Neurological Neurological AssessmentAssessment

andandDisordersDisorders

Fetal Brain DevelopmentFetal Brain Development The brain is one of the earliest organs formed during

embryonic development Week 3 - beginning development of the brain, spinal

cord, and heart Weeks 4 to 5 - formation of tissue that develops into

the vertebra and some other bones At 7weeks the medulla, pons, and midbrain is

formed By 9 wks, the fetus will display spontaneous mvmt 10 wks – begins to breathe 13 wks - sucking motions are made with the mouth 20 wks – responses to sound

NutritionNutrition Certain nutrients have a important roles in brain

development; some have greater impact than others Protein, fats, iron, zinc, iodine, vitamins A, B6

&K, folate Any nutrient deficit effect is dependent on

timing, doseand duration

Timing in terms of brain development process Timing in terms of prevalence of nutrient deficit in

population Little evidence currently for enhancement of brain

development in typically developing humans

Anatomy and Physiology of Anatomy and Physiology of the Brainthe Brain

Brain anatomy Composed of 2 major areas

Cerebellum Cerebrum

Anatomy and Physiology of Anatomy and Physiology of the Brainthe Brain

Cerebellum Promotes intregrative muscle function Maintains balance Enables smooth, purposeful movements

Anatomy and Physiology of Anatomy and Physiology of the Brainthe Brain

Cerebrum Central hemispheres with 4 lobes

Parietial Frontal Occipital Temporal

Corpus Callosum – fiber bundles connecting the cerebral hemispheres

Cerebral Cortex The ‘mind’, intellect Grey matter Third ventricle Thalamus (integrates sensory input) Hypothalamas (regulates body tempature)

Anatomy and Physiology of Anatomy and Physiology of the Neurological Systemthe Neurological System

Brain Anatomy Brain stem

Relays input and output signals between the higher brain and the spinal cord.

It has three main components The medulla, which controls areas of the abdomen,

thorax, neck, throat, and mouth, and is part of cranial nerves VIII, IX, X, XI, and XII

The pons, which carries information between the brainstem and cerebellum

The midbrain, which is involved in eye movements

Glucose Glucose Glucose metabolism

The neonatal brain is glucose dependent and the serum glucose provides the brain with a glucose pool.

The body preferentiallypreferentially pumps glucose against the gradient to the brain and cerebral metabolism is influenced by the availability of glucose and oxygen.

Anything that causes inadequate cerebral perfusion will compromise the glucose and oxygen supply to the brain.

Anaerobic metabolism causes lactic acid build-up and produces significantly smaller amounts of energy.

The premature infant has minimal-to-no glucogen stores and a less efficient glucose uptake mechanism.

Physiology of the Physiology of the Neurological SystemNeurological System

Cerebral blood flow Affected by pH, K, hypoxemia,

osmolarity,and Ca The brain will increase blood flow to spare

itself inadequate supply of oxygen and electrolytes.

Low pH, hypoxia, hyperkalemia, and increased osmolarity will cause an increase in cerebral blood flow.

Increased Ca ion causes a decrease in cerebral blood flow.

Physiology of the Physiology of the Neurological SystemNeurological System

Autoregulation Maintains a steady-state of cerebral blood flow

despite systemic blood pressure changes. Limited or impaired autoregulation exists in

premature or sick newborns. Without autoregulation systemic blood pressure

regulates cerebral perfusion. Hypotension will lead to ischemia, which

damages blood vessels, and surrounding tissues. When adequate blood supply resumes,

hemorrhage can occur into the ischemic areas. Hypertension without autoregulation will

increase cerebral flow rupturing blood vessels leading to hemorrhage

Neurological AssessmentNeurological Assessment

Determination of state, posture, and evaluation of

movements

ReflexesReflexes Assessment is based

on gestational age Reflexes

Root Suck (begins at

~32wks, intact by ~36wks)

Moro Tonic neck Grasp (very strong in

premmies) Babinski Hand- Mouth Others

The presence or absence of these reflexes are indicative of: Gestational age Neurological

abnormalities Progression of care

Neurological AssessmentNeurological Assessment

State Deep sleep, drowsy, quiet-alert, active, crying,

or active with stimulation Posture

Posture is determined by gestational age The more premature infant has extended, open

posture, reflecting diminished tone. The more term infant has flexed posture, reflecting

adequate tone Abnormal posture includes hyperextension,

asymmetry, and flaccidity.

Neurological AssessmentNeurological Assessment Evaluation of movements

Should be symmetrical with smooth, coordinated quality. Note if jittery or tremulous, or if seizure activity is present.

Respiratory activity is observed for any signs of distress or apnea.

Cranial nerve function assessment. Corneal reflex indicates intact cranial nerves V and

VII. An absent corneal reflex with tonic neck reflex present is associated with severe brain damage

Blink reflex requires intact cranial nerves III and VII. Tongue movement, suck, swallow, gag, and cry are regulated by cranial nerves IX, X, XII

Muscle tone: Evaluate head lag, ventral suspension, clonus and recoil from extension. Check symmetry and briskness vs. flaccidity

SeizuresSeizures

SeizuresSeizures

Result from excessive simultaneous electrical discharge or depolarization of neurons; can have many different causes. Metabolic: Due to ischemia, hypoxemia,

hypoglycemia, hypo- or hypernatremia, hypocalcemia, and hypomagnesemia.

Structural: Due to IVH, intrapartum trauma or hypoxic ischemic encephalopathy.

Intracerebral meningitis. Withdrawal from maternal drug use

IncidenceIncidence

Incidence of neonatal seizures in term vs preterm infants 1.5-3.0 per 1000 live term births 50-150 per 1000 live preterm births

Incidence as a function of birth weight 57.5 per l000 in infants < 1500 grams 2.8 per 1000 in infants 2500 to 3999 grams

IncidenceIncidence

Precise frequency is difficult to delineate Most of neonatal seizures we know is

based on clinical observation EEG monitoring with video recorder or

direct inspection showed Some clinical seizures do not have EEG

correlation Some electrographic seizures are not

accompanied by clinical seizures

HistoryHistory Family history may suggest genetic syndrome

Many of these syndromes are benign In the absence of other etiologies, family history

of seizures may suggest good prognosis Pregnancy history is important

Search for history that supports TORCH infections, history of fetal distress, preeclampsia or maternal infections

Apgar scores offer some guidance Low Apgar score without the need for

resuscitation and subsequent neonatal intensive care is unlikely to be associated with neonatal seizures

What are the causes of neonatal seizures ?What are the causes of neonatal seizures ?

Cause Frequency

Hypoxic-ischemic encephalopathyIntracranial hemorrhageCerebral infarctionCerebral malformationsMeningitis/septicemiaMetabolic Hypoglycemia Hypocalcemia, hypomagnesemia Hypo/Hypernatremia Inborn errors of metabolism Pyridoxine deficiency Kernicterus HyperammonemiaMaternal drug withdrawalIdiopathicBenign idiopathic neonatal seizuresNeonatal epileptic syndromesCongenital infectionsUnintentional injection of local anesthetic during laborMaternal vitamin D deficiency

30-53%7-17%6-17%3-17%2-14%

0.1-1.5%4-22%

3-4%

1%

4%2%1%

Clinical PresentationClinical Presentation

May be subtle tonic, multifocal, focal clonic, or myoclonic seizure activity

Subtle SeizuresSubtle Seizures Selected Major Manifestations

Ocular phenomena Tonic horizontal deviation of eyes with or without

jerking of eyes Sustained eye opening with ocular fixation

Oral-buccal-lingual movements Chewing, sucking, lip smacking

Limb movements Cycling, swimming, rowing

Autonomic phenomena Increase in blood pressure, brady/tachycardia

Apneic spells

Clonic SeizuresClonic Seizures

Usually involve one limb or one side of the body jerking rhythmically at 1-4 times per second

Focal Clonic Seizures Well localized clonic jerking Infant usually not unconscious

Multifocal Clonic Seizures Involves several parts of the body in

migrating fashion (non-ordered fashion) Simultaneous or in sequence

Focal and Multifocal Focal and Multifocal MyoclonicMyoclonic

Focal and Multifocal Myoclonic Seizures Well localized, single, multiple, migrating jerks

usually of limbs Usually not accompanied by EEG seizure

discharges Rare in neonates

Focal Typically involve flexor muscle of upper extremity Uncommon

Multifocal Asynchronous twitching of several parts of the

body Characteristic in full term infants with hypoxic

ischemic encephalopathy (HIE).

Tonic SeizuresTonic Seizures Characteristic of infants < 2500 gms and

seen with severe IVH Focal Tonic Seizures

Sustained posturing of limb Asymmetrical posturing of trunk and neck

Generalized Tonic Seizures Tonic extension of upper and lower limbs

(mimics decerebrate posturing) Tonic flexion of upper limbs and extension of

lower limbs (mimics decorticate posturing) Most are not accompanied by EEG seizure

discharges (only 30% have EEG correlates)

SeizuresSeizures Patient Care:

Positively identify movement as seizure activity by trying to stop movement with gentle passive flexion of the affected extremity

If the movement stops, it was not seizure activityIf the movement stops, it was not seizure activity Check lab work; electrolytes, glucose, ABG,

and septic work up. Administer Phenobarbital or Valium as

ordered. Monitor oxygenation and vital signs. During a

seizure, an infant’s blood pressure will rise, O2 saturation will drop, and CO2 will increase

Document carefully. Educate and support the family.

Jitteriness vs SeizureJitteriness vs SeizureClinical Feature Jitteriness Seizure

Abnormality of gaze or -- + eye movementMovements stimulus + --

sensitivePredominant movement Tremor

Clonic jerkingMovements cease with passive

flexion + --Autonomic changes -- +

Volpe JJ. Neonatal seizures. In: Neurology of the Newborn 1995

TreatmentTreatment Ensure adequate ventilation and perfusion If with hypoglycemia

Glucose 10% 2 ml/kg IV If without hypoglycemia

Anticonvulsant therapy (Phenobarbital) Etiology-Specific (as indicated)

Hypocalcemia (calcium gluconate 5% 4 ml/kg IV) Hypomagnesemia (MgSO4 50% 0.2 ml/kg IV) Pyridoxine deficiency (pyridoxine 50-100 mg IV) Meningitis (Ampicillin/Cefotaxime

Neurological DisordersNeurological Disorders

Microencephaly OFC is < 10% for gestational age It is caused by a neuronal proliferation

defect and occurs between 2 and 4 months gestation

The most severe cases occur in earlier gestation

Outcome is dependent on severity and may be associated with developmental delays

Neurological DisordersNeurological Disorders Anencephaly - absence of

neural tube closure exposing neural tissue Occurs as a malfunction in the

first state of neurological development,dorsal induction.

Infants lack brain above the brainstem and partial absence of skull bones with absent cerebrum.

May or may not lack cerebellum, brainstem and spinal cord.

Infants do not usually survive beyond the neonatal period.

Care includes comfort measures for the infant, and family support.

Encourage the family to see the infant and obtain genetic counseling for the family.

Neurological DisordersNeurological Disorders Hydrocephaly

Congenital hydrocephalus is excess cerebral spinal fluid (CSF) in the ventricles of the brain.

CSF is produced from the parenchyma, cerebral ventricles, areas along the spinal cord, and the choroids plexus

Excessive CSF production, inadequate CSF absorption, or obstruction in the outflow tract can all result in hydrocephalus

Infants have a large head, separated sutures, full and tense fontanelles, increasing OFC, sunset eyes (signifies brain tissue damage) and visible scalp veins

Neurological Disorders - Neurological Disorders - HydroencephalyHydroencephaly

Patient care Neurosurgery and

genetic consults Placement of VP shunt,

subgaleal shunt or reservoir

Careful positioning of the head using cushions to prevent skin break down

Normalize care as much as possible

Review shunt placement and care with parents

Post shunt placement Watch for signs of

infection or blocked shunt, such as irritability, vomiting, increased head circumference, lethargy.

Monitor for a change in feeding pattern.

Assess for full or bulging fontanelles.

Keep infant’s head off of the shunt site

Neurological DisordersNeurological Disorders Post-hemorrhagic hydrocephalus

The progressive dilatation of the ventricles following intraventricular hemorrhage (IVH).

Caused by periventricular white matter injury in the area of the germinal matrix.

Can be acute or chronic. Acute rapidly appears within days of the initial

bleed occurring secondary to malabsorption of CSF due to a clot in the ventricles.

Chronic is from inhibition of CSF flow or from blood from an ICH or pus from infection. The infants have a rapid increase in OFC, apnea, lethargy, increased intracranial pressure, and tense fontanelle, or may be asymptomatic. As progression occurs the signs of an acute bleed may become evident.

Neurological Disorders – Neurological Disorders – Post Hemorrhagic Post Hemorrhagic

HydrocephalusHydrocephalus Patient care:

Daily OFC. Serial head ultrasound. Neurosurgery consult Serial LP if indicated to

decrease ICP or medications to decrease CSF production, such as Lasix or Diamox. Infant may need a ventroperitoneal shunt placed if hydrocephalus does not resolve.

Observe for signs of increased intracranial pressure and hydrocephalus

Neurological DisordersNeurological Disorders Myelomeningocele

A neural tube defect that is a protrusion of the meninges lying directly under the skin, the internal surface of the spinal cord and or the nerve roots

It results from an error in dorsal induction The majority of cases occur in the thoracolumbar,

lumbar and lumbosacral regions. There may be a herniated sac protruding from

the back; it can be sealed or leaking It is usually associated with hydrocephalus or

Arnold-Chiari malformation, which involves some common features like reflux and aspiration, laryngeal stridor, central hypoventilation and apnea

MyelomeningoceleMyelomeningocele Pt CarePt Care

Examine and measure defect, note location and appearance.

Culture lesion of sac if open. Wrap the lesion with sterile gauze moistened with

warm NS. Keep the infant in a prone position; place drape over

the buttocks below the lesion to avoid contamination. Consult a neurosurgeon for surgical closure of defect

and a urologist to rule out neurogenic bladder Follow developed protocol

MyelomeningoceleMyelomeningocele

Most infants will have no significant mental retardation with varying degrees of paralysis of lower extremities. Lesions below S-1: Children will learn to

walk unaided. Lesions between L-4 and L-5: Children

will be able to walk with crutches. Lesions above L-2: Children are usually

wheel chair dependent.

EncephaloceleEncephalocele

A neuronal herniation that may or may not contain meninges or brain parenchyma

Presents as a skin-covered sac protruding from the head or base of the neck

The majority occur in the occipital region

EncephaloceleEncephalocele Patient care

Close physical examination Neurosurgeon consult Diagnostic studies such as CT scan and HUS Treat any seizure activity Educate and support the family

Outcome is dependent on brain involvement. May have motor deficits Impaired intellectual function May be complicated with hydrocephalus

CraniosynostosisCraniosynostosis Patient care

Close physical examination.

Neurosurgeon consult. Diagnostic studies such

as CT scan and HUS Treat any seizure activity. Educate and support the

family. Outcome is dependent

on brain involvement. May have motor deficits. Impaired intellectual

function. May be complicated with

hydrocephalus

Clinical presentation The infant presents

with suture lines that have a bony prominence, unmovable sutures, and abnormal cranial shape

Later signs include, increased ICP, increased irritability, and separation of other sutures

Diagnosis Skull x-rays or CT scan

CraniosynostosisCraniosynostosis

Patient care Thorough physical examination Neurosurgical consult Observe for signs of increased ICP, such

as irritability, lethargy, vomiting, and bulging fontanel

Support and educate the family Early surgical treatment recommended Good outcome with surgical correction

Birth InjuriesBirth Injuries

Any condition that affects the fetus adversely during the entire phase of labor and delivery

Patient care. Observe for bleeding, shock. Monitor blood pressure. Transfuse if needed. Observe for hyperbilirubinemia. Once

the infant has survived the acute phase recovery occurs in 2-3 weeks

CephalohematomaCephalohematoma

CephalohematomaCephalohematoma

A subperiosteal hemorrhage (bleeding between scalp and bone) resulting from a traumatic delivery

It is limited to the surface of the bone and does not cross suture lines

Resolution may take up to several months

CaputCaput

CaputCaput

A diffuse edema of the scalp, resulting from compression of local blood vessels

The edema crosses suture lines and disappears in a few days

Subgaleal HemorrhageSubgaleal Hemorrhage A hemorrhage beneath the scalp that can

enter the subcutaneous tissue of the neck Presents with acute blood loss after birth, is

often a moveable mass and may increase in size post-natally

Can actually bleed entire blood volume into injury

Patient care. Observe for bleeding, shock. Monitor blood pressure. Transfuse if needed. Observe for hyperbilirubinemia. Once the infant has

survived the acute phase recovery occurs in 2-3 weeks

InjuriesInjuries Skull fractures: Can be

linear or depressed secondary to excessive force with forceps and extreme molding. Linear fractures are

asymptomatic. Depressed fractures

present with a depressed surface of skull indented without craniotabies; does not cross suture lines and adjacent sutures are markedly separated

Patient care. Neurosurgical consult

and assess for neurological deficits.

Will usually resolve in a few weeks if lesion is less than 2 cm and patient is without neurological deficits.

Outcome of a linear fracture is good, depressed fracture outcome is dependent of cerebral injury

InjuriesInjuries Brachial nerve plexus

injuries Occurs from excessive

stretching of the brachial plexus during delivery.

Can involve part or entire arm depending on which nerves are affected.

Patient care. There is no treatment

except for passive range motion after swelling has decreased.

It may be necessary to immobilize the limb during the acute phase to decrease the pain.

Generally spontaneous recovery in the majority of cases by 3-4 months of life

InjuriesInjuries Phrenic nerve paralysis.

Caused by torn sheaths, edema, and hemorrhage of cranial nerves III, IV, and V

Results in diaphragmatic paralysis

Infant may have episodes of cyanosis, decreased breath sounds on the affected side, irregular labored breathing, and may have Erb’s palsy on the affected side

Patient care: Give oxygen and

ventilator support as needed.

Position with affected side down.

Educate parents

HemorrhagesHemorrhages Subdural hemorrhage

Caused by tears of cerebral veins or venous sinuses.

Occurs with or without laceration of the dura. Can be caused by CPD, abnormal labor duration,

vaginal breech delivery, malpresentation or assisted delivery.

Clinically are often normal but may have seizure activity on day 2 or 3 of life.

In more severe cases may be lethargic progressing to coma with dilated, poorly reactive pupils on the same side as the hemorrhage.

Infants with large tears and worsening condition have a poor outcome, but a high percentage of infants do well

Subdural HemorrhageSubdural Hemorrhage

HemorrhagesHemorrhages Subarachnoid hemorrhage

Bleeding in the subarachnoid space of venous origin.

May be due to prematurity, trauma, and hypoxia.

Clinically the infants range from normal, to having seizure activity on day 2 of life, and the infant looks healthy between seizures.

May have recurrent apnea and hydrocephalus.

Infants generally have a good outcome

Intracerebellar hemorrhage Occurs in the subependymal

region. Resulting from a primary bleed

or an extension of a hemorrhage into the cerebellum.

Is associated with RDS, perinatal asphyxia, and prematurity.

Contributing factors include: IVH with large volume of blood present, increased intracranial pressure, and incomplete myelination of the cerebellum.

Clinically the infant may present with apnea and bradycardia with decreasing Hct and bloody CSF

Subarachnoid Subarachnoid HemorrhageHemorrhage

Intracerebellar Intracerebellar hemorrhagehemorrhage

InsultsInsults

Hypoxic-Ischemic Encephalopathy (HIE) Occurs in term infants with a history of perinatal

asphyxia who exhibit clinical signs of acute brain injury.

Caused by hypoxemia and ischemia that follow asphyxia.

During asphyxia, cardiac output is redistributed, cerebral blood flow increases, and autoregulation is impaired or lost.

As asphyxia continues, cardiac output decreases causing decreased cerebral blood flow and ischemia, resulting in neurologic impairment

HIEHIE

HIEHIE Mild: Characterized by brief lethargy, jitteriness,

hyper-alert state, irritability, tachycardia, dilatation of pupils, decreased secretions, transient hypoglycemia, and EEG is WNL

Moderate: Lethargy, hypotonia, decreased spontaneous movement, jitteriness, and discrepant muscle strength between shoulders and pelvic region. The infant either improves or deteriorates in the 48-72 hours after birth

Severe: Infants progresses from obtund to stupor to comatose

Mechanical ventilation is required to sustain life. Symptoms include apnea, seizures, severe hypotonia, absent reflexes, reactive pupils, with deterioration 24-72 hours after birth

PVLPVL

Ischemic, necrotic periventricular white matter that can occur after severe IVH, inadequate cerebral perfusion or HIE in premature infants.

Clinical presentation: Weakness in lower extremities.

Outcome can include spastic quadriplegia, visual impairment, and lower limb weakness, but depends on location and severity.

PVLPVL

Cerebral PalsyCerebral Palsy Any one of a number of neurological disorders

that appear in infancy or early childhood and permanently affect body movement and muscle coordination but aren’t progressive

Not caused by problems in the muscles or nerves Caused by abnormalities inside the brain that

disrupt the brain’s ability to control movement and posture

Damage is a result of injury to the brain either before, during, or after birth.  In either case, the damage is not repairable and the disabilities that result are permanent.      

Cerebral PalsyCerebral Palsy Symptoms: 

Lack of muscle coordination when performing voluntary movements (ataxia)

Stiff or tight muscles and exaggerated reflexes (spasticity)

Walking with one foot or leg dragging Walking on the toes, a crouched gait, or a “scissored”

gait Variations in muscle tone, either too stiff or too floppy Excessive drooling or difficulties swallowing or

speaking Shaking (tremor) or random involuntary movements Difficulty with precise motions, such as writing or

buttoning a shirt.

CystsCysts

Fluid- filled ‘holes’ or ‘voids’ where brain tissue should be

Can be located anywhere within brain Etiology is usually anoxia or structural

insult Rises from both in-utero and post-natal

insults Outcome based on location and extent of

cyst Ranges from benign to devastating

CystsCysts

Types:Types: ArachnoidArachnoid

Names based on locationNames based on location Dandy-WalkerDandy-Walker Ependymal Ependymal Rathke’s CleftRathke’s Cleft PorencephalicPorencephalic OthersOthershttp://www.thamburaj.com:80/cysts.htmhttp://www.thamburaj.com:80/cysts.htm

Ependymal CystEpendymal Cyst

Porencephalic CystPorencephalic Cyst