peds in 862

prosencephalon

• telencephalon

• diencephalon

telencephalon

cerebral hemispheres

• cerebral cortex

• subcortical white matter

• basal ganglia

• basal forebrain nuclei

diencephalon

• thalamus

• hypothalamus

• epithalamus

mesencephalon

• cerebral peduncles

• midbrain tectum

• midbrain tegmentum

rhombencephalon (hindbrain)

• metencephalon

• mylencephalon

metencephalon

• pons

• cerebellum

myelencephalon

medulla

key steps in neural development

• Patterning

• Neuronal migration

• Axonal migration

• Synaptogenesis

• Myelination

patterning

the process that helps decide the fate of the neural precursors, like which become motor neurons or sensory etc.

neural patterning

biological process that cells in developing nervous system get their identities according to their position through morphogens and a concentration gradient

• gradients in cranial to caudal and dorsal (sensory) to anterior (motor)

morphogens

signaling molecules that help with differentiation in pattern generation

neural migration

when mitosis occurs at center of the neural tube, the immature neurons migrate to the outer surface. they migrate by following fibers called radial glial cells. it occurs in parallel with patterning

what happens when there is an issue with neural migration?

this can cause abnormalities with gyri and sulcri

axonal migration

• pathway selection

• target selection

• address selection

pathway selection

immature axon is told to start in the right general direction

target selection

immature axon is given more fine tune directions

address selection

immature axon is given the final destination

chemoattractor

guide cell that is part of the genetic makeup to get where the axon needs to go by attracting it towards it

repellents

guide cell that is part of the genetic makeup to get where the axon needs to go by repelling to axon to go away from it

agenesis of corpus callosum

axons did not know where to go and did not form the corpus callosum

synaptogensis

• more synapses by the age of two than at birth, and double the amount of an adult brain

• synaptic eliminnation

synaptic elimination

optimize and prune those that are not needed

• Interaction between pre- and post-synaptic sites

• Inactive synapses are eliminated

how does nature and nurture impact synaptic connections?

• nature is crucial for neuronal patterning and migration, as well as axonal path and synaptic connection and refinement

• nurture enriches the environment to learn

myelination

• Begins at 28 weeks of gestation

• 36 weeks: posterior limb of internal capsule

• Most CST: 2 years of age

• Continues through adolescence

how does myelination occur

• Inferior to superior

• Posterior to anterior

• Proximal to distal pathways

• Sensory before motor

• Projection fibers before association

Leukodystrophy

a white matter disorder of the brain

A group of inherited neurological disorders, progressive in nature

occular dominance

occurs as retinal input is connected to the lateral geniculate nucleus, of which each of the multiple layers are connected to a single eye. the IV layer connect to the visual cortex and develops occular dominance.

• can be disturbed if one eye is deprived of vision in 1-9 weeks after birth, otherwise no long-term effects

how does enriched environments impact infants

improve motor outcomes for infants at high risk of CP

cerebral palsy

• group of permanent disorders to the development of movement and posture causing limitations in activity due to non-progressive disturbances that occured in developing infant or fetal brain

• motor disorders accompanied by disturbances in sensation, perception, cognition, communication, and behavior, by epilepsy and secondary MSK problems

classification of CP

• muscle tone

• distribution of limb involvement

types of muscle tone

• Hypertonia

• Hypotonia

• Dystonia

• Ataxia

• Choreoathetosis

distribution of limb involvement

• Monoplegia

• Diplegia

• Hemiplegia

• Triplegia

• Quadriplegia

Dystonia, ataxia, choreoathetosis

accompiaed by writhing movements

monoplegia

single limb

diplegia

both LE

hemiplegia

one side of the body

triplegia

1 arm and both LE

quadreplegia

both UE & LE

Incidence and Prevalence of CP

• CP is the most common motor disorder in childhood

• About 18 million people in the world have CP

• About 3 in 1000 children have CP

• Around 8,000 to 10,000 babies and infants are diagnosed per year with cerebral palsy

most common CP

• spastic

• diplegia over quadreplegia

prenatal etiologic risk factors

• Infection

• Maternal diabetes or illness

• Abnormal placental attachment

• Congenital abnormalities of the brain

• Clotting abnormalities

• Intrauterine growth restriction

perinatal etiologic risk factors

• Prematurity

• Obstetric complications

• Umbilical cord abnormalities

• Low birth weight

• Low Apgar scores

• Placenta previa or abruption

• Maternal illness such as preeclampsia

postnatal etiologic risk factors

• Neonatal infection

• Environmental toxins

• Trauma

• Kernicterus

• Anoxia

• CVA

• Neonatal hypoglycemia

• Acidosis

pretoerm infant vulnerability

due to the structural and functional immaturity of blood vessels, preterm infants are susceptible to ischemic and hemorrhagic injury

arterial and venous system of preterm infants

arteries grow from the outside in and are very fragile to a rupture. An occlusion would cause the central to not receive any blood

veins do not have pressure regulation to take the blood and waste out. Germinal matrix does not have great arterial access

what causes preterm infant vulnerability

• Anatomic underdevelopment of cerebral vasculature

• Physiologic immaturity of cerebral vasculature

• Susceptibility of cardiovascular system

• Oligodendrocyte development

Physiologic immaturity of cerebral vasculature

• Less autoregulation of cerebral blood flow

• Increased risk of underflow/overflow

• Affected by systemic blood pressure

Susceptibility of cardiovascular system

• Ineffective myocardial function

• Delayed closure of patent ductus arteriosis and foramen ovale

• Can cause fluctuations of blood pressure and systemic oxygenation

Oligodendrocyte development

• Responsible for myelination

• Susceptible to oxidative stress

• Injury can result in disruption of future myelination of white matter

Periventricular leukomalacia (PVL)

• Primary arterial ischemic injury to arterial end-zones

• Affects immature white matter

• Ischemia causes necrosis to all cell types within area of injury and to adjacent axonal pathways

• Loss of oligodendrocytes

• Usually bilateral; frontal and/or parietal

• Frontal

• LEs > UEs

• Spastic Diplegia

• May affect trunk and UEs

• Cognition often spared

• Parietal

• Cognition and vision

• May affect moto

Intraventricular Hemorrhage (IVH)

• Grade I Bleed in the germinal matrix (GMH)

• Grade II Bleed extends into lateral ventricles with no hydrocephalus

• Grade III Bleed extends into lateral ventricles with subsequent hydrocephalus

• Grade IV Parenchymal hemorrhage/Periventricular hemorrhagic infarction(PVHI)

complications pf IVH

• Periventricular hemorrhagic infarction (PVHI)

• Obstructed drainage in terminal vein can cause large areas of ischemia

• Typically unilateral

• Severe involvement in that limb(s)

• Post-hemorrhagic hydrocephalus

• Blood obstructs CSF pathways

• Ventricular distension

• Compression/possible ischemia of white matter

• LEs more affected than UEs

Cerebellar Injury

• Likely a form of germinal matrix hemorrhage

• Could also be infarction followed by hemorrhage

• Present with hypotonia or ataxia

Inflammatory Injury

Infection and subsequent inflammation releases cytokines

• Toxic to developing oligodendrocytes

• May affect circulation and predispose to ischemia

• Events of ischemia cause release of cytokines, further cascading injury

Additional Non-motor Symptoms

• Cognitive and learning deficits in 25-50% of kids

• More common in severe bilateral injury or when vision/auditory also affected

• Risk of epilepsy

• Cognitive/language/behavior challenges with cerebellar lesions

• Up to 40% may exhibit features of autism-spectrum disorder

full term infant brain injury

• Cerebrovascular system is anatomically mature

  • Areas most vulnerable to ischemia are areas supplied by ACA, MCA, PCA

• Oligodendrocytes more mature

  • Developing neurons in deep grey matter of cortex and brainstem susceptible to ischemia due to high demand of glucose and oxygen

Cerebrovascular injuries

• Global hypoxia/ischemia

• Ischemic infarct/stroke

• Hemorrhage

Global hypoxia/ischemia

• Fetus is equipped with “brain-sparing” system of blood flow so if brief interruption of blood supply, brain tissue is still supplied

• Blood diverts from major organs to the brain

• Areas supplied most are those that need it most

• Basal ganglia, thalamus, brainstem, sensorimotor cortex

brief/incomplete

may have no deficits

prolonged/incomplete

• Parasagittal watershed and white matter injury;

  end-organ damage

• distal may have damage, like kidney failure

Brief/complete

• Injury to areas with greatest demand (basal ganglia, thalamus, brainstem, sensorimotor cortex);

• May have little to no organ damage

Stroke (ischemic or hemorrhagic)

• Arterial Ischemic Stroke (AIS)

• Cerebral Venous Sinovenous Thrombosis (CVST)

• Intracranial Hemorrhage (ICH)

AIS: Arterial Ischemic Stroke

• Embolus

• Thrombus

• Clotting disease

• Narrow or damaged arteries

Cerebral sinovenous thrombosis (CSVT)

• Clotting in the venous system

• May be transient or can cause an ischemic stroke or intracranial hemorrhage

ICH: Intracranial hemorrhage

• Aneurysm

• Arteriovenous Malformation

• Damaged or fragile blood vessels

• Clotting abnormality

causes of stroke in full term infant

• Cardiac disease (25%)

• Arteriopathy

• Inflammatory attack

• Dissection and clot formation

• Intravascular

• Blood abnormalities

• Trauma/clotting at birth

linking pathology to outcomes

• Typical clinical presentations based on history or MRI

• Potential for neuroplasticity

• Child is a constantly changing system

  • Maximize development

  • Minimize secondary complications

  • Lifelong follow-up

what contributes to milestone acquisitoion?

• MSK factors

• enviormnet/exposure

• predispotion

Developmental Domains

• Sensory

• Cognitive

• Motor (fine and gross)

• Communication

gestational age

time from 1st day of last period to birth

typicaaly 40 for calculation

Postmenstrual age

time from 1st day of last period to date of assessment

age adjusted

time from expected date to date of assessment

what outcome measure allows for age adjusting

PDMS-2 until 2 years old

how do we measure development

• Observation

• Comparing to typical development

• Screening tools

• Standardized assessments

what occurs during development screening

• Checklist

• Milestones

• Validated measures of global development

where does developmental screening typically occur?

• Physician well visit

• Daycare/Preschool milestones

• School referral

Ages & Stages Questionnaire – 3rd Edition

• for 1-66 months

• has 6 items in a few categories: Communication, Gross Motor, Fine Motor, Problem Solving, Personal-Social

• can use age adjust

role of screening

• Education

• Referral

• Examination and Intervention

standardized assessments

• used to identify or quantify atypical development

• can determine the presence of a diagnosis

• goals change with growth and maturation

Pediatric Clinical Specialization

• APTA Specialist Certification

• American Board of Physical Therapy Specialties

• Minimum 2,000 hours of direct patient care or Pediatric Residency path

• Board examination

why use a standardized test?

• screening purposes

• identify or justify need for services

• evaluate progress or end a course of care

• provide an objective measure

what is different of pediatrics than adults?

• expectations change with growth so there is a changing system

• need to consider trajectory of expected development vs. actual development

pyschometric properties

• Validity

• Reliability

• Internal consistency

• External (test-retest; inter-rater)

• Sensitivity – screening and don’t want to miss anyone

• Specificity – important with expensive or higher risk interventions

test type

• norm-referenced

• criterion-referenced

norm-referenced

• relative to a certain population

• must be administered in a standard manner

• will have percentiles rank

• can define age equilvalents

• follows normative distribution

age equivalents

age of the score would be an average performance

criterion-referenced

• relative to an established criteria

• looks at change in the individual

• might have standard instruction in administration

• useful over a period of time

considerations for test selection

• Patient’s age

• Purpose of testing

• Diagnosis or medical and birth history

• Setting

• Space, time and availability of test materials

• Behavior and functional abilities of the child

what might be some additional documentation for pediatric assessments?

• Standardized assessments are one piece of the puzzle

• Was the test able to be administered properly?

• Do you think you saw the child’s typical performance?

• Are there outside factors that may justify (or deny) the need for PT services?

neonate standardized tests

• General Movements Assessment (GMA)

• Movement Assessment of Infants (MAI)

• Neonatal Individualized Developmental Care and Assessment Program (NIDCAP)

• NICU Network Neurobehavioral Scale (NNNS-II)

• Test of Infant Motor Performance (TIMP)

infant/toddler standardized tests

• Alberta Infant Motor Scale (AIMS)

• Peabody Developmental Motor Scales 2 (PDMS-2)

school age standardized tests

• Bruininks–Oseretsky Test of Motor Proficiency 2 (BOT-2)

• Gross Motor Function Measure (GMFM)

• Peabody Developmental Motor Scale 2 (PDMS-2)

impairment-level measures

• Range of motion

• Strength testing using handheld dynamometer

• Assessment of muscle tone using Modified Ashworth

• Pediatric Balance Scale

• Pain using the FACES scal

Activity measures

• Alberta Infant Motor Scale (AIMS)

• Peabody Developmental Motor Scales 2 (PDMS-2)

• Bruininks–Oseretsky Test of Motor Proficiency 2 (BOT-2)

• Gross Motor Function Measure (GMFM)

participation measures

Children’s Assessment of Participation and Enjoyment (CAPE/PAC)

AIMS

• identifies gross motor delay and performance over time

• norm-referenced and observational

• for birth to 18 months

• 58 test items in prone, supine sitting, and standing that do not have to be administered in order

• observes WB, posture, and anti-gravity movement

• can complete across sessions if less than one week apart

• infant should be naked or in a diaper

• find the infants motor window and score observed or not observed, where they get a point

what is abnormal for AIMS

below 5th percentile

PDMS-2

• identifies gross and fine motor delay and assess performance over time

• norm-referenced

• birth to 6

• requires a standardized kit and must be completed within 5 days since start of testing

• composed of at least 3 gross motor composites: reflexes, stationary, locomotion, object manipulation

• score a 2 based on criteria, finding basal and ceiling levels

BOT-2

• identifies motor impairment and assessed performance over time

• norm referenced and has short-form available

• 4-21 years

• has a standardized kit, can have 2 trials

• has 4 composites: fine manual control, manual coordination, body coordination, strength and agility

GMFM

• assess motor performance in children with CP

• criterion-referenced, 88 and 66 items

• 5-16 or any child with motor skills at or below a 5-year old without a motor disability

• 4 collections of items through a performance algorithm

• 5 dimensions: A. Lying and rolling, B. Sitting, C. Crawling and kneeling, D. Standing, E. Walking, running, and jumping

• has a standardized booklet

• can be used against motor growth curves