Child Muscular Dysfunction

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Cerebral Palsy (CP) 1: Characterized by early onset and
impaired movement and posture
Incidence 1.5-3 per 1000 live births
Most common permanent physical
disability in childhood
Etiology of CP 2: Intrauterine hypoxia/asphyxia
ntrapartum asphyxia
1 2%-23% of CP occurs in term infants with intrapartum asphyxia
Postnatal
Often no identifiable immediate cause
Etiology of CP (cont) 3: â€¢Preterm birth of ELBW and VLBW is single most important determinant of CP
Anoxiaâ€”most common cause of brain damage whenever it occurs
Types of CP 4: Spastic
Athetoid/dyskinetic
Ataxic
Mixed/dystonic
Types of CP 5: Spastic
Most common clinical type
Presents most often as hypotonia
Types of Spastic CP 6: Quadriparesis (tetraparesis)
Four extremities involved/severe disability
Speech and swallowing difficulties
Tongue protrusion (incomplete)
Labile emotions in some patients
Types of CP (cont'd) 7: Diplegia
Monoplegia
Triplegia
Paraplegia
Possible Motor Signs of CP 8: Poor head control after age 3 mos
Stiff or rigid limbs
Arching back/pushing away
Floppy tone
Unable to sit without support at age 8 mos
Clenched fists after age 3 mos
Possible Behavioral Signs 9: Excessive irritatbility
No smiling by age 3 mos
Feeding Difficuties
Persistent tongue thrusting
Frequent gagging or choking with feeds
CP and IQ 10: Wide variation
70% of CP patients have normal IQ
Difficult to assess
Rigid, atonic, and quadriparetic CP have highest incidence of profound impairment
Neural Tube Defects 11: Failed closure of neural tube
May involve entire length of the neural tube or small portion
Incidence
More girls than boys
Occurs three times more often in whites than African-Americans
Neural Tube Defects (cont'd) 12: Cause
50% or more = folic acid deficiency
Other cases = multifactorial
Neural Tube Defects (cont'd) 2 13: Treatment = prevention
Supplementationâ€”0.4 mg/ day
If history of NTDâ€”4.0 mg/day
In 1998 FDA fortifies cereal grains with folic acid
Begin at preconception; can reduce risk by 50%
Antenatal Diagnosis 14: Elevated alpha-fetoprotein in amniotic fluidâ€”16-18 wks gestation
Uterine ultrasound
Why do we want to know?
Types of Neural Tube Defects 15: Two most common types
Anencephaly
Spina bifida/myelomeningocele
Anencephaly 16: Absence of cerebral hemispheres
Brainstem function may be intact
Incompatible with life
Few hours to few days
Death due to respiratory failure
Spina Bifida 17: Failure of osseous spine to close
Two types
Spina bifida occulta
Not visible externally
Spina bifida cystica
Visible defect ⬢Sa c-like protrusion
Spina Bifida Occulta 18: Usually lumbosacral, L5 S1
Skin indicators (absent, singly or combos)
Sacrat dimple
Sacral angioma or port wine nevus
Sacral tufts of dark hair
Sacral lipoma
Spina Bifida Occulta 19: "Tethered cord"
Abnormal adhesion to bony or fixed structure
Puts traction on the cord
Altered gait
Bowel/bladder problems
Foot deformities
May not be seen in early infancy
Spina Bifida Occulta (cont'd) 20: Diagnostics -X-ray -MRI -CT - Ultrasonography
Spina Bifida Cystica 21: Definition: visible defect with external sac-like protrusion
Two types
Meningocele
Myelomeningocele
Meningocele 22: Sac contains meninges and spinal fluid but no neural elements
No neurologic deficits
Myelomeningocele 23: Neural tube fails to close
May be anywhere along the spinal
column
Lumbar and lumbosacral areas most common
May be diagnosed prenatally or at birth
Myelomeningocele (cont'd) 24: Sac contains meninges, spinal fluid, and nerves
Varying and serious degrees of
neurologic deficit
Clinically "myelomeningocele" term is interchangeably with phrase "spina bifida"
Myelomeningocele: The SAC 25: May be fine membrane
Prone to leakage of CSF, easily ruptured
May be covered with dura, meninges, or skin
Rapid epithelialization
Myelomeningocele (cont'd)2 26: Location and magnitude of defect
determine nature and extent of
impairment
If defect below 2nd lumbar vertebra
Flaccid paralysis of lower extremities
Sensory deficit
Not necessarily uniform on both sides of defect
Initial Management of Myelomeningocele 27: Prevent infection
Assessment of neuro and associated anomalies
Early closure in 12-72 hrs after birth
Prevent stretching of other nerve roots and further damage
Latex Allergy 28: Identified as serious health hazard when a child with SB experienced anaphylaxis due to latex allergy
SB patients at high risk for latex allergy due to repeated exposure to latex products from multiple surgeries and repeated urinary catheterizations
Allergic Reactions to Latex 29: Range from urticaria, wheezing, rash, toanaphylaxis
Reactions tend to increase in severity when latex comes in contact with mucous membranes, wet skin, bloodstream, or airway
Cross-reactions with foods:b banana, avocado, kiwi, chestnuts
Populations at Risk for Latex Allergy 30: Patients with urogenital anomalies
Patients with multiple surgeries
Healthcare workers
Goals 31: To reduce exposure to latex for SB clients who are at risk of developing allergy
To create a latex-safe environment for allergic individuals
Muscular Dystrophies (MDs) 32: Largest group of muscular diseases in children
All have genetic origin with gradual degeneration of muscle fibers, progressive weakness and wasting of skeletal muscles
All have increasing disability and deformity with loss of strength
Duchenne Muscular Dystrophy (DMD) 33: AKA pseudohypertrophic muscular
dystrophy
Most severe and most common of
muscular dystrophies in childhood
X-linked inheritance pattern; one third are fresh mutations
Incidence: 1 in 3500 male births
Characteristics of DMD 34: Onset between age 3-5 yrs
Progressive muscle weakness, wasting and contractures
Calf muscles hypertrophy in most patients
Progressive generalized weakness in adolescence
Death from respiratory or cardiac failure
Diagnostic Evaluation of DMD 35: Suspected based on clinical appearance
Confirmation by EMG, muscle biopsy, and serum enzyme measurement
Serum CPK and AST levels high in first 2 yrs of life, before onset of weakness;
levels diminish as muscle deterioration continues
Clinical Manifestations of DMD 36: Waddling gait, frequent falls, "Gower sign"
Lordosis
Enlarged muscles, especially thighs and upper arms
Profound muscular atrophy in later stages
Mental deficiency common
Therapeutic Management of DMD 37: No effective treatment established
Primary goal: maintain function in unaffected muscles as long as possible
Keep child as active as possible
ROM, bracing, performance of ADLs, surgical release of contractures prn
Genetic counseling for family
Nursing Considerations of DMD 38: Helping child and family cope with chronic, progressive, debilitating disease
Help design a program to foster independence and activity as long as possible
Teach child self-help skills
Appropriate healthcare assistance as child's needs intensify (home health, skilled nursing facility, respite care for family, etc.)
Spinal Cord Injuries 39: Generally the result of indirect trauma
Especially in MVC without child
restraints
Vertebral compression from blows to the head or buttocks (diving, surfing, falls
from horses)
Birth injuries from traction force on spinal cord during breech delivery
Levels of Spinal Cord Injuries 40: Higher injuryâ€”more extensive damage
Paraplegia: complete or partial paralysis of lower extremities
Tetraplegia: lacking functional use of all four extremities (formerly called "quadriplegia")
High cervical cord injury affects phrenic nerve, paralyzes diaphragmâ€”>vent
dependent
Spinal Cord Injury 41: Therapeutic management: stabilization and transport to pediatric trauma center
environment
Management is complex and controversial
Nursing considerations
Stabilization, careful assessment, prevention of
complications, maintain maximum function
Rehabilitation: evaluation and support
Increased Intracranial Pressure (ICP) 42: Early signs and symptoms may be subtle
As pressure increases, signs and symptoms become more pronounced
and level of consciousness (LOC) deteriorates
Clinical Manifestations of Increased ICP in Infants 43: Irritability, poor feeding
High pitched cry, difficult to soothe
Fontanelles: tense, bulging
Cranial sutures: separated
Eyes: setting-sun sign
Scalp veins: distended
Clinical Manifestations of Increased ICP in Children 44: Headache
Vomiting: with or without nausea
Seizures
Diplopia, blurred vision
Behavioral Signs of Increasing ICP 45: Irritability, restlessness
Drowsiness, indifference, decrease in physical activity and motor skills
C/O fatigue, somnolence
Inability to follow commands, memory loss
Weight loss
Late Signs of Increasing ICP 46: Decreased LOC
Decreased motor response to command
Decreased sensory response to painful stimuli
Alterations in pupil size and reactivity
Papilledema
Decerebrate or decorticate posturing
Cheyne-Stokes respirations
Levels of Consciousness (Continued Descending Order) 47: Obtundation: arouses with stimulation
Stupor: responds only to vigorous and repeated stimulation
Coma: no motor or verbal response to noxious stimuli
Persistent vegetative state: permanently lost function of cerebral cortex
Levels of Consciousness (in Descending Order) 48: Full consciousness
Confusion: impaired decision making
Disorientation: to time and place
Lethargy: sluggish speech
Special Diagnostic Procedures 49: Lab tests: glucose, CBC, lytes, blood culture if fever, evaluate for toxic
substances, liver function
Imaging: CT, MRI,
echoencephalography, ultrasound, nuclear brain scan, PET
Lumbar puncture
EEG
X-ray (r/o skull fractures, dislocations; evaluate degenerative changes, suture
Nursing Care for Child with Increased ICP 50: Patient positioning
Avoid activities that may increase ICP
Eliminate or minimize environmental noise
Suctioning issues
Encephalitis p1 51: ⬢Herpes Simplex most
common; severe and life threatening
Causes cerebral edema and ICP
⬢LP- normal to cloudy, WBC si elevated w/ increased lymphs,
Protein normal to si elevated, IgM for type 1 herpes simplex
Encephalitis p2 52: ⬢ Sx: HA, malaise, fever,
dizziness, apathy, stiffness of neck, n&v, tremors, hyperactivity, speech difficulties
Severe: High fever, stupor, seizures, disorientation, spasticity, coma, death
Rx: acyclovir, antibiotics
Mgt: Neuro checks, LOC, ICP monitor
Meningitis 53: Hib, strep most common
Sx: Child/Adolescents:
abrupt with fever, chills, HA, vomiting, sz, Kernig and Brudenski signs, nuchal rigidity
Infants and Neonates:
poor feeding, poor tone with lack of mvt, poor cry, hypothermia or fever, full, tense, bulging fontanel may not be
Dx: LP with elevated WBC, decreased glucose, increased protein, increased pressure
Mgt IVF, Isolation, analgesics, anitbiotics, antipyretics, monitor respirations, dexamethasone for ICP (short term)
Hydrocephalus 54: A syndrome or sign, resulting
from disturbances in fluid
balance of CSF caused by
impaired absorption or
obstruction of flow thru
ventricular system ⬢ Sx: Infancy- head grows
abnormal, ant fontanel tense.
bulging, dilated scalp veins.
bones of skull become thin.
frontal protrusion, setting-sun
sign, irritable, feeds poorly, cries when picked up
Childhood-caused by ICP ie, HA, papilledema, strabismus, ataxia,
irritable, lethargic, apathetic, confused, incoherent
VP Shunt Complications 55: Infection
Malfunction
Signs and Symptoms of Shunt Malfunction 56: Emergency
Increased ICP
Worsening neurologic status/altered LOC
Signs and Symptoms of Shunt Infection 57: Shunt malfunction
Fever and inflammation of tract
Abdominal pain
Seizures 58: Caused by malfunctions of brain's electrical system
Determined by site of origin
Most common neurologic dysfunction in kids
Occur with wide variety of CNS
conditions
Seizures (cont'd) 59: Sign and symptoms
Change in LOC
Involuntary movements
Posturing
Changes in perception, behaviors, or sensations
Types of Seizures 60: Epileptic
Non-epileptic
Most seizures are idiopathic
Etiology of Seizures 61: Idiopathic (no known cause)
Genetic factors
Acquired
Incidence and Occurrence 62: 2.3 million Americans affected
Especially children and elderly
More seizures in those under 2 yrs than any other age group
Epilepsy 63: Definition: S2 "unprovoked" seizures
Idiopathic epilepsy: cause unknown
Seizures are indispensable characteristic of epilepsy
Seizures 64: A single seizure not generally classified as epileptic
Single seizure not generally treated with LTRx
Major Causes of Seizures in Children 65: Birth injuries [anoxia] or congenital defects of CNS
Acute infections in late infancy and early childhood
Usually idiopathic in children >3 yrs
Absence Seizures 66: Formerly called "petit mal" or "lapses"
Brief loss of consciousness
Minimal or no change in muscle tone
Almost always appear in childhood (4-12)
Absence Seizures (cont'd)p2 67: Sudden onset of up to 20+ events per day
No warning/no aura
Duration 5-10 seconds
Motor: lip smacking, twitching of eyes, face, slight hand movements
May drop object, child rarely falls
No incontinence
Absence Seizures (cont'd)p3 68: Often misdiagnosed
Inattention/daydreaming
ADD/ADHD
Absence Seizures (cont'd)p4 69: School issues
Behavioral management
Atonic Seizures 70: Sudden momentary loss of muscle tone
Onset usually age 2-5
May or may not have LOC
Sudden fall to ground, often on face
Less severeâ€”head droops forward
several times
Myoclonic Seizures 71: Sudden brief contractions of muscle group
May be single or repetitive
NoLOC
Often occur with falling asleep
May be nonspecific symptom in many CNS disorders
May be mistaken as exaggerated startle reflex
Infantile Spasms 51: Onset in first 6-8 months of life
Usuallv associated with some dearee of mental retardation
Cause: possibly due to disturbance of central neurotransmitter regulator at
specific phase of brain development
Infantile Spasms (cont'd)p2 52: Specific spike seen on EEG
Two times more frequent in males
Head and neck flex forward; knees drawn up (jackknife position)
May or may not have LOC
No postictal drowsiness
Other variants
Infantile Spasms (cont'd)p3 53: Often associated with cerebral
anomalies, anoxic brain injury, PKU
May also have microcephaly, tonic posture, or other abnormal movements
Poor prognosis (mental and
developmental)
Treatment
Pediatric Diagnosis of Seizure Disorders 54: Ascertain type of seizure
History, observation
Determine the cause of the event
Diagnostics
EEG ,MRI
Labs: glucose, lytes, BUN, Ca" LP
Common Pediatric Seizure Triggers 55: Changes in dark-light patterns (camera flashes, headlights, rotating fan blades, reflections off snow or water)
Sudden loud noises
Extreme temperature changes
Dehydration
Fatigue
Febrile Seizures 56: Transient disorder of childhood
Affect approximately 3% of children
Usually occur between ages 6 mos and 3 yrs
Rare after age 5
Two times more frequent in males
Febrile Seizures p2 57: Cause?
Usually in temps >101.8°F
Seizure occurs when temp is increased, not after
Seizure usually over before arriving in ER
Febrile Seizure Treatment 58: Fever reduction
Evaluate history (episodic and family)
Seizure control if ongoing
Diazepam (rectal)
Febrile Seizures p3 59: 95%-98% of children with febrile seizures will not have epilepsy or neurologic damage
Management
Avoid tepid bathsâ€”usually ineffective
Vigorous use of antipyretics
Protect child from injury during seizure
Call 911 if seizure >5 minutes duration
Febrile Seizure Treatment 60: Prophylaxis with medications
If focal or prolonged seizures
If neurologic anomalies
If first degree relative has seizure history
Child <1 yrold
If multiple seizures in 24 hr period
Phenobarbital 61: Febrile seizures, neonatal seizures
Also for other seizures: front line IV choice if patient does not respond to diazepam
High dosage may require respiratory support
Phenytoin (Dilantin) 62: PO or slow IV push (<50 mg/minute)
Precipitates when mixed with glucose
Side effects: gingival hvperplasia, ataxia, rashes, acne, hirsutism, osteoporosis
Onset 5-30 minutes; duration 12-24 hrs
Fosphenytoin 63: May be given with saline or glucose
Rate up to 150 mg/min
IVorIM
Valproic Acid 64: AKA depakote, depakene
IVorPO
IV for status epilepticus
S.E.: hepatotoxicity
Diazepam 65: AKA Valium IV, Diastat (rectal gel)
Rx of choice for status epilepticus
Rectal gel for home or prehospital management
Onset 3-10 min short duration (minutes)
Concurrent loading with phenytoin for sustained control of seizures
Afivan 66: Alternative to diazepam Mav be preferable to diazepam Longer duration of action LOSS respiratory distress in kids >2 yrs
Midazolam (Versed) 67: Intranasat route
For acute epileptic seizures
Onset 3-5 minutes; duration
Pharmacologic Management 68: Monitor therapeutic levels
he. i,3se dosage as child grows
Mocii-or for known side effects
Avoid abrupt discontinuationâ€”gradual dose reduction
When to Discontinue Pharmacologic Management 69: When seizure free for 2 years
Normal EEG
Avoid during puberty or when subject to frequent infections
Recurrence possible within first year
Nursing Interventions 70: Observe and document episode - Protect from injury
Stay cairn
Remain with child
Privacy if possible
Home Management of Seizures 71: CPR training for family members
Rectal diazepam available for intractable seizures
Activity restriction on individual basis
Safety devicesâ€”helmets, no swimming alone, awareness of school, other caregivers

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