Download Neural Tube Disorders

Neurological
Common Neurological Disorders
in Children
Hydrocephalus
Neural Tubes Defects
Bacterial Meningitis
Guillain-Barre Syndrome
Reye’s Syndrome
Seizures
Structural Defects
Hydrocephalus
“Water on the Brain”
Imbalance between the production and
absorption of CSF.
Often from congenital CNS
malformations
Results in rapid head enlargement in
infants
Can lead to irreversible neurological
damage
INFANT
Hydrocephalus
Early Signs
Projectile vomiting not associated with
feeding
Scalp veins become prominent
**Shrill, high pitched cry
**Increasing irritability
Infant Hydrocephalus
Later signs & symptoms
•Bulging anterior fontanel and a head circumference
that increases at an abnormal rate
•Enlargement of the forehead
•Depressed eyes rotated downward: “sunset eyes”
(pupils sink downward)
Hydrocephalus
Signs & symptoms
in older Child
No enlargement of head (skull is closed)
Begins with generalized neuro
symptoms
HA in morning
Nausea
Vomiting
Followed by signs of increased ICP
Relief of hydrocephalus
Create a new
pathway to divert
excess CSF.
A catheter or shunt
is placed in the
ventricle and passes
the CSF to the
peritoneal cavity
Needs to be
replaced PRN
Post-op Nursing Care Shunt
Placement
Keep child flat unless ICP is present the
bed slightly elevated
Slowly increase HOB over few days
Support head when moving child
Pain management
Vital Signs
Post-op Nursing Care
Shunt Placement
Observe for signs of increasing ICP
– neurologic assessment
Observe for abdominal distention
Strict I & O
Antibiotics
Meticulous skin care
Support family
Discharge Management Post
Shunt Placement
Teach parents to monitor for shunt
complications:
Headache, progressive or worsening
Drowsiness or inappropriate sleepiness during
the day, irritability
Nausea, vomiting
Personality changes or changes in school
performance
Fever
Redness or swelling along the shunt tract
Neural Tube Disorders
Defects of closure of neural tube during
fetal development
Congenital (present at birth)
Believed to be caused by genetic or
environmental factors, but exact
etiology is unknown
Common in women with poor folic acid
intake before and during pregnancy
Nursing Implications
Advise all women to adhere to
routine screening/diagnostic
testing
Advise all women capable of
becoming pregnant to consume
0.4 mg of folic acid daily
Neural Tube Disorders
Types:
Spina Bifida
Occulta
Cystica
Meningocele
Myelomeningocele
Spina Bifida
Most common CNS defect
Caused by failure of neural tube to
close at some point along spinal
column
Types:
 spina bifida occulta
 spina bifida cystica
Spina Bifida Occulta
 Not
visible
externally
 Lamina fail to
close but spinal
cord does NOT
herniate or
protrude through
the defect
 No motor or
sensory defects
Spina Bifida Cystica
 Meningocele
External sac that
contains
meninges and
CSF
 Protrudes
through defect in
vertebral column

Meningocele

Not associated
with neurologic
deficit – good
prognosis

Hydrocephalus
may be an
associated
finding, or
aggravated after
repair
Spina Bifida Cystica

Myelomeningocele

Same as above, but
the spinal cord and
meninges protrude
through the defect in
the bony rings of the
spinal cord
Contains nerves
therefore the infant
will have motor and
sensory deficits
below the lesion
Myelomeningocele
 Visible
at birth,
most often in the
lumbaosacral area
 Covered
with a
very fragile thin
membrane/sac
which can tear
easily, allowing
CSF to leak out
Nursing Interventions
•
•
Protect the sac from injury
Keep free from infection
•
•
•
Position: prone or side lying
Cover sac with sterile, moist non-adherent
dressing, sterile technique imperative
Parents need emotional support &
education regarding short and long term
needs of infant
Nursing Interventions
•
Surgical repair usually within first
24 hours
observe for early signs of infection:
elevated temp, irritability, lethargy,
nuchal rigidity
– observe for signs of increasing ICP
(may indicate hydrocephalus)
–
Habilitation
Emphasizes constructive use of
‘normal’ parts of body & minimizes
the disabilities making the child as
self-helpful as is possible in the
activities of daily living
Major problems: incontinence,
constipation, obesity or
malnutrition
Neurologic Infections
Meningitis
• Acute inflammation of the cerebral
meninges as a result of a bacterial or viral
infection
Bacterial Meningitis
Follows 2-3 days of upper respiratory
infection
• Haemophilus influenzae type b was the most
common cause of bacterial meningitis in
children prior to the use of the Hib conjugate
vaccine
• May be caused by Strep pneumoniae in child <
24months (not fully vaccinated yet)
• Meningococcal predominantly in school-age
children & adolescents (vaccine preventable)
Bacterial Meningitis
Signs & Symptoms
Abrupt onset of fever
Chills
Increasing irritability
Headache
Convulsions
Blurred vision
Cranial nerve paralysis
Opisthotonic position
Classic Signs and Symptoms
1.
Nuchal rigidity
2.
(+) Kernig’s
sign
3.
(+)Brudzinski’
s sign
Signs & Symptoms
in Newborn
Above slide’s signs plus:
• poor suck
• weak cry
• lethargy
• can lead to sudden shock,
seizures, apnea
• bulging fontanel
Bacterial Meningitis
Diagnosis:
 lumbar puncture to analyze CSF

increased WBC’s and protein; decreased glucose
(bacteria feed on glucose)
Treatment:
 ABX x 10 days, IV or Intrathecal
 Respiratory isolation x 24 hours while on ABX
 Maintenance of optimum hydration
 Maintenance of ventilation
 Reduction of increased ICP
 Management of bacterial shock
 Control of seizures
 Prophylactic ABX for family members
Complications
Life Threatening Condition
If Survival:
Hearing loss
Blindness
Paresis
Intellectual impairment
Nursing Management
Frequently assess vital signs, LOC,
neurologic assessment to identify
changes in the child’s condition.
Measure head circumference
frequently- risk for hydrocephalus.
Monitor intake and output
Nursing Management
Promote comfort
• reduced stimulation (dim lights, quiet
room)
Side-lying position
Identify parents’ concerns, provide support
Prevention is a major role for nurses.
Encourage parents to get their infants and
children fully immunized!
Guillain-Barre Syndrome
•
Immune-mediated disease of motor weakness
that is often associated with viral or bacterial
infection of respiratory or GI tract or vaccine
administration
•
Adults have increased susceptibility, can affect
children usually ages 4-10
•
Inflammation of nerve fibers, impairs nerve
conduction though demyleination
•
Ascending paralysis from lower extremities
Initial Symptoms
Peripheral neuritis occurs several days
after primary infection
Muscle tenderness
Tendon reflexes decreased or absent
Paresthesia & cramps
Proximal symmetric muscle weakness
Urinary incontinence or retention
Decreased swallowing & respiratory
efforts-may lead to respiratory failure
Treatment
Wait for disease to stabilize
Intravenous immune globulin IVIG
Physical Therapy
Rarely fatal, often residual paralysis
Nursing Care
Monitoring respiratory status
Managing autonomic nervous system
dysfunction
Preventing complications associated
with immobility
Providing emotional support
tTeaching the parents how to
care for the child after discharge.
Reye’s Syndrome
A life threatening acute
encephalitis
Occurs after viral infection if tx’d
w/ aspirin
High Mortality Rate
Education efforts has helped to
reduce incidence (use Tylenol or
Ibuprofen not ASA)
Reye’s Syndrome
Begins with mild viral infection that worsens w/i
24-48 hours
Lethargy
Vomiting
Followed by
Agitation
Anorexia
Combativeness
Confusion leading to stupor, coma, seizures,
respiratory arrest
Reye’s Syndrome
Labs
Increased:
Liver enzymes
Serum ammonia
PT, PTT
WBC
Decreased:
Serum glucose
Reye’s Syndrome
Nursing Care
If Survival in PICU
Monitor:
Neurological status
Respiratory effort
Hypoglycemia
Cerebral edema
Seizures
Seizures
Involuntary contraction of muscle
caused by abnormal electrical
brain impulses
They are episodic and abrupt
Often triggered by environmental
of physiological stimuli
Exact location of the electrical foci
and the number of brain cells
involved determines the nature of
the seizure (sterotypical)
Types of Seizures
Nonrecurrent –
Acute
Febrile episodes
Drugs
Metabolic
alterations
Recurrent –
Chronic (Epilepsy)
Idiopathic
(primary) epilepsy
Epilepsy
secondary to
trauma,
hemorrhage,
infections,
congenital defects
Seizures: 2 categories
Partial
Simple
Complex
Only 1 area of
brain involved
Symptoms are
associated with
the area affected
No LOC or
consciousness is
impaired
Generalized
Infantile spasms
Febrile
Absence
Tonic Clonic
Entire brain
Usually have loss of
consciousness
May have aura
Postical
Partial Seizures
Simple Partial Seizures
Complex Partial Seizures
Simple Partial Seizures
• Seizure is short, lasts < 30 seconds
• No loss of consciousness, aura, postical state
Seizure Activity:
Abnormal motor activity
•
One extremity or part of extremity, uncontrolled
movement
Abnormal sensory activity
Numbness, tingling, paresthesia or pain starting in 1
area of body, may spread to other parts of body
May include abnormal auditory, olfactory and visual
sensations
Complex Partial Seizures
Seizure is longer, 30 seconds-5 minutes
Consciousness is impaired immediately
May have slight aura
Seizure Activity:
Sudden change in posture
Abnormal motor activity, twitching, loss of tone,
tingling or numbness
Automatisms-lip smacking, chewing, sucking
Circumoral pallor
Afterward: drowsiness
Generalized Seizures
Infantile spasms
Absence
Tonic Clonic
Febrile
Infantile Spasms
Age: 4 months to 2 years
• Occur in clusters 5-150/day, worse
at night
• Altered consciousness
Abrupt flexion/extension of neck,
trunk, extremities
Eye rolling
May have permanent cognitive &
developmental delays
Absence Seizure
• Lasts 5-10 seconds, multiple times a day 50-
100 per day
• Seizure is a brief loss of consciousness
• Appears to look like a staring spell
Rhythmic blinking & twitching of mouth or arm
Mistaken for daydreaming or behavior
problems
Interferes with learning
1/3 of children will grow out of them by
adolescence
Tonic Clonic
(Grand Mal Seizure)
4 stages of Seizure
1. Prodromal:
Drowsiness, dizziness, malaise, lack of
coordination, “not himself”
2. Aura:
May precede seizure, reflects portion of
brain where seizure originates
Tonic-clonic stage
3. Tonic-Clonic:
Tonic: 20 seconds, all muscles cx (rigid),
child falls to ground, LOC, respiratory
muscles affected, grunting, airway
compromised
Clonic: 20-30 seconds, jerky muscle
contract & relax rapidly, froth or bloody
sputum, urinary or bowel incontience
4. Postictal:
Appears to relax, semi-conscious, sound
sleep for 1-4h, no recollection of event
Acute Febrile Seizure
Due to increased temperature
> 102 F (but may occur as low as
100 F)
Higher fever=higher risk
Occur between 6 months and 5
years, with a peak incidence
between 18 and 24 months of age
Tonic-clonic pattern
Lasts 15-20 seconds
Epilepsy
• Chronic disorder with recurrent
seizures in children 3 and older
• Symptoms depend on type of
seizure
• No association with illness, injury
• Seizure may be triggered by
something
Epilepsy Management
 Anticonvulsants-monotherapy




is desired
Dosage increased as child
grows
Control the seizures or reduce
their frequency
Discover and correct the cause
when possible, know triggers
Help child live a normal life
Epilepsy Management
 Instruct parents on importance of
giving meds to achieve therapeutic
drug levels
 Med can be withdrawn when child
is seizure free for 2 yrs with
normal EEG
 TAPER! Gradually decreased over
1-2 weeks
Triggers
Changes in dark-light patterns
Sudden loud noises, specific voices
Sudden or startling movements
Extreme changes in temperature
Dehydration, fatigue
Hyperventilation
Hypoglycemia
Caffeine, insufficient protein in diet
Status Epilepticus
(Intractable Seizures)
Continuous seizure activity lasting > 30
minutes or a series of seizures from
which the child does not regain a
premorbid level of consciousness
If A Patient is Admitted for
Seizure Activity or has PMH of
Seizures
Ensure IV access and Patency
Check MD orders for Seizure Medication
Check that medication is on the Unit
Check Suction at bedside, ambu bag, mask
and 02 tubing, SaO2 is available and working!
Are Side Rails Padded?
ID band- correct? Indicate Seizure Risk?
Know how to initiate emergency or rapid
response
Nursing Management during
Seizures
1. Maintain Patent Airway
Place nothing in the child’s mouth
during a seizure
Loose teeth may be knocked out and
aspirated.
Position side so secretions can drain
Pulse oximetry reading (SpO2)
Oxygen for < 95%- use mask
Suction prn
Nursing Management during
Seizures
Ensure Safety
• If OOB gently assist to floor
• Bed in lowest position
• Stay near child
• Protect head from injury
Nursing Management during
Seizures
Administer Mediation
Initial order is intravenous medications
IV push slowly
Benzodiazopene IV push slowly to avoid
apnea
Diazepam (Valium) or lorazepam
(Ativan)
Followed by anticonvulsant
Nursing Management during
Seizures
Observe and Record
Type of seizure activity
Vital Signs
Time seizure started and stopped
Dilantin
Toxicity: nystagmus, ataxia, decresed
mental capacity
Low levels: seizure activity
S/E: gingival hyperplagia (discuss oral
hygiene), drowsiness,
thrombpcytopenia, leukopenia,
increased liver enzymes
Nursing responsibility:
Monitor CBC, LFT, therapeutic drug levels
Practice Questions!
A 10-year old is diagnosis is Guillain-Barre
Syndrome. It would be imperative for the
nurse to inform the physician after
observing which of the following?
Weak muscle tone in the feet
2. Weak muscle tone in the legs
3. Increasing hoarseness and cough
4. Tingling in the hands
1.
A 4-year-old is being evaluated for
hydrocephalus. The nurse notes which
of the following as an early sign of
hydrocephalus in a child?
1. Bulging fontanels
2. Rapid enlargement of the head
3. Shrill, high-pitched cry
4. Early morning headache
A child with a history of a seizure was admitted
2 hours ago. The history indicates fever,
chills, and vomiting for the past 3-4 hours.
In report the nurse is told that the child had
a positive Brudzinski’s sign. The nurse infers
this is most likely caused by:
Increased intracranial pressure
2. Meningeal irritation
3. Encephalitis
4. Intraventricular hemorrhage
1.
A nurse is assessing a new admission.
The 6-month-old infant displays
irritability, bulging fontanels, and
setting-sun eyes. The nurse would
suspect:
1.
2.
3.
4.
Hydrocephelus
Hypertension
Skull fracture
Myelomeningocele
An 8-year-old client with a ventriculoperitoneal
shunt was admitted for shunt malfunction.
He presents with symptoms of increased
intracranial pressure. The mechanism of the
development of his symptoms is most
probably related to:
Increased flow of CSF
2. Increased reabsorption of CSF
3. Obstructed flow of CSF
4. Decreased production of CSF
1.
The nurse is taking a history of a child
admitted for EEG testing to determine
seizure activity. The parent reports that
the child has “odd” behavior, including
periods of lip smacking, and muscle
twitching . The nurse suspects:
1. Simple Partial Seizures
2. Complex Partial Seizures
3. Absence Seizures
4. Tonic-Clonic Seizures