Pediatric Cognitive Behavioral and Neuromuscular Impairments

Cognitive Impairment, Autism, Attention Deficit Disorder and Cerebral Palsy

Developmental Delay

Any significant lag in a child’s physical, cognitive, behavioral, emotional or social development when compared to dev. norms.

Cognitive Impairment

Is a permanent impairment encompassing cognitive ability and adaptive behavior that are functioning significantly below average

Typical delay/impairment

In 2+ Areas (sensory, speech, orthopedic, disability, seizure)

Requires a thorough history & professional evaluation

Prenatal Causes of CI

Chromosomal Causes:

• Trisomy 21/Downs Syndrome- extra chromosome at the 21st spot

• Fragile X

Biological/Biochemical Causes

• PKU (phenylketonuria)

• Metabolic disorders

• Hypothyroidism

• Endocrine disorder

Fetal Alcohol Syndrome

Trisomy 21/Down Syndrome

Chromosomal abnormality that causes approximately 95% of all cases of Down syndrome

Not hereditary

Greater risk in older woman over 35 years of age, however, the majority of infants with Down syndrome are born to women younger than 35 due to higher fertility rates in that age group

Average IQ: 50

Additional Causes of Cognitive Impairment

Traumatic Brain Injury

CNS infection (meningitis)

Hypoxia

Prematurity, low birth weight, post maturity

Maternal infections (rubella, CMV)

Lead ingestion

Environmental deprivation

Childhood psychiatric disorders that develop, such as Autism(ASD)

Clinical Manifestations of Cognitive Impairment

Growth and Developmental milestones delayed

Decreased alertness to voice, movement, abnormal eye contact

Altered sensory, speech, orthopedic, disability, seizures

Requires history & evaluation at various times

Severity (IQ tests) and Educational Potential screening

Assist the parents, teachers, nurses to guide therapy

IQ Test

Intelligence Quotient Test

Wechsler Intelligence Scale for Children provides the score and is the actual test name

Score of 50-75 IQ

Mild impairment

Score of 35-55 IQ

Moderate impairment

Score of 20-40 IQ

Severe impairment

Score below 20-25

Profound impairment

Cognitive Impairment Goals

Parents will have an increased understanding of the condition and the appropriate treatment plans and interventions.

Early intervention programs will be utilized to promote optimal development of the child.

Assistance and guidance will be available to the child and family in order to provide an environment that fosters the child’s optimal development.

The family will be knowledgeable regarding appropriate support and community resources

Diagnosis & Testing of Cognitive Impairment

Health & Physical

Evaluation of developmental level & adaptive functioning

Full chromosome analysis- 85% of the diagnosis can be identified by 12 weeks.

Blood enzyme level

Cranial imaging

***must rule out other problems**

Planning/Implementation of Cognitive Impairment

• Safe environment – developmentally appropriate

• Appropriate Education/Training IDEA:1999

  • Promote the child’s optimal functioning

• Family support

  • Self-care skills

  • Play and exercise

  • Provide appropriate modes of communication

  • Establish discipline

  • Encourage socialization

  • Provide info on sexuality

• Encourage use of resources

Autism Spectrum Disorder

• A complex developmental disability

• Occurs in 1/54

• 5x more in males

• Screen at 2-4 years of age

Possible Causes of Autism 

• Link between hereditary, genetic, and medical problems

• Correlation with fragile X, tuberous sclerosis, metabolic disorders, fetal rubella syndrome, meningitis, hyperbilirubinemia, untreated PKU

• Genetic factor vs. familial occurrence

• Typically appears during first 3 years (true etiology unknown)

Fragile X

The most common cause of inherited cognitive impairment. Common clinical features include a typical facial appearance, with an elongated face and large ears; macroorchidism in adolescent males; connective tissue dysplasia; and behavioral problems, including autism spectrum disorder.

Fragile X Medical Management

Carbamazepine (Tegretol) or fluoxetine (Prozac), to control violent temper outbursts

Clonidine (Catapres) to improve attention span and decrease hyperactivity, and melatonin for sleep difficulties.

Fragile X Nursing Care

There’s no cure, can inform about genetic counseling since it is hereditary. In addition, any male or female with unexplained or nonspecific mental impairment should be referred for genetic testing and, if needed counseling.

Tuberous Sclerosis

A genetic disorder that causes the formation of non-cancerous (benign) tumors in multiple organs, including the brain, skin, kidneys, heart, and lungs.

Fetal Rubella Syndrome

Vision issues: clouded eyes (cataracts), glaucoma, blindness

Heart issues: narrowed pulmonary artery

Growth/development issues: low birthweight, microencephaly, developmental delays

Other signs: enlarged liver and spleen, blood disorders like a low platelet count, and skin rashes may be present.

Meningitis

Can cause visual impairment

Hyperbilirubinemia

An excessive level of accumulated bilirubin in the blood and is characterized by jaundice, or icterus, a yellowish discoloration of the skin and other organs

Untreated PKU (Phenylketonuria)

(Can’t break down phenylalanine)

An inadequate nutrition and metabolic disorder that can lead to cognitive impairment.

A treatable inborn error of metabolism for which newborn screening is used as a strategy to prevent developmental disabilities

Clinical Manifestations of Phenylketonuria

Growth failure (failure to thrive)

Frequent vomiting

Irritability

Hyperactivity

Unpredictable, erratic behavior.

Older children commonly display bizarre or schizoid behavior patterns such as fright reactions, screaming episodes, head banging, arm biting, disorientation, failure to respond to strong stimuli, and spasticity or catatonia-like positions.

Severely cognitively impaired children: Seizures

5 Disorders of Pervasive Developmental Disorder

• Autistic Disorder:

• Asperger’s Disorder: Characterized by impairments in social interactions and the presence of restricted interests and activities, with no clinically significant general delay in language, and testing in the range of average to above average intelligence.

• Childhood Disintegrative Disorder (CDD): Normal development for at least the first 2 years, significant loss of previously acquired skills.

• Rett’s Disorder: A progressive disorder that, to date, has occurred only in girls. Period of normal development and then loss of previously acquired skills, loss of purposeful use of the hands, replaced with repetitive hand movements beginning at the age of 1 - 4 years.

• PDD: Not Otherwise Specified – (PDD-NOS) — atypical autism

Autism Spectrum Disorder Clinical Manifestations (TIme)

Some children will demonstrate characteristics associated with ASDs within the first few months of life. In others, symptoms may not be detected until 24-36 months

Autism Spectrum Disorder Clinical Manifestations

• Altered social interactions:

  • Inability to make eye contact

• Failure to develop peer relationships

  • Lack of spontaneous seeking of enjoyment

• Lack of emotional reciprocity

• Altered behavior

  • Repetitive, aggressive, and/or dangerous

• Altered Communication:

  • Speech/language delays

  • Echolalia

  • Difficulty with reciprocal communication

Autism Spectrum Disorder Diagnosis and Testing

Brain scans

Visual and Hearing evaluations

Eye Hand coordination tests

Blood Lead Levels

Classifications based on characteristics of behavior

Identification of Risk factors

Autism Spectrum Disorder Nursing Diagnosis

Social Isolation

Risk for Injury

Ineffective Coping

Altered Family Process

Altered Verbal communication

Autism Spectrum Disorder Planning & Nursing Interventions

• Early intervention

• Diet changes

• Highly structured environment

• Social communication

• Referral to Autistic Society of America

• Family Counseling

• Medications –may be recommended if the child has an inability to focus, exhibits extreme hyperactivity, depression, or has seizures

• Applied behavior analysis (ABA)/ Behavioral Modification

• Treatment and education of autistic and communication-related handicapped children

• Occupational therapy

• Sensory integration therapy

• Speech therapy

• The Picture Exchange Communication System (PECS)

Attention Deficit Disorder Risk Factors

• Unknown yet possibilities include:

• Brain chemistry: dopamine dysfunction

• Genetics; history of alcohol abuse; mood disorders

• Maternal smoking, alcohol, drugs, and prematurity

• More males than females

Attention Deficit Disorder Clinical Manifestations

• Inattention

• Distractibility

• Impulsive behavior

• May/may not be hyperactive

• Often blurts out answers before questions have been completed.

• Often has difficulty awaiting turn.

• Often interrupts or intrudes on others (e.g., at school or work and at home)

Attention Deficit Disorder Diagnosis and Testing

IQ tests (elevated IQ common)

Eye/hand coordination tests

Hearing/vision

Memory, comprehension

Attention Deficit Disorder Nursing Diagnosis

• Ineffective coping

• Altered family process

• Social Isolation

• Disturbed self-esteem

• Risk for Injury

Attention Deficit Disorder Planning and Nursing Interventions

Regular/controlled routines

Behavioral Modifications

Decreased Stimuli

Medications: (teach side effects)

Attention Deficit Disorder Medications

Stimulants: Ritalin, Dexedrine, Concerta, Metadate, Focalin, Adderall

Selective norepinephrine reuptake inhibitors (SNRIs): Strattera (Amoxetine)

Stimulants: Ritalin, Dexadrine, Concerta, Metadate, Focalin, Adderall

Makes the brain produce more norepinephrine

Side effects: 

• Appetite loss

• Abdominal pain

• Headaches

• Sleep disturbances

• Growth velocity- the speed at which a child's height and weight increase

Sudden death (reported in children with structural cardiac abnormalities), circulatory collapse, exfoliative dermatitis, anorexia, liver failure, psychologic dependence

Selective norepinephrine reuptake inhibitors (SNRIs): Strattera

Prevents norepinephrine from being degraded in the synapse

Side Effects:

Headache, insomnia, upper abdominal pain, vomiting, decreased appetite, dry mouth

Severe liver injury (rare), suicidal ideation

Attention Deficit Disorder Goals

• Parents and child will have an increased understanding of the condition and treatment plan.

• Child will be able to function effectively in the home and educational environment.

• Child will learn to identify their areas of weakness and learn to compensate for them.

Cerebral Palsy Types

Spastic- tense contracted muscles; hypertonicity (most common)

Athetoid- constant, uncontrolled motion of the limbs, head, and eyes

Ataxic- poor sense of balance, often causing falls and stumbles

Rigidity- tight muscles that resist effort to make them move

Tremor- uncontrollable shaking, interfering with coordination

Cerebral Palsy Causes

Congenital; acquired and brain damage

Hemiplegic

Arm and leg on one side of the body

• Arm bent; hand spastic or floppy, often of little use

• Walks on tiptoe or outside of the foot on the affected side

Paraplegic

Both legs only

• Upper body is usually normal or with very minor signs

• Child may develop contractures of the ankles and feet

Diplegic

Slight involvement elsewhere

Quadriplegic

Both arms and both legs

• Arms, head, and even his mouth may twist strangely

• Often have such severe brain damage that they never are able to walk

• Knees press together

• Legs and feet turned inward

Cerebral Palsy Contributing Factors

Maternal:

• LBW; prematurity

• Multiples

• Infections

• Blood incompatibility

Infant:

• Breech presentation

• Complicated birth

• SGA- Small for gestational age

• Low APGAR- Under 7

• Jaundice

Low APGAR

Under 7

Mild Apgar 

7 - 9

High Apgar

9 - 10

Cerebral Palsy Clinical Manifestations

• Muscle coordination problems

• Seizures (~50%)

• Delayed growth and development

• Cognitive impairment (~2/3)

• Spinal deformities

• Impaired vision, hearing, speech

Cerebral Palsy Diagnosis/Testing

• CAT scans and MRIs can identify lesions in the brain.

• Increased % of children with cerebral palsy diagnosis may take months/years to confirm

• Delay in reaching developmental ”milestones” such as rolling over, sitting and standing

• May feel unusually stiff or floppy

• A diagnosis of CP is unlikely to be given until the child’s progress is observed over a period of time and other conditions are ruled out

Cerebral Palsy Nursing Diagnosis

• Impaired physical mobility

• Self care deficit

• Risk for injury

• Impaired verbal communication

• Interrupted Family process

• Disturbed body image

Cerebral Palsy Planning & Nursing Interventions

• Early recognition - Early intervention

  • Physical therapy, Occupational therapy

• Prevent/Correct defects

  • Orthotics, Casts, and Splints

  • Surgery

• No “cure”

• Medications for seizures, and/or spasticity

• Promote socialization

• Adequate Nutritional Support

• Adaptive Equipment

  • Wheelchairs (manual, power and sports)

  • Scooters

  • Specially made bicycles and tricycles

  • Walkers and crutches

• Communication Devices include:

  • Symbol boards- for the nonverbal 

  • Voice synthesizers

  • Head sticks and key guards for computers

• Antispasmotics- bacellfen, diazefam (valium) 

• Depakote, trileptol for seizures

Cerebral Palsy Goals

• Parents will have an increased understanding of the condition and the appropriate treatment plan and interventions.

• Early intervention programs will be utilized to promote optimal development of the child.

• The child will be assisted to realize their potential within the limits of their existing health problems.

• The family will be knowledgeable regarding appropriate support and community resources.