Wasted copy A2 Neurologic Disorders: Brain COPY Flashcards
When does head control develop
4 months
When do babies babble
6 - 10 months
When do babies sit
7 - 8 months
When does a baby stand, creep on all fours, have a pincer grasp, and finger feeds
10 months
When does a baby talk
12 months
When does a baby walk with help
12 months
When does a baby walk alone with a wide base
14 months
When does a child run, do stairs, and demonstrate hand dominance
2 years
When can a child ride a trike, be toilet trained, speak a 3 work sentence, and draw a circle
3 years
When can a child hop, use a pencil, use scissors, and dresses
4 years
When can a child skip, speak fluently, and tie shoes
5 years
When can a child use roller skates and recite the alphabet
6 years
CP Prevalence
1 - 5/1,000 births worldwide
2/1,000 Europe
3/1,000 US
What is the trend in Prevalence of CP
Decreasing in Preterm
Stable in term
Combined stable
4 Perinatal risk factors for CP
Prematurity
Breech
Birth complications (placental detachment, cord, uterine rupture)
Birth asphyxia (<10%)
3 Postnatal risk factors for CP
Trauma
Infection
Coagulopathies (sickle cell, clotting deficiencies, etc.)
Which is true regarding CP
- Prenatal factors major
- Labor causes high %
- Premature no longer a problem
- Most CP children premature
- Prenatal is major contributor
- Labor <10%
- Premature strongest risk factor
- Most children with CP are born at or near term
Neuroimaging in CP
- 90% abnormal
- Most commonly prenatal
- Destructive 90% (normal brain insulted with PVL or deep grey matter injury)
- Brain Mal-development 10%
- Normal Neuroimaging 10%
Classification: Motor CP
Spastic: 80%
Dyskinetic: 10% (Athetosis, Chorea, Dystonia)
Ataxic/hypotonic: 10%
What Cobb angle is cut-off for scoliosis fusion
> 40 to 50 degrees
Risk factors for hip migration in CP
Malalignment (valgus neck shaft angle) Femoral anteversion Decreased weight-bearing Spasticity Weakness of hip abductors and extensors
Which of the following contributes to hip subluxation in CP:
- Congenital hip dysplasia
- Overactive gluteus medius and minimus
- Weakness of adductors
- Femoral anteversion and coxa valgus
- Femoral anteversion
Risk factors for poor bone health in CP
- nutrition and absorption
- decreased exposure to sun
- non-ambulatory
- anti-seizure meds
What is the z-score in DXA scans for Osteoporosis in children
z-score <2.0
What is used for DXA scan measure for children
z-score
Not T-score
Distal Femur
CP Low Bone Density Fractures
Prevalence: 20% for non-ambulatory Patients
7-9.7% per year incidence for severe CP
Most common distal femur
3 Means of prevention of low bone density in CP
- proper nutrition and calcium intake
- vitamin D
- Increase weight bearing
Optimal use of DEXA in CP
- scan hip or L-spine
- avoid scan in fracture area
- Low bone density is Z-score -2 or below
- low bone density T-score 2.5 or below
- Low bone density CP defined at Z-score < 2
Spasticity
Increased resistance to velocity dependent stretch
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
Tardieu Scale
R1 - first catch
R2 - end range
Hypertonia Assessment Tool (HAT)
7 item tool to differentiate spasticity, dystonia, and rigidity
Barry-Albright Dystonia Scale (BADS)
5 point dystonia scale
specific areas of the body covered.
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
Definition of Chorea
Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments
Definition of Athetosis
Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture
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
Definition of Tremor
Tremor is a rhythmic back-and-forth or oscillating involuntary movement about a joint axis
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
Name 4 lower extremity functional measures for CP
- Instrumented and clinical gait parameters
- Gross Motor Function Measure (GMFM)
- GMPM
- 6 minutes walk test
Name 2 upper extremity pediatric functional tests
- Melbourne assessment
2. QUEST
Name 2 global functionality pediatric systems
- PEDI
2. weeFIM
Name 2 individual goal attainment measures
- COPM
2. GAS
Name 2 Quality of life pediatric measures
- Child Health Questionnaire
2. Caregiver Questionnaire
What does SEMLS stand for?
Single Event MultiLevel Surgery
How does diazepam work?
CHECK THIS–
Presynaptic inhibition of GABA-A receptors
How does baclofen work?
CHECK THIS–
Presynaptic inhibition GABA-B receptors
How does Dantrolene sodium work?
Inhibition skeletal muscle contraction by suppressing release of calcium
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
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
Name 8 treatments for dystonia
- Baclofen
- Artane
- Benzodiazepinews
- Clonidine
- Gabapentin
- DBS
- ITB
- Botox
What is the life expectancy for independently ambulatory children with CP compared to their peers?
Same
What is the life expectancy of a child with CP who can’t lift his head up from supine
<20% norm
Risk of early mortality for CP
- Seizures
- Cognitive impairment
- Severe vision and hearing loss
- Non-verbal
Simple demographics of pediatric TBI
Agest 0-4 (falls and 15-19 (MVA) are highest.
Males higher than females
Factors of the pediatric brain that worsen or affect TBI injury
- Increased brain water
- Less myelin
- increased blood flow
Signs of physical abuse of a child
- Shaking or impact
- Skull fracture
- Subdural
- Brain contusions
- Retinal hemorrhages
- Skeletal survey findings
Causes of anoxic brain injury in children
- Drowning
- Seizures
- Illnesses
Complication of anoxic brain injury in children
- Higher seizure rates
- Heterotopic ossification
- GI complications
- Pneumonia
- High risk of autonomic dysfunction and storm
Symptoms, association, and treatment of Autononomic instability (Paroxysmal sympathetic hyperactivity)
- High temp
- High BP
- High respiratory rate
Associated with increased duration of coma. Treat with beta blockers and ITB
Incidence of HO in children
- UNCERTAIN %
2. Hip, knee, shoulder, and elbow most common
Determinants of TBI pediatric outcome
- Duration of post-traumatic amnesia
2. Measure with Children’s Outcome and Amnesia Test (COAT)
Pediatric Glascow coma scale
Less than 5 is the cut-off for pediatric scale (compares for 8 with adults)
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
What are the symptoms of ADEM (Acute Disseminated Encephalomyelitis)?
Weakness
Loss of vision
Numbness
Loss of Balance
Differential diagnosis of ADEM
Tumors MS Direct infection LP shows lymphocytes No oliclonal bands (Seen in. MS)
Etiology of pediatric stroke`
Cardiac Vasculoar Sickle Cell Infection Trauma Genetics
Pediatric CNS tumors
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
2 classification systems for CNS pediatric tumors
WHO: tumor histology
International Classification of Childhood cancer: Site and morphology
What is the largest group of CNS pediatric tumors
Low grade astrocytoma
What is the most malignant pediatric CNS tumor?
Meduloblastoma
Treatments for ADEM
- IV steroids
- IVIG
- Plasmapheresis
- Clyclophosphamide
- Mitoxantrone
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%
Symptoms of pediatric CNS tumors
Sudden onset headache Nausea and vomiting Ataxia Cranial Nerve Palsy Impaired vision
List 3 types of neural tube defects:
- Encaphalocele
- Anencephaly
3,. Spina Bifida
Name 3 types of Spina Bifida
- Myelomeningocele
- Meningocele
- Occulta
Babies at higher risk of spina bifida
Female Children of mom with spina bifida Maternal febrile Lack of folic acid Depakote Maternal obesity Solvents
Dose of folate for prevention of spina bifida in general
4 mg/day
Prenatal screen for spina bifida
increased alphafetoprotein at 16-18 months
Ultrasound findings at 18-22 weeks
What is more predictable of function in Spina Bifida
a. Surgical anatomy
b. Exam and motor capacity
b,
When do children with spina bifida have their maximum ambulation
9 years of age
Complications of spina bifida
- Hydrocephalus
- Tethered cord
- Syrinx
- Arnold-Chiari II
Signs of Syrinx on spina bifida
- Weakness
- Spasticyt
- Irritability
What is Arnold Chiari II?
Herniation of fetal cerebellum and medulla downward into spinal canal and up into the middle fossa
Who is at highest risk for scoliosis in spina bifida?
Higher (Thoracic) level has 100% incidence
Describe hips in Spina Bifida
Often dislocated
Most problematic with 1 hip out
Often do not need surgery
Describe clubfoot with spina bifida
Common
Early casting helpful
Surgery helpful
Another name for rocker bottom
Vertical talus. 10% children with Sina bifida
4 factors of hip dislocation in spina bifida
FILL OUT
What does SCWORA stand for?
Spinal Cord Injury WithOut RAdiographic These are 30-40% of all pediatric SCI
What is a calcium risk in pediatric SCI?
Hypercalcemia
Lethargy and Nausea, esp with adolescent boys
Describe factors in pediatric Transverse Myelitis
- Immune mediated CNS inflammation
- MRI has high T2
- Children do better than adults
- 1/2 make full recovery by 2 years
Cut offs to look diligently for Neuromuscular diseases
- Can’t sit by 9 months
- Can’t walk by 18 months
- Can’t run by 2 1/2
- Can’t stand on one leg by 4
What is the GMFM
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.
What is GMPM?
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.
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
Inheritance of Spinal Muscular Atrophy (SMA)
90% Autosomal Recessive
with neurologic structure degenerates in SMA?
Anterior Horn Cells
what gene diminishes neuronal functional survival in SMA?
SMN1 (Survival Motor Neuron 1)
On chromosome 5q13
SMN protein essential for anterior horn cell survival
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
