Neuromuscular 1: Cerebral Palsy, TBI, Brain Tumor

cerebral palsy definition

group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of CP are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.

preconception risks of CP

history of still births, miscarriages, low socioeconomic status, assisted reproduction, genetic differences

pregnancy risks of CP

genetics, birth defects, multiples, males, maternal thyroid disease or preeclampsia, infection, intrauterine growth restriction, prematurity, substance abuse

perinatal birth risks of CP

acute intrapartum hypoxia-ischemia, seizures, hypoglycemia, jaundice, infection

postneonatal risks for CP

stroke, infection, surgical complication, accidental and non-accidental brain injury occurring before 24 months

when must a developmental incident occur for subsequent developmental disorder to be considered cerebral palsy

before 2 years of age

an CP be a genetic disorder?

yes, in 25-30% of cases. It may also be co-diagnoses with another genetic disorder

what are the most common brain injuries leading to CP?

perinatal stroke, intraventricular hemorrhage, periventricular leukomalacia, hypoxic-ishemic encephalopathy

periventricular leukomalacia

commonly seen in premature infants dt difficulty regulating BP, changes in blood flow lead to bilateral white matter necrosis adjacent to the lateral ventricles, which affects the descending fibers of the corticospinal tract. Type of CP depends on extent of this lesion

intraventricular hemorrhage

common in premature infants, bleeding in the brain arounds the lateral ventricles leads to injury of parenchyma. Increased fluid can also lead to the development of hydrocephalus

grade 1 IVH

bleeding is near ventricle, often resolves and results in no disability

grade 2 IVH

blood is in ventricle, associated with less impairment

grade 3 IVH

blood is in ventricle and ventricle enlarges, likely to lead to CP and lasting disability

grade 4 IVH

ventricle has englarged and blood is now in brain tissue, likely leading to CP and lasting disability

treatment for hydrocephalus

drain fluid from brain

hypoxic-ischemic injury

usually in full-term infants, caused by a lack of oxygen and blood flow to brain around time of birth, leading to widespread bilateral brain injury. early intervention with therapeutic induced hypothermia can improve outcomes

when should you initiate therapeutic induced cooling?

with 6 hours of birth until 72 hours

therapeutic induced hypothermia

lowers temp of brain, reduces mortality and neurodevelopmental disability

perinatal stroke

can occur before or after birth within 1 month of birth, can be ischemic or hemorrhagic leading to local death of brain tissue, most commonly affects L MCA

lissencephaly

atypically smooth brain

schizencephaly

abnormal clefts in cerebral hemispheres

porencephaly

fluid filled cyst

brain malformations & neuronal migration disorders

absent or incorrect signals during fetal brain development leads to structural abnormalities or missing areas

how do you identify a stroke in infancy

seizures, atypical movement, presence of perinatal risk factors

injury to the cerebral cortex associated with…

stiff muscles/ spasticity

injury to basal ganglia associated with…

uncontrollable movements/ dyskinesia

injury to the cerebellum associated with…

poor balance and coordination/ ataxia

damage to multiple areas of the brain

mixed presentation with two or more types of CP

diplegia

legs are more affected than arms, most common in premature babies

hemiplegia

one side of the body is affected, usually arm is more affected than leg

quadriplegia

the entire body is affected, most common in babies who experience a lack of oxygen

spastic cerebral palsy

most common type of CP, upper motor neuron involvement in pyramidal system

spastic cerebral palsy presentation

hyperreflexia, clonus, synergistic patterns, impaired selective motor control, persistent or exaggerated primitive reflexes

spastic hemiplegia

one side of the body involved, usually more arm involvement than leg, right side more commonly involved as L MCA usually involved

spastic diplegia

lower extremities are more involved than upper extremity, may see asymmetry, mild coordination impairment in upper extremity

spastic triplegia

three extremities involved (both lower and one upper), mild coordination deficits in non-involved arm, features similar to quadriplegic

spastic quadriplegia

all extremities are involved, may have high or low trunk tone, oromotor dysfunction is common, and cognitive impairment is common

dyskinetic cerebral palsy

extrapyramidal impairment so injury is in basal ganglia, thalamus, etc and presents with atypical patterns of posture and involuntary, uncontrolled, recurring stereotyped movements of affected body parts

athetosis

slow, continuous, writhing involuntary movements particularly in distal extremities which prevent stable posture and increase with purposeful activity, less common

chorea

abrupt, jerky movements, occur in neck, trunk, and extremities

choreoathetosis

combination of athetosis and chorea

dystonia

slow, rhythmic movements with fluctuating tone and posturing, involving trunk and extremities, increases with emotion and intent, and can occur in any topography of CP although full body posturing is more common in quadriplegic CP

ataxia

cerebellar injury leading to inability to generate normal movement trajectories that are not attributed to weakness or involuntary muscle activity at affected joints that is not attributed to weakness or involuntary muscle activity at affected joints

ataxia presentation

dysmetria, nystagmus, general instability, abnormal patterns of posture, lack of coordinated, accurate movements

primary impairments of CP

abnormal muscle tone, spasticity, selective motor control, postural control

secondary impairments of CP

contracture, skeletal malalignment, muscle strength, muscle extensibility

muscle tone

resting tension or resistance of muscle to passive movement of muscle lengthening

presentation of muscle tone in CP

hypertonia, hypotonia, combination

spasticity

muscle tension which increases with speed and varies with direction of movement

spasticity presentation CP

clonus, pathologic reflexes, particular patterns of posture or movement

muscle structure in CP

muscle development affected by differences in muscle innervation, altered loading patterns, and impaired growth leading to decreased muscle volume, muscle length, and force production, alterations of sarcomere properties, muscle fiber size, and muscle type

muscles at increased risk for contracture

shoulder adductors, elbow flexors, wrist flexors, finger flexors, hip flexors and adductors, knee flexors, ankle plantarflexors

presentation of impaired muscle strength in CP

diminished force production capability, inappropriate co-activation of antagonistic muscle groups, secondary myopathy, altered muscle tissue, weak proximal muscles, weak eccentric contractions, and slower speeds of movement

how does muscle activity impact skeletal development

abnormal biomechanical fores due to spasticity, muscle imbalances, weakness, abnormal extensibility lead to scoliosis, torsion, joint instability, premature degenerative changes in long bones, and low bone mineral density

scoliosis

prevalence inc with age and GMFCS level, 60% presence in spastic quadriplegia

hip subluxation/ dislocation in CP

decreased standing and ambulatory weight bearing leads to malformed or shallow acetabulum leading to joint instability. Risk is 30% overall but increases with GMFCS level

selective muscle control

inability to isolate muscle activation

presentation of selective muscle control

move in synergistic patterns, reduced speed of movement, abnormal reciprocal activation, mirror movements

how to measure selective muscle control

SCALE, mini-SCALE, BabyOSCAR

postural control

impaired ability to control body position in space

presentation of impaired postural control

difficulty with both reactive and anticipatory postural adjustments, difficulty adapting degree of muscle contraction appropriate to degree/ situation

cause of fatigue in CP

high rates of energy expenditure, decreased force production, excessive co-contraction and inefficient movement patterns

impact of fatigue

reduced participation and QOL

cognitive impairment and CP

IQ <70, present in 23-44% of children with CP, most common in spastic quadriplegia

incidence of behavioral impairment

25%

Seizures in CP

22-40%, most common in spastic quadriplegia and hemiplegia

visual impairment

62-71%, strabismus, refractive errors, cerebral or cortical visual impairment

incidence of hearing impairment

25%

tactile sensory impairments

44-51%, stereognosis, proprioception, two point discrimination

oromotor dysfunction

most common in spastic quadriplegia and dyskinesia, inc risk for aspiration and malnutrition, may present as feeding dysfunction, drooling, or dysarthria

incidence of dysarthria

42-81%

gastrointestinal dysfunction

GERD, 59% with constipation

genitourinary dysfunction

neurogenic bladder, 23% with incontinence

pulmonary dysfunction

deficient ventilation, aspiration pneumonia, reactive airway disease or bronchopulmonary dysplasia

chronic pain in CP

increases with age and GMFCS level, more common in dyskinetic or mixed dyskinetic-spastic CP and is caused by musculoskeletal changes over time, neuropathy, and autonomic dysfunction

intrinsic sources of chronic pain in CP

musculoskeletal pain such as hip dislocation, muscle spasm, or inc tone, GI, neuro system, etc

induced sources of chronic pain in CP

surgery, Botox, poorly fitting assistive devices

chronic conditions more common in adults with CP

diabetes, asthma, hypertension and other cardiovascular conditions, stroke, emphysema, joint pain, arthritis

when can you diagnose someone with CP?

at or before 6 months

what are the criteria used for a CP diagnosis

MUST have motor dysfunction and BOTH of the following: abnormal neuroimaging and a clinical history indicating risk for CP

when does someone have high risk of CP?

clinical diagnosis is suspected but cannot be made with certainty, infant has motor dysfunction but only ONE of the following: abnormal neuroimaging and a clinical history indicating risk for CP

GMA

assess at 9-20 weeks corrected age but optimally at 12-16 weeks, high sensitivity and specificity, looks at infants movements to assess development of nervous system

HINE

assesses cranial nerve function, posture, muscle tone, and reflees at 2-24 months of age

what HINE score predicts CP?

<57 at 3 months

what HINE score indicates likelihood of being non-ambulatory?

<40

what are early motor assessments used for CP?

GMA, HINE, TIMP, AIMS

when should you be concerned about CP and refer a child?

if…

• after 4 months: keep their hands fisted, have persistent head lag, have consistent asymmetry of posture and movements

• at 9 months: still unable to sit without support

• at 6-12 months: have stiffness or tightness in legs

• before 12 months: have hand preference

GMFCS

5 level classification system that describes gross motor function through self initiated movement, has emphasis on sitting and trunk control, transfers, and mobility via walking or wheeled mobility to represent usual performance

clinical application of GMFCS

allows providers to make prognoses, assists with goal setting, can be used to plan episodic care

research application of GMFCS

sample selection and description

GMFM

reliable and valid criterion-referenced assessment designed to detect change in children with CP, aged 5 months to 16 years

GMFM-88

items grouped in 5 dimensions: lying and rolling, sitting, crawling and kneeling, standing, and walking

GMFM-66

improved scoring, interpretation, and requires fewer items to be tested

gross motor curves

Compares GMCS level and GMFM-66 scores to show GMFM-66 scores by age for each of the 5 GMFCS levels, can be used to predict age when 90% of skills are attained

manual ability classification system

assesses child’s ability to handle objects in important ADLs, measures level of support and adaptation required

communication function classification system

assesses ability to perform sender and receiver roles during communication

children walk at home, school, outdoors, and in the community. they can climb stairs without the use of a railing, children perform gross motor skills such as running and jumping but speed, balance, and coordination are limited

GMFCS Level 1

children walk in most settings and climb stairs holding on to a railing. They may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces. children may walk with physical assistance, a handheld mobility device, or used wheeled mobility over long distances. children only have minimal ability to perform gross motor skills such as running and jumping

GMFCS level 2

children walk using a handheld mobility device in most indoor settings. they may climb stairs holding on to a railing with supervision or assistance, children use wheeled mobility when traveling long distances and may self propel for shorter distances

GMFCS level 3

children use methods of mobility that require physical assistance or powered mobility in most settings. they may walk for short distances at hime with a physical assistance of use powered mobility or a body support walker when positioned. at school, outdoors, and in the community, children are transported in a manual wheelchair or use powered mobility

GMFCS level 4