Peds: Neurologic Conditions (CP, SCI and Spina Bifida, Autism and Down Syndrome, TBI and stroke, Brachial Plexus, CF and CHD)

A group of permanent disorders of movement and posture causing activity limitations due to nonprogressive disturbances in the developing fetal or infant brain

Cerebral palsy (CP)

Is the brain lesion in CP progressive?

No, although signs, symptoms, and functional abilities can change as the child ages

When can the brain injury causing CP occur?

Prenatally, during birth, or postnatally up to approximately 2 years of age

What is a major risk factor for CP?

Prematurity, particularly delivery before 37 weeks

What birth weight is associated with increased CP risk?

Less than 2.5 kg (5.5 lb)

What three areas contribute to the diagnosis of CP?

Neurological assessment, neuroimaging, and assessment of posture/movement

What clinical findings may suggest CP?

Failure to reach early motor milestones, abnormal muscle tone, and qualitative differences in movement patterns

What are the 3 major ways CP is classified?

Functional ability

Distribution of motor disability

Type of muscle tone/motor control disorder

What are the major motor-control types of CP?

Spastic, dyskinetic, ataxic, and mixed

Spastic CP is associated with dysfunction in what area?

Motor cortex/cortical sensorimotor areas

What characterizes spastic CP?

Spasticity, exaggerated reflexes, and abnormal patterns of posture and movement

Dyskinetic CP is associated with dysfunction of what structure?

Basal ganglia

What characterizes dyskinetic CP?

Atypical patterns of posture and involuntary, uncontrolled, recurring, and sometimes stereotyped movements

Dystonic: involuntary, sustained or intermittent muscle contraction with repetitive movements and abnormal postures

Athetoid: slow, continuous, twisting movements

Ataxic CP is associated with what area?

Cerebellar lesion/dysfunction

What characterizes ataxia?

Lack of coordination of voluntary movements

What is mixed CP?

Combination of movement disorders, such as spasticity and dyskinesia

Early signs of CP

Early hand dominance

Delayed motor milestones

Persistent primitive reflexes

Scissored legs below 6 mths

Floppiness

Stiffness

What does the GMFCS classify?

A child's gross motor functional abilities

At which GMFCS levels is spasticity generally higher?

Levels IV and V

What does MACS classify?

How a child uses their hands in daily activities

What does CFCS classify?

Communication abilities, including sender/receiver roles, pace, and familiarity of the communication partner

What does EDACS classify?

Eating and drinking ability in individuals with CP

What does VFCS classify?

Visual function

GMFCS Level I

Walks without limitations. Can climb stairs without a railing; running and jumping are possible but speed, balance, and coordination may be limited

GMFCS Level II

Walks in most settings but may need a railing on stairs and have difficulty with uneven surfaces, inclines, crowds, or confined spaces

GMFCS Level III

Walks using a hand-held mobility device in most indoor settings

GMFCS Level IV

Uses mobility methods requiring physical assistance or powered mobility in most settings

GMFCS Level V

The child is transported in a manual wheelchair in all settings

What early findings may occur with spastic diplegia at 3-6 months?

Decreased neck control, stiffness/floppiness, back arching, LE stiffness, and leg crossing

What may be seen after 10 months in infants with spastic diplegia?

Abnormal crawling

Approximately what percentage of children with CP have spastic hemiplegia?

20-30%

What functions are especially affected in spastic hemiplegia?

Hand functions

What LE posture is common with spastic hemiplegia?

Plantarflexion and inversion, often with forefoot adduction → equinovarus

What other problems may accompany spastic hemiplegia?

Sensory abnormalities, seizures, and visual problems

What is associated with spastic quadriplegia?

Severe motor/sensory deficits, cognitive deficits, seizures, and vision problems

What 5 major movement characteristics should be examined in CP?

Muscle tone, muscle strength, skeletal structure/integrity, selective motor control, and postural control

How does CP affect muscle strength?

Children may be unable to generate normal voluntary muscle force or joint torque

Is CP weakness generally greater proximally or distally?

Distally > proximally

What should be prioritized first during the CP examination?

Functional movement assessment of activities important to the child and family

After examining function, what should the PT assess?

Potential body structure/function causes of activity and participation restrictions

What is the major goal of PT for CP?

Improve motor ability for activity and participation at home, school, and in the environment

What oral medications may be used for CP spasticity?

Diazepam, dantrolene, baclofen, and tizanidine

What are other spasticity-management options?

Intrathecal baclofen, Botox injections, and selective dorsal rhizotomy

What are general resistance-training recommendations in the lecture?

1-3 sets × 6-15 reps at 50-85% RM, 2-4×/week on nonconsecutive days

What are the 3 major areas affected in Autism Spectrum Disorder (ASD)?

Social interaction

Communication

Restricted/repetitive behaviors

What diagnoses are encompassed under ASD?

Autism, Asperger syndrome, and PDD

What does DSM-5 require regarding social-communication deficits for ASD?

Deficits in ALL 3 areas:

Reciprocity

Nonverbal communication

Relationships

What percentage of individuals with ASD demonstrate sensory-processing differences?

>80%

When do ASD symptoms typically become evident?

By 2-3 yrs

Severity of autism: level 1

Requiring support: without supports in place, deficits in social communication cause noticeable impairments. Difficulty initiating social interactions, and clear examples of atypical or unsuccessful response to social overture of others. An inablitiy to be flexible in behavior creates functional difficulties.

Severity of autism: level 2

Requiring substantial support: Significant deficits in verbal and nonverbal social communication skills; social impairments apparent even with supports in place; limited initiation of social interactions; and reduced or abnormal responses to social overtures from others. Behavior inflexibility and repetititve behaviors are obvious.

Severity of autism: level 3

Requiring very substantial support: Severe deficits in communication and socialization creating significant limitations in function. Extreme difficulty in responding to change.

Why is early intervention important for ASD?

The young brain has greater plasticity, giving intervention a better opportunity to produce long-term effects

Common clinical features of ASD

Difficulty with eye contact/joint attention

Repetitive movements

Sensory-processing impairments

Language delays

Risk for cognitive impairment

Fine and gross motor difficulties

What sensory responses may occur with ASD?

Hypersensitivity: commonly tactile/auditory

Hyposensitivity: may involve vestibular input or pain

What motor impairments may PTs see with ASD?

Toe walking

↓ postural control

Impaired motor coordination

Lower standardized motor-test scores

Difficulty with ball skills

Overall goals of ASD treatment

Maximize functional independence

Facilitate adaptive-skill acquisition

Minimize/prevent problem behaviors interfering with function and quality of life

Which treatment approach has the most evidence for treating symptoms of autism?

Behavioral approaches

What is OT's major role in ASD?

Sensory integration

Fine motor skills

Life skills

Participation in daily activities

What is the most common genetic cause of Down syndrome?

Trisomy 21 — 3 copies of chromosome 21; accounts for approximately 95% of cases

What neurologic changes are associated with Down syndrome?

Small cerebellum and brainstem

Delayed/reduced myelination in the cortex and cerebellum

Major medical comorbidities associated with DS

Hearing deficits: 75%

Vision deficits: 60%

Congenital heart defects: 50%

Mild-moderate cognitive impairment

Language delay

Seizures

Increased risk of overweight/obesity

In Down syndrome, which language ability is generally stronger?

Receptive > expressive language

Major musculoskeletal characteristics of Down syndrome

Short stature

Hypotonia

Ligamentous laxity

Atlantoaxial instability

Developmental hip/acetabular dysplasia

Inefficient postural control

Increased mobility of C2 relative to C1 associated with ligamentous laxity and inadequate tension of the transverse ligament

Atlantoaxial instability

What is the major danger of AAI?

Spinal cord compression, which can lead to paralysis

What findings should make a PT concerned about symptomatic AAI/spinal cord involvement?

Neck pain

Limited neck mobility

Torticollis

Difficulty walking/abnormal gait

Incoordination/clumsiness

Change in hand function

New sensory impairments

New-onset spasticity

New urinary retention/incontinence

What motor abilities are expected on average around 18 months in DS?

Floor mobility and sitting

What motor abilities are expected around 18-36 months in DS?

Standing and walking

What motor abilities are expected around 3-6 years in DS?

Walking, running, and jumping

What findings are associated with hypotonia in DS?

↓ strength

Hypermobile joints

↑ flexibility

Rounded shoulders

Delayed motor skills

↓ activity tolerance

Leaning on external supports

What postural-control deficits occur in DS?

Inefficient co-contraction strategies

Insufficient balance reactions

Impaired proprioceptive feedback

Slow muscle-onset latency

Increased reaction time

What skeletal alignment problems may occur with DS?

Hyperlordosis

Hip instability

Patellar instability

Genu valgum

Flat feet

What are typical gait characteristics in DS?

↓ gait velocity

↓ stride length

↑ step width

Movement patterns/stages resemble typically developing children, with enough tone, strength, and voluntary control to initiate, adapt, and sustain movement.

Mild hypotonia

Child can initiate/adapt/sustain movement, but movement is less efficient with:

Excessive motion at weight-bearing joints

Wide BOS

↓ balance

Compensatory movements

Moderate hypotonia

Difficulty initiating/adapting/sustaining movement

Limited movement frequency

↓ endurance

Inefficient, compensatory movement patterns

Severe hypotonia

Which outcome measures can quantify motor delay in DS?

AIMS and PDMS-3

What is the most common norm-referenced test for children with DS?

PDMS-3

Which assessment focuses more on functional activity?

PEDI

Which measures assess participation?

CAPE and PAC

Major PT interventions for DS

Infant treadmill training

Strength/coordination exercise

Orthotics

Life skills

Cardiovascular/community programs

Why is tummy time encouraged in infants with DS?

To develop extensor muscles and scapular stabilizers

What tummy-time dosage was associated with decreased overall motor delays?

Early tummy time started before 11 weeks, totaling 90 min/day

When does the lecture suggest generally waiting to begin orthotic intervention in DS?

Until the onset of independent walking

When might orthoses be appropriate BEFORE independent walking?

When biomechanics are severely altered, such as excessive pronation causing poor alignment during standing/cruising

Why is jump training important for children with DS?

Jumping is often difficult but is important for group games and physical activity.

What jump-training dosage improved jump performance and dynamic balance?

3×/week for 6 weeks

What bike intervention was successful for children with DS?

A 1-week camp with 75-minute training sessions; 56-74% could ride a two-wheeled bike >100 ft within 5 days

What was the dosage/effect of dance intervention?

1 hour/week for 20 weeks improved upright motor skills

What is the approximate life expectancy for DS?

60 years

When does federal IDEA postsecondary transition planning begin?

16 years

When does transition planning begin in Illinois?

14½ years old

What long-term health concerns should PTs consider in adults with DS?

Depression/anxiety

Early-onset Alzheimer's disease

Degenerative joint disease

Osteoporosis/osteopenia → fracture risk

Obesity