Cerebral palsy Flashcards
Types of genetic changes in CP
Single nuceotide variants
Mitochondrial
Copy number variant
Name 2 features of each GMFCS status between 2-4 years old
Name 4 treatments for sialorrhea in CP?
Environmental: Optimize positioning, manage medical comorbidities (GERD, dental pain, epilepsy)
Behavioural strategies: Reminders
Medications: Anticholinergics - atropine, glycopyrrolate, scopolamine, botox
Surgical: Duct ligation, gland excision, duct rerouting (contraindicated in posterior drooling)
Name 4 indications for surgery in idiopathic toe walking
Significant plantarflexion contraction (-20 degrees)
Functional impairment
failure of conservation management
(ideally post growth spurt)
5 requirements for ambulation
stability in stance
sufficient foot clearance in swing
appropriate pre position of foot
energy conservation
adequate step length
components of gait
Stance phase:
Initial contact (heel strike)
Loading response (foot flat)
Mid-stance
Terminal stance (heel off)
Pre-swing (toe off)
Swing phase
Initial swing
Mid-swing
Late swing[5]
name 3 muscle groups affected in CP in the upper extremity
elbow flexion
wrist flexors
forearm pronators
shoulder adductors
thumb adductors
Side effects of contraint therapy
- injury to involved hand due to less sensory awareness and motor contol
- loss of independence
- increase risk of falls or loss of balance
What does the tardieu scale measure and what does it quantify?
Spasticity
Measures R1 (angle at fast stretch) and R2 (angle at passive stretch) to determine dynamic component of spasticity
what are 3 reasons why GMFCS should not be used in populations other than CP
Based on CP data
Not valid in other populations
other diagnosis have different prognosis and motor patterns
Increase spasticity x 2 weeks and abdo pain, on carbamazepine
- investigations ?
Electrolytes, CRP, CBC, urine culture, carbamazapine level, liver enzymes
Fast MRI/shunt series
Abdominal ultrasound
Contraindication for botox injections
allergy
infection over site of injection
fixed contracture
excessive weakness
(only generalized hypertonia, significant concerns with swallowing/aspiration)
Indications for tube feeding in GMFCS IV ?
Recurrent aspiration
Growth failure
Excess feeding times
Dysmotility
GERD limiting intake
6 red flags warrant urgent CP evaluation ?
early handedness <12 months
persistent fisting >4 months
asymmetry in posture or movement
persistent head lag > 4 monhs
inability to roll or sit unsupported by 9 months
(Not weight baring thrugh legs at 12 months)
(Not walking at 18 months)
Spasticity, dystonia or hyperreflexia at any age / Stiffness or tightess in the legs
5 components of body function to ask in hemi CP
Contracture
Pain
Vision
Siezure
Fracture
When do you have an adult gait pattern
8 years old
2 components of HAT that assess spasticity
Velocity dependent resistant to stretch
Spatic catch
Differentiate spasticity and dystonia
Spasticity:
Velocity dependent
Reproducible/consistent catch
Dystonia:
Inducible by stimulus (emotion, sensation, movement, concentration)
Variable
Twisting/posturing
Indication to treat dystonia with medication in CP?
- Dystonia causing pain
- Dystonia affecting sleep
- Dystonia interfering with positioning
- Dystonia impeding function
- Status dystonicus (or “periodic status dystonicus”)
Indications for surgery referral in hip dysplasia
Migration percentage >30%
pain on physical exam
Hip abduction passive range of motion <30
(Rapid progression >10% in 1 year indication to increase frequency of monitoring)
3 year old with 5 degrees ankle dorsiflexion
- investiation?
- 3 initial management strategies?
- surgical indications
CK
Night splinting
gastroc/soleus stretching
possible splinting
trunk or lower extremity testing
possible serial casting with or without botox after 4-6 month
failure of conservative management
significant contracture
older age
pain with AFO, hemi CP, 4 options to discuss
changing AFO
botox
serial casting
surgery
PT for strength and ROM
10 yo GMFCS IV, no active knee extension, weak hip adductors
Which gait pattern
Which orthotic would you recommend?
Crouch gait pattern
Rigid AFO
Knee splint
Describe GMFCS II in 2-4 year old
Sitting independently
get in and out of sitting without assistance
pulls to stand independently
prefers to cruise when walking or walking with hand support
4-point crawl
3 characteristics associated with life expectancy in CP ?
- GMFCS status non-ambulatory
- feeding difficulties
- seizures
- cognitive impairment
- visual impairment
Pros and Cons of modified ashworth scale (MAS)
Pros
- Track change over time
Cons
- Cannot differentiate spasticity from other hypertonia
- high inter-rater reliability
benefits of wheelchair in young children
Increased independence
Improved social skills perceived by caregivers/parents
Improved disability and developmental scales
Mobility activities during play
Benefits of CIMT?
additional treatments to supplement?
improved quality of hand use;
new motor movements of the affected arm or hand;
more spontaneous use of the affected arm or hand;
improved hand function such as fine motor and grasp.
Supplement of CIMT: Botulinum toxin followed by OT
Examples of bimanual activities
swimming
jump rope
knife + fork
scissors
use as stabiliser
holding large objects
Definition of CP
Heterogeneous group of disorders of the development of movement and posture, causing activity limitation, as a result of a non-progressive insult to the developing fetal or infant brain.
Accompanied by differences in cognition, communication, behavior, seizures, perception and MSK complications.
Side effects of ITB pump
Constipation
Hypotonia/weakeness
surgical complications
infection
pump malfunction
ways to decrease risk of osteopenia in CP?
Optimize calcium and vitamin D intake (treat deficiency)
Promote weight baring activities
Differential for idioapthic toe walking
CP
HSP
Autism
Muscular dystrophy
CMT
tethered cord
spina bifida
GMFCS IV parents want to know if they will walk at 18 years old
Clarify what the parent means by ambulation
GMFCS can be used to predict outcome
May walk indoors with physical assistance
Most use manual or powered wheelchair for mobility and participation
Will need adaptive seating for pelvic and trunk control
Definition of hypotonia
abnormally low muscule tone, or reduced resistance to passive. relatively rapid movement (AACPDM)
5 consequences of central hypotonia
1) impede motor function through decreased joint stability, joint hypermobikity, weakeness and or decreased activity and endurance
2) interfere with ability to attain positions and acquisition of motor milestones
3) interfere with function activities and lead to participation restrictions
4) limit sleep due to reduce abikity to change positions (discomfort, poor sleep quality)
5) result in reflux and constipation
6) drooling or feeding problems
Describe the impacts of central hypotonia using the ICF framework
List 3 motor function assessments than can be used for infants at high risk for motor delays and neuromotor problems (and associated age range)
- Hammersmith Infant Neurological exam, HINE (3 - 24 months)
- Test of Infant Motor Performance (TIMP, term to 3 months)
- Peabody Developmental Motor Scales (PDMS)
- Developmental Assessment of Young Children (DAYC-2) Motor scales
- Aberta Infant Motor Scale (AIMS, 0-18 months)
Name 2 outcome measures for children with neuromotor conditions (can be used for goal setting)
Canadian Occupational Performance Measure (COPM)
Goal Attanment Scaling
SMART goals
List 5 components of developmental strategies for management of central hypotonia (performaned by therapist or early intervensionists)
Give 3 examples of specific therapeutic interventions or tools to support interventions
- goal directed to promote function and participation
- child active, child initiated, child directed
- provided by caregiver whenever possible
- avoid extreme positions, optimize stability and seating support
- encorporate into daily life
- avoid passive interventions
Tummy time
Massage (bonding and sleep promotion)
treadmill training (>10 month)
Orthotics
Adaptive equipment
Posture management
3 medical strategies for central hypotonia management
screening for comorbidities: vision, hearing, feeding reflux, communication
Hip surveillance
Orthosis for foot stability
Adaptive equipment
Name 3 gross motor function assessments for central hypotonia / CP
GMFCS (gross motor function classification scale)
GMFM (Gross motor function measure)
Brigance
PDMS-2 (Peabody Developmental Motor Scales)
Name 4 interventions for treatment of central hypotonia in children <10 months old
massage (low evidence)
tummy time (low evidence)
active motor therapy - goal directed, child-initiated, therapist coached, parent delivered, context relevant (low evidence)
postural management and supported seating (very low evidence)
compression garments (very low evidence)
Definition of dystonia
type of hypertonia
movement disroder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive movements, postures or both
resistance to passive stretch of a muscle across a joint, present at low speeds, fluctuates with repeated stretches, and where tactile stimulus or purposeful movement of another body party triggers increased tone or increased involuntary postures
patterned, twisting, or may be tremulous
Name 3 impacts of dystonia
impede motor function through involuntary muscle contractions, limitations in muscle relacation and overflow (involuntary activation of unintended muscles during attempted voluntary movements)
interfere with positioning for sitting and lying
painful
interefere wtih sleep
high energy expenditure and malnutrition
impact wellbeing
associated with orthopedic deformities
indication for treatment of dystonia
dystonic posutres, hypertonia, and slow involuntary movements that are causing interference
definition of spasticity
a velocity-dependent resistance of a muscle ot stretch - resistance to externally imposed movement increases with increasing speed of stretch
definition of chorea
ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments (more rapid than dystonia)
definition of athetosis
slow continuous, involuntary writhing movement that prevents maintenance of a stable posture
myoclonus definition
sequence of repeated, often non-rhythmic, biref shock-like jerks due to sudden involuntary constraction or relaxation of oneof more muscle
definition of status dystonicus
status dystonicus is characterized by the development of increasingly frequent or continuous severe episodes of generalized dystonic spasms (contractions) and requires urgent (hospital) management
assessment of dystonia
hypertonia assesssment tool (HAT)
Name 4 measures of dystonia severity
Barry Albright Dystonia Scale
Burke-Fahn-Marsden Dystonia Rating Scale
Dyskinesia Impairmetn Scale - dystonia subscale
Global Dystonia Severity Rating Scale
Dyskinetic CP Functional Impact Scale
Dystonia Severity Action plan
Approach to general dystonia management
family centered decision making - balancing benefits and harms
- Optimize general health
- rehabilitation - promote function, comfort, and participation (evidence for specific rehab strategies absent)
- Medications
– Baclofen
– Trihexyphenidyl & Benzodiazepines (second line after favorable trial) - AGAINST at first line
- NO cannibis
- Levodopa to rule out dopamine responsive dystonia
– Gabapentin and clonidine for pain, sleep disturbances, or seating intolerance
Treatment options for severe dystonia
Intrathecal baclofen
deep brain stimulation
Benefits of early intervention
optimize natural plasticity and imporved motor/cognitive function through intense, motor learning task-specific approaches
Goal of early intervention therapies in CP
exercise muscules through thier functional length
train specific actions to promote motor learning and drive plasticity and effective functional motor performance
Specific therapy options in hemiplegic CP
Constraint Induced Movement therapy
“GAME” - Goals - Activity - Motor Enrichment - early, intense, enriched, task-specific, training-based interventions at home
Hemiparesis + CP
what 4 interventions can OT do (evidence based)
-constraint therapy (constraint induced movement therapy)
-bimanual training
-goal-directed training (GAME - Goals, activity, motor enrichment)
-botox + OT
2 specific genetic mutations found in CP?
COL4A1
CTNNB1
CP associated conditions & percentages (10)
Deaf 1/25
Blind 1/10
Epilepsy 25%
Sleep issue 20%
Bladder incontinence 25%
Non-verbal 25%
Non-oral feeds 1/5
IDD 50%
Behaviour issues 25%
Pain 75%
indications for SDR?
Primarily lower extremity involvement (diplegia)
Primarily spasticity as hypertonia type
Anti-gravity/adequate strength for ambulation if goal is to improve ambulation and function
Failure of oral medication, other spasticity management strategies