Motor Function Measure (GMFM)
3. GMPM
4. 6 minutes walk test
27
What is the z-score in DXA scans for Osteoporosis in children
z-score <2.0
28
When do babies babble
6 - 10 months
29
Risk factors for poor bone health in CP
- nutrition and absorption
- decreased exposure to sun
- non-ambulatory
- anti-seizure meds
30
Etiology of pediatric stroke`
```
Cardiac
Vasculoar
Sickle Cell
Infection
Trauma
Genetics
```
31
what gene diminishes neuronal functional survival in SMA?
SMN1 (Survival Motor Neuron 1)
On chromosome 5q13
SMN protein essential for anterior horn cell survival
32
When can a child use roller skates and recite the alphabet
6 years
33
When does head control develop
4 months
34
What is the PEDI test/scale?
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
35
Pediatric Glascow coma scale
Less than 5 is the cut-off for pediatric scale (compares for 8 with adults)
36
Risk factors for hip migration in CP
```
Malalignment (valgus neck shaft angle)
Femoral anteversion
Decreased weight-bearing
Spasticity
Weakness of hip abductors and extensors
```
37
When can a child hop, use a pencil, use scissors, and dresses
4 years
38
What is more predictable of function in Spina Bifida
a. Surgical anatomy
b. Exam and motor capacity
b,
39
Prenatal screen for spina bifida
increased alphafetoprotein at 16-18 months
| Ultrasound findings at 18-22 weeks
40
Dose of folate for prevention of spina bifida in general
4 mg/day
41
What is the most malignant pediatric CNS tumor?
Meduloblastoma
42
CP Prevalence
1 - 5/1,000 births worldwide
2/1,000 Europe
3/1,000 US
43
When does a baby talk
12 months
44
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
3. Low bone density CP defined at Z-score < 2
45
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. Only A is true
46
Locations of pediatric CNS tumors
```
Supratentorial: 28/%
Infratentorial: 26%
Pituitary/pineal: 17%
Non-specific: 14%
Cranial nerves: 6%
Spinal cord/cauda eq: 5%
Meninges: 3%
```
47
What is Arnold Chiari II?
Herniation of fetal cerebellum and medulla downward into spinal canal and up into the middle fossa
48
4 prenatal risk factors for CP
Major birth defects
Fetal growth restriction
Maternal Infection
Multiparous Births
49
What is the other gene for SMA?
SMN 2
Also codes for the SMN protein. Quantities are not absent but inadequate.
Absence is fatal. 4-8 copies more milder disease
50
Modified Ashworth Scale
```
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
How does diazepam work?
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
Who is at highest risk for scoliosis in spina bifida?
Higher (Thoracic) level has 100% incidence
53
How does Dantrolene sodium work?
Inhibition skeletal muscle contraction by suppressing release of calcium
54
What is the Melbourne (Upper Extremity) Assessment?
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
Definition of Tics
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
Signs of Syrinx on spina bifida
1. Weakness
2. Spasticyt
3. Irritability
57
Symptoms, association, and treatment of Autononomic instability (Paroxysmal sympathetic hyperactivity)
1. High temp
2. High BP
3. High respiratory rate
Associated with increased duration of coma. Treat with beta blockers and ITB
58
When does a baby stand, creep on all fours, have a pincer grasp, and finger feeds
10 months
59
When do children with spina bifida have their maximum ambulation
9 years of age
60
Name 2 global functionality pediatric systems
1. PEDI
| 2. weeFIM
61
When does a baby walk alone with a wide base
14 months
62
Complication of anoxic brain injury in children
1. Higher seizure rates
2. Heterotopic ossification
3. GI complications
4. Pneumonia
5. High risk of autonomic dysfunction and storm
63
2 classification systems for CNS pediatric tumors
WHO: tumor histology
| International Classification of Childhood cancer: Site and morphology
64
Signs of physical abuse of a child
1. Shaking or impact
2. Skull fracture
3. Subdural
4. Brain contusions
5. Retinal hemorrhages
6. Skeletal survey findings
65
Simple demographics of pediatric TBI
Agest 0-4 (falls and 15-19 (MVA) are highest.
| Males higher than females
66
CP Low Bone Density Fractures
Prevalence: 20% for non-ambulatory Patients
7-9.7% per year incidence for severe CP
Most common distal femur
67
Definition of Myoclonus
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
Definition of Athetosis
Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture
69
Name 8 treatments for dystonia
1. Baclofen
2. Artane
3. Benzodiazepinews
4. Clonidine
5. Gabapentin
6. DBS
7. ITB
8. Botox
70
Classification: Motor CP
Spastic: 80%
Dyskinetic: 10% (Athetosis, Chorea, Dystonia)
Ataxic/hypotonic: 10%
71
Which is true regarding CP
1. Prenatal factors major
2. Labor causes high %
3. Premature no longer a problem
4. Most CP children premature
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
Factors of the pediatric brain that worsen or affect TBI injury
1. Increased brain water
2. Less myelin
3. increased blood flow
73
What does SEMLS stand for?
Single Event MultiLevel Surgery
74
Describe hips in Spina Bifida
Often dislocated
Most problematic with 1 hip out
Often do not need surgery
75
Babies at higher risk of spina bifida
```
Female
Children of mom with spina bifida
Maternal febrile
Lack of folic acid
Depakote
Maternal obesity
Solvents
```
76
Definition of Chorea
Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments
77
What Cobb angle is cut-off for scoliosis fusion
>40 to 50 degrees
78
Definition of Tremor
Tremor is a rhythmic back-and-forth or oscillating involuntary movement about a joint axis
79
Cut offs to look diligently for Neuromuscular diseases
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
Determinants of TBI pediatric outcome
1. Duration of post-traumatic amnesia
| 2. Measure with Children's Outcome and Amnesia Test (COAT)
81
Definition of Dystonia
Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both
82
3 Means of prevention of low bone density in CP
- proper nutrition and calcium intake
- vitamin D
- Increase weight bearing
83
Causes of anoxic brain injury in children
1. Drowning
2. Seizures
3. Illnesses
84
Hypertonia Assessment Tool (HAT)
7 item tool to differentiate spasticity, dystonia, and rigidity
85
Describe factors in pediatric Transverse Myelitis
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
Safe dosing for Botox includes all the follow factors EXCEPT:
a. Body weight
b. Previous Orthopedic Surgery
c. Functional mobility level
d. Comorbidities
b. Previous orthopedic surgeries
Lower dosing for GMFCS IV and V, prior swallowing or respiratory difficulties, and adjusted for body weight
87
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
4. Femoral anteversion
88
Inheritance of Spinal Muscular Atrophy (SMA)
90% Autosomal Recessive
89
When can a child skip, speak fluently, and tie shoes
5 years
90
What is a calcium risk in pediatric SCI?
Hypercalcemia
| Lethargy and Nausea, esp with adolescent boys
91
Treatments for ADEM
1. IV steroids
2. IVIG
3. Plasmapheresis
4. Clyclophosphamide
5. Mitoxantrone
92
Risk of early mortality for CP
1. Seizures
2. Cognitive impairment
3. Severe vision and hearing loss
4. Non-verbal
93
Spasticity
Increased resistance to velocity dependent stretch
94
Another name for rocker bottom
Vertical talus. 10% children with Sina bifida
95
Incidence of HO in children
1. UNCERTAIN %
| 2. Hip, knee, shoulder, and elbow most common
96
Contrast 2 definitions CP
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
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
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
Describe the ideal SDR candidate
- spastic diplegia
- premature birth
- PVL
- GMFCS 1-3
- GMFM greater than 60
Contraindications: spasticity of spinal cord origin, other movements like dystonia, weakness
99
Tardieu Scale
R1 - first catch
| R2 - end range
100
What does SCWORA stand for?
```
Spinal
Cord
Injury
WithOut
RAdiographic
These are 30-40% of all pediatric SCI
```
101
Barry-Albright Dystonia Scale (BADS)
5 point dystonia scale
| specific areas of the body covered.
