Alterations in Intracranial Regulation/Neurologic Disorders

Pediatric Differences

• Open Fontanelles:

  • A child with open fontanelles can compensate for increased volume through widening of sutures and skull expansion; however, spatial compensation is limited.

  • Once all compensatory mechanisms are exhausted, further volume increase can result in a rapid rise in intracranial pressure (ICP).

• Persistence or Reappearance of Primitive Reflexes (e.g., Babinski):

  • May be an indicator of a pathologic condition.

• Importance of Obtaining Pregnancy and Delivery History:

  • It is important to obtain a thorough pregnancy and delivery history, as both intrauterine and extrauterine factors can influence how the central nervous system matures.

Neurologic Assessment

• Loss of Consciousness (LOC):

  • ****A change in LOC is the earliest indicator of improvement or deterioration in neurologic status.****

  • Full consciousness: Awake and alert; oriented to time, place, and person; exhibits age-appropriate behaviors.

  • Confusion: Disorientation is present; the child may be alert but responds inappropriately to questions.

  • Obtunded: Limited responses to the environment; falls asleep unless stimulated.

  • Stupor: Responds only to vigorous stimulation.

  • Coma: Cannot be aroused, even with painful stimuli.

• Extreme irritability or lethargy is considered an abnormal finding.

• Vital Signs:

  • Temperature may be variable.

  • Cushing’s Triad (Late Sign):

    • Elevated blood pressure with widened pulse pressure, bradycardia, and irregular respirations.

• Eyes:

  • Assess pupil size, reactivity, and symmetry.

• Motor Function:

  • Observe for spontaneous activity and response to painful stimuli.

• Posturing:

  • Decorticate (Flexion): Extremities flexed toward the “core.”

    • Indicates severe dysfunction of the cerebral cortex or lesions to the corticospinal tracts.

    • Usually happens before decerebrate.

  • Decerebrate (Extension):

    • Indicates dysfunction at the level of the midbrain or lesions of the brainstem.

Glasgow Coma Scale (GCS)

• Most popular coma scale

• Three-part assessment:

  • Eye response

    • ****Response to pain: squeeze the patient’s fingertip, trapezius muscle, or apply pressure to the supraorbital notch.****

  • Verbal response

  • Motor response

    • Best Motor Responses:

      • Moves to localized pain: Attempts to reach toward the painful site, bringing the hand above the clavicle.

      • Flexion withdrawal from pain: Flexes the arm at the elbow to pull away from pain.

      • Abnormal flexion (decorticate): Internal rotation of the shoulder, flexion of the elbow, pronation of the forearm, and wrist flexion.

      • Abnormal extension (decerebrate): Head is extended; arms and legs are extended.

• Score of 15: Unaltered level of consciousness.

• Score of 8 or below: Accepted definition of coma (“Less than 8… intubate.”)

• Score of 3: Deep coma or death.

scores 9 to 14—more frequent neuro checks**

[Neuro] Lab and Diagnostics Tests

• Lumbar Puncture (LP)

• Head and Neck X-ray—(a lot of times our starting point)

• Computed Tomography (CT)

• Ultrasound—(cranial US for open anterior fontanelle)

• Magnetic Resonance Imaging (MRI)

• Electroencephalogram (EEG)—seizure activity

• Intracranial Pressure Monitoring

Signs of Increased Intracranial Pressure (ICP)

Clinical Manifestations in Infants:

• Tense, bulging fontanelle

• Irritability/restlessness

• Drowsiness

• High-pitched/neuro cry**

• Poor feeding

• Setting sun sign (eyes rotated downward)***

• Separated cranial sutures

• Distended scalp veins

Clinical Manifestations in Children:

• Headache

• Nausea/vomiting

• Diplopia, blurred vision—(kids will get closer to things to see)

• Seizures

• Indifference, drowsiness

Late Signs in Infants and Children:

• Bradycardia

• Decreased motor response to command

• Decreased sensory response to painful stimuli

• Alterations in pupil size and reactivity

• Flexion (decorticate) and extension (decerebrate) posturing

• Cheyne-Stokes respirations

• Papilledema

• Decreased consciousness

• Coma

Seizures

Epilepsy:

• Seizures are triggered recurrently from within the brain.

• Many children outgrow seizures, while some will have them for their entire lifetime.

• Goal: Manage seizures while maintaining or improving quality of life.

Diagnostic and Laboratory Testing:

• EEG, Lumbar Puncture, MRI, CT

• CBC, Electrolytes (hyponatremia), Glucose (hypoglycemia), BUN

Nursing Interventions:

• Keep the patient safe: suction, oxygen, rail padding, etc.

• If a seizure lasts longer than 5 minutes, medicate!

• Monitor respiratory status after administering medications.

Management:

• Focuses on controlling seizures or reducing their frequency.

  • Medications: Benzodiazepines, anticonvulsants (e.g., Keppra, phenobarbital, valproic acid, diazepam, lorazepam)

  • Check blood glucose levels and electrolytes; evaluate the underlying cause.

  • Surgery: May involve removal of the area of the brain responsible for the seizure.

  • Diet: Ketogenic Diet

    • High intake of fats, adequate protein, and very low intake of carbohydrates, resulting in a state of ketosis.

    • The child is maintained in a mild state of dehydration.

  • Implant: Vagal Nerve Stimulator

    • A nerve stimulator is implanted, and a lead wire is wrapped around the vagus nerve.

    • The stimulator is programmed to provide the appropriate dose of stimulation at preset intervals; additional stimulation can be administered if needed.

Patient and Family Education:

• No bathing or swimming alone.

• May delay or exclude obtaining a driver’s license. (typically have to be seizure-free for a year)

• Avoid alcohol or illegal drugs.

• Be aware of medication interactions.

Types of Seizures

• Febrile Seizure

• Absence (Petit mal)

• Tonic-Clonic (Grand mal)

• Myoclonic

• Atonic (Drop attacks)

• Status Epilepticus

Febrile Seizure

Most common seizure type in children.

• Occurs between 6 months to 5 years of age**

• No previous neurologic abnormalities.

• Benign and self-limiting**.

• Child will receive a physical examination with appropriate tests during assessment.

• Returns to alert mental status within 3 minutes.

• Rapid rise of fever >100.4°F (38°C)**

• Risk factors: Viral infection, family history.

• Most children will outgrow this condition.

Absence (Petit Mal)

Sudden cessation of activity or speech with a blank facial expression.

Tonic-Clonic (Grand Mal)

Generalized and most dramatic seizure type.

• Often associated with an aura.

• Loss of consciousness, may be preceded by a piercing cry.

• Tonic phase: Entire body experiences sustained contractions.

• Clonic phase: Rhythmic jerking alternating with relaxation of muscles.

• Cyanosis may occur due to apnea.

• Saliva may collect in the mouth due to inability to swallow (suction + oxygen!).

• Child may bite their tongue.

• Loss of sphincter control, especially the bladder, is common (incontinence).

Myoclonic

Sudden, brief muscle jerks.

• May involve the whole body or one body part.

• Child may or may not lose consciousness.

Atonic (Drop Attacks)

Sudden loss of muscle tone.

• In children, may present as a sudden drop of the head.

• Child regains consciousness within a few seconds to a minute.

• Can result in injury due to a sudden, violent fall.

Status Epilepticus

• Neurologic emergency!

• Prolonged seizure or clustered seizures where consciousness does not return between episodes.

  • Using up a lot of energy, so check that glucose!

• Prognosis depends on child’s age, cause of seizures, and duration of status epilepticus.

• Prompt medical intervention is essential to reduce morbidity and mortality.

Postictal Phase

• Child may be semi-comatose or in a deep sleep for 30 minutes to 2 hours

• Responds only to painful stimuli

• No memory of the seizure

• May complain of headache and fatigue

• Safety of the child is a primary concern

Neural Tube Defects (NTD)

• Failed closure of the neural tube around the 3rd or 4th week of gestation.

• May involve the entire length of the neural tube or only a small portion.

Cause:

• Folic Acid Deficiency is responsible for 50% or more of cases.

  • All pregnant mothers should take 0.4 mg of folic acid daily!***

  • A higher dosage is recommended if there is a history of neural tube defects (NTDs).

• Other cases are multifactorial in origin.

Types of Neural Tube Defects (NTDs):

• Anencephaly

• Spina Bifida Occulta

• Spina Bifida Cystica:

  • Meningocele

  • Myelomeningocele

NTD: Anencephaly

• Absence of cerebral hemispheres.

• Brainstem function may be intact.

Incompatible with life:

• Survival typically only lasts a few hours to a few days.

• Death usually occurs due to respiratory failure.

Nursing Management:

• Focuses on supportive care and comfort measures for the dying infant.

• Provide emotional support for parents.

  • An infant cap may be helpful for comfort and bonding.

Categories of Spina Bifida

Spina Bifida Occulta:

• Not visible externally.

• Defect of the vertebral bodies without protrusion of the spinal cord or meninges.

  • May present with:
    • Sacral dimple
    • Hairy patch
    • Skin discoloration (big birthmark)

• Potential complication: Tethered Cord

---

Spina Bifida Cystica:

• Meningocele:

  • Sac contains meninges and spinal fluid, but no neural elements.

  • Spinal cord is usually normal

  • Minor to no neurologic effects

• Myelomeningocele:

  • Sac contains meninges, spinal fluid, and nerves.

  • Varying and often serious neurologic deficits.

  • Spinal cord ends at the level of the defect.

    • Results in absent motor and sensory function below the lesion.

Myelomeningocele: The Sac

• May present as a fine membrane.

• Could be covered with dura, meninges, or skin.

• Location and magnitude of the defect determine the nature and extent of the impairment.

• The sac is prone to leakage of cerebrospinal fluid (CSF) and is easily ruptured.

Precautions:

• Avoid contamination from urine or feces. Use saran wrap, proper positioning, or a urinary catheter.

Management:

• Prevent infection!

  • If the sac is open, use sterile saline or antibiotic-soaked dressings to prevent drying out.

  • Positioning: Keep the baby in a prone position.

• Assess neurologic status and associated anomalies.

• Manage genitourinary function, bowel control, and orthopedic concerns, especially regarding locomotion.

• Early closure of the defect is critical.

• Maintain a latex-free environment.

  • Children with spina bifida are often sensitive to latex due to increased exposure during surgeries and catheterizations.

Skull Deformities

Craniosynostosis: “syn = surgery”

• Premature closure of the cranial sutures.

• Premature closure may lead to decreased brain growth.

• When only one suture is fused, neurologic impairments are rarely seen.

  • When two or more sutures are fused, neurologic complications, such as hydrocephalus with increased intracranial pressure (ICP), are more likely to occur.

• Neurological complications: Hydrocephalus with increased ICP.

• Treatment: Surgery. “sooner rather than later!”

---

Plagiocephaly: “P = positional”

• Positional plagiocephaly refers to asymmetry in head shape without fused sutures.

• The “Back to Sleep” program has led to an increase in this condition.

• Treatment:

  • Increased tummy time when awake.

  • Avoid keeping the baby in a car seat other than when in the car.

  • Molding helmet if necessary.

Hydrocephalus

• Imbalance in the production and absorption of cerebrospinal fluid (CSF) in the ventricular system.

  • With the accumulation of CSF, the ventricles dilate, compressing brain tissue and leading to increased intracranial pressure (ICP).

  • Causes include developmental defects, neoplasms, infections, trauma, or hemorrhage.

Therapeutic Management:

• Ventriculoperitoneal (VP) Shunt Placement:

  • The VP shunt will need to be replaced as the child grows.

  • Be aware of signs of increased ICP in children with VP shunts.

  • Shunts may become occluded, infected, or may break.

• Endoscopic Third Ventriculostomy (ETV):

  • Improves the flow of CSF out of the brain.

  • A tiny hole is made at the bottom of the third ventricle, allowing CSF to divert and relieve pressure.

  • Sometimes done with choroid plexus cauterization to decrease CSF production.

  • Choroid plexus cauterization uses an electric current to burn the CSF-producing tissue (the choroid plexus) in the lateral ventricles, reducing CSF production.

Prognosis:

• Depends on the rate at which hydrocephalus developed.

• The duration of increased ICP.

• The underlying cause of hydrocephalus.

Nursing Care Management:

• Observe for signs of increasing ICP.

• Measure head circumference.

• Palpate fontanelles and suture lines.

Post-Operative Care:

• Pain management.

• Monitor for signs of increased ICP and infection.

• Provide family support and education.

• Contact sports and activities that promote twisting at the waist should be avoided unless specifically approved by their healthcare provider.

Bacterial Meningitis

• Infection of the meninges, the lining that surrounds the brain and spinal cord.

  • Common pathogens: Haemophilus influenzae type B, Escherichia coli, Neisseria meningitidis (meningococcal meningitis)

• Vaccination for Haemophilus influenzae type B and pneumococcal infections has significantly decreased the incidence.

Symptoms:

• Stiff neck** (#1 hallmark sign!!)

• Fever

• Headache

• Vomiting

• High-pitched cry (infants)

• Bulging fontanelle

• Photophobia** (lights are dimmed)

• Rash (petechiae or purpuric)—doesn’t blanch.

  • If petechiae or purpura appear on the extremities, it is likely meningococcal meningitis. Rapid treatment is essential to save the patient’s extremities and life.

• Positive Kernig (kick) and Brudzinski (bend) signs**

Post-Lumbar Puncture Care:

• After a lumbar puncture, the patient should lay flat in bed to prevent CSF leakage and spinal headache.

Lumbar Puncture Results:

• CSF pressure will be elevated.

• CSF will show:

  • Elevated white blood cells (WBCs)

  • Elevated protein (bacterial)

  • *Low glucose (bacteria feed on glucose—so it’s a big sign its a bacterial infection)

Treatment:

• Droplet Isolation:

  • Nurse wears a mask in the patient’s room.

  • Patient wears a mask when outside the room.

  • Isolation maintained until 24 hours after antibiotics have been administered.

• Antibiotics:

  • IV antibiotics started immediately after CSF and blood cultures are obtained.

• Seizure Precautions:

  • Minimize stimuli.

• Monitor:

  • Watch for signs of increased intracranial pressure (ICP).

  • Full monitor (BP, HR, RR, Pulse Ox).

• Fluid Management:

  • Administer fluids cautiously to avoid worsening ICP.

• Fever and Pain:

  • Treat promptly.

• Prevention:

  • Vaccinations: Hib, meningococcal vaccine.

Encephalitis

• Is an inflammation of the brain that may also involve the meninges.

• Can be caused by protozoan, bacterial, fungal, or viral invasion (most commonly viral).

Common Symptoms:

• Fever**

• Flu-like symptoms

• Altered level of consciousness (LOC)**

• Headache**

• Lethargy

• Generalized weakness

• Seizure activity

Diagnostics and Lab Tests:

• Lumbar puncture (LP)

• CBC

• MRI, CT

• Electrolytes

• Glucose

Treatment:

• Antivirals (as the cause is most often viral)

• Seizure precautions (suction + oxygen @ bedside)

• Monitor LOC

Key Points:

• Unlike meningitis, this typically does not involve a stiff neck.

• Altered LOC is almost always present in encephalitis.

• Seizures are common.

• **Clinical hallmark is the triad**:

  • Fever

  • Headache

  • Altered LOC

Reye Syndrome

Cause:

• Most often a reaction triggered by the use of salicylates to treat a viral infection.

• The exact cause of this is not fully understood; salicylates are a common association, but not definitively causal.

Clinical Manifestations:

• Brain swelling

• Liver failure (look for jaundice)

• Potentially fatal (may result in death)

Patient Symptoms:

• Profuse vomiting

• Change in mental status

• Lethargy

• Irritability

• Hyperreflexia

• Seizures

Diagnostic Evaluations:

• Elevated ammonia levels (altered LOC/confusion)

• Elevated liver enzymes (ALT, AST)

• Extended bleeding times (PT/PTT/INR)

• Liver biopsy

Nursing Management:

• Provide care similar to any unconscious child and for increased intracranial pressure (ICP):

  • Seizure precautions

  • IV fluids to correct glucose and electrolyte imbalances

  • Diuretics to decrease ICP

  • Vitamin K for correction of bleeding

  • Plasma and/or platelets as needed

  • Ammonia detoxicants to reduce ammonia levels

  • Anticonvulsants for seizure control

  • Mannitol for increased ICP if indicated

  • Frequent neuro checks

Family Education:

• NO aspirin or aspirin-containing products (e.g., Pepto-Bismol)

• Avoid aspirin-containing products for children under 16 years of age

Cerebral Palsy (CP)

Etiology:

• Describes a range of non-specific clinical symptoms characterized by abnormal motor patterns and postures caused by nonprogressive abnormal brain function.

  • 80% of causes occur before delivery.

  • Can also occur in the natal and postnatal periods.

  • Cause may be unknown.

  • Most common movement disorder of childhood.

  • Wide variation in severity of symptoms.

Complications:

• Mental impairment

• Seizures

• Growth problems

• Impaired vision or hearing

• Abnormal sensation or perception

• Hydrocephalus

Classifications:

• Spastic (most common type):

  • Poor control of posture, balance, and movement.

  • Exaggerated deep tendon reflexes.

  • Hypertonicity of affected extremities.

  • Continuation of primitive reflexes.

  • May fail to develop protective reflexes.

• Dyskinetic:

  • Abnormal involuntary movements; infant appears limp and flaccid.

  • Uncontrolled, slow, worm-like writhing or twisting movements.

  • Affects all four extremities; may include face, neck, and tongue.

  • Movements worsen with stress.

  • Dysarthria and drooling may be present.

• Ataxic:

  • Affects balance and depth perception; poor coordination.

  • Unsteady, wide-based gait.

  • Delayed motor milestones and language skills.

• Mixed Type:

  • Combination of spastic and dyskinetic features.

Therapeutic Management:

• Early diagnosis

• Optimize mobility, communication, and self-help skills

• Provide adapted education

• Promote socialization with peers

• Encourage rest periods to avoid fatigue

• Feeding support (may need special techniques or devices)

• Prioritize safety in home and school settings

• Implement physical therapy

• Provide family support and education

Medical Management:

• Medications:

  • Baclofen: Decreases spasticity

  • Anticholinergics: Decrease involuntary movements

  • For spasticity: Baclofen, Dantrolene sodium, Diazepam

  • Botulinum toxin: Injected into affected muscles to reduce spasticity.

Pediatric Head Injury

Causes:

• Falls

• Motor vehicle injuries

• Bicycle or sports-related injuries

• Child abuse

  • Shaken Baby Syndrome (SBS): Often occurs in infants younger than 9 months.

  • SBS is a form of child abuse; a significant number of head traumas result from it.

Risk Factors:

• Physical characteristics of the child

• Immature motor development

• Natural curiosity and liveliness

• Unattended child

Mild Head Injury:

• No loss of consciousness

• No penetrating injury to the brain

• Typically managed at home with close observation.

  • Signs and Symptoms to Report:

    • Vomiting

    • Slurred speech

    • Changes in ambulation (walking/movement)

    • Headache

    • Fluid draining from the nose or ears

  • Home Monitoring:

    • Check the child every 2 hours for changes in responsiveness during the night.

Severe Head Injury:

• Requires hospitalization until stable.

• NPO (nothing by mouth)

• IV fluids

• No opioids/narcotics given

• Seizure precautions

• Close monitoring of fluid and electrolyte balance

Traumatic Brain Injury Therapeutic Management

Nursing Management:

• Maintain the child’s airway; monitor breathing, circulation, and neurological status closely.

• Implement spinal precautions as indicated.

  • If a patient has a hard cervical collar and its immobilizing their neck, we do not, as the nurse, take it off!

• Treat any other injuries resulting from the trauma.

• Frequently reassess neurological status, paying attention to changes in level of consciousness (LOC) and signs of increased intracranial pressure (ICP).

• Initiate seizure precautions as ordered.

Medications:

• Acetaminophen

• Opioids (as prescribed)

• Anticonvulsants

• Antibiotics

• Corticosteroids (if spinal cord injury)

• Sedatives (not used in the acute phase)

Surgical Interventions:

• Suture of lacerations or torn dura.

• Reduction of fracture and/or removal of bone fragments.

Down Syndrome (Trisomy 21)

• Is a genetic disorder caused by the presence of all or part of an extra 21st chromosome.

• It is the most common chromosomal abnormality associated with intellectual disability.

• Occurs in approximately 1 in 730 live births.

• Higher incidence with maternal age over 35 years.

• Between 1979 and 2003, the number of babies born with Down syndrome increased by about 30%.

Anticipatory Guidance:

• Early intervention services: Occupational Therapy (OT), Speech Therapy (ST), Physical Therapy (PT)

• Routine pediatric well exams and specialist follow-ups

• Family support and education

Common Features of Down Syndrome:

• Varying degrees of intellectual disability

• Hypotonia (poor muscle tone), short stature

• Depressed nasal bridge, small nose

• Protruding tongue (appears large relative to the size of the mouth)

• Single deep transverse crease on the palm (simian crease)

• Short neck with excess skin at the nape

Associated Health Problems:

• (1) Congenital heart disease occurs in 40% to 50% of individuals.

• Most common heart defects:

  • AVSD

  • ASD

  • VSD

  • PDA

  • Tetralogy of Fallot

• Increased risk for:

  • Leukemia (3)

  • Thyroid disorders (2)

Autism Spectrum Disorder (ASD)

• Described as a “spectrum” of disability — ranging from high-functioning to low-functioning.

• Etiology is unknown.

Social-Communication Deficits:

• Non-verbal communication difficulties:

  • Trouble interpreting body language

  • Limited eye contact

  • Lack of facial expressions

• Relationship difficulties:

  • Struggles with adjusting behavior to fit social context

  • Difficulty making friends or engaging in imaginative play

• Social-emotional challenges:

  • Abnormal social approach

  • Poor back-and-forth conversation

  • Failure to initiate or respond to social interactions

Restrictive and Repetitive Behaviors:

• Motor behaviors:

  • Hand flapping

  • Finger flipping

• Other behaviors:

  • Excessive adherence to routines/sameness

  • Highly restricted or fixated interests with abnormal focus or intensity

ASD Severity Levels:

• Level 1 – Requires some support

• Level 2 – Requires substantial support

• Level 3 – Requires very substantial support

Possible Related Conditions:

• Restrictive diets

• Seizure disorders

• Sleep and toileting difficulties

• Self-injurious behaviors

Nursing Implications:

• Early intervention (Speech Therapy, Occupational Therapy, Physical Therapy)

• Develop a plan of care in collaboration with the caregiver.

  • Coping plan: do they like to be touched, hot, cold, etc?

• Avoid physical touch until rapport is built.

• Involve a Child Life Specialist