Chapter_032

The Child with Neuromuscular or Muscular Dysfunction : 

The Child with Neuromuscular or Muscular Dysfunction Dr S.Dakak

Cerebral Palsy (CP) : 

Cerebral Palsy (CP) 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 : 

Etiology of CP Intrauterine hypoxia/asphyxia Intrapartum asphyxia 12%-23% of CP occurs in term infants with intrapartum asphyxia Postnatal Often no identifiable immediate cause

Etiology of CP : 

Etiology of CP Preterm birth of ELBW and VLBW is single most important determinant of CP Anoxia—most common cause of brain damage whenever it occurs

Types of CP : 

Types of CP Spastic Athetoid/dyskinetic Ataxic Mixed/dystonic

Types of CP : 

Types of CP Spastic Most common clinical type Presents most often as hypotonia

Types of Spastic CP : 

Types of Spastic CP Quadriparesis (tetraparesis) Four extremities involved/severe disability Speech and swallowing difficulties Tongue protrusion (incomplete) Labile emotions in some patients

Types of CP (cont’d) : 

Types of CP (cont’d) Diplegia Monoplegia Triplegia Paraplegia

Possible Motor Signs of CP : 

Possible Motor Signs of CP 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 of CP : 

Possible Behavioral Signs of CP Excessive irritability No smiling by age 3 mos Feeding difficulties Persistent tongue thrusting Frequent gagging or choking with feeds

CP and IQ : 

CP and IQ 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 : 

Neural Tube Defects 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) : 

Neural Tube Defects (cont’d) Cause 50% or more = folic acid deficiency Other cases = multifactorial

Neural Tube Defects (cont’d) : 

Neural Tube Defects (cont’d) Treatment = prevention Supplementation—0.4 mg/ day If history of NTD—4.0 mg/day In 1998 FDA fortifies cereal grains with folic acid Begin at preconception

Antenatal Diagnosis : 

Antenatal Diagnosis Elevated alpha-fetoprotein in amniotic fluid—16-18 wks gestation Uterine ultrasound Why do we want to know?

Types of Neural Tube Defects : 

Types of Neural Tube Defects Two most common types Anencephaly Spina bifida/myelomeningocele

Anencephaly : 

Anencephaly Absence of cerebral hemispheres Brainstem function may be intact Incompatible with life Few hours to few days Death due to respiratory failure

Spina Bifida : 

Spina Bifida Failure of osseous spine to close Two types Spina bifida occulta Not visible externally Spina bifida cystica Visible defect Sac-like protrusion

Defects : 

Defects (Note to instructor: Image available in Electronic Image Collection)

Spina Bifida Occulta : 

Spina Bifida Occulta Usually lumbosacral, L5 S1 Skin indicators (absent, singly or combos) Sacral dimple Sacral angioma or port wine nevus Sacral tufts of dark hair Sacral lipoma

Spina Bifida Occulta : 

Spina Bifida Occulta “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) : 

Spina Bifida Occulta (cont’d) Diagnostics X-ray MRI CT Ultrasonography

Spina Bifida Cystica : 

Spina Bifida Cystica Definition: visible defect with external sac-like protrusion Two types Meningocele Myelomeningocele

Meningocele : 

Meningocele Sac contains meninges and spinal fluid but no neural elements No neurologic deficits

Myelomeningocele : 

Myelomeningocele 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) : 

Myelomeningocele (cont’d) 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 : 

Myelomeningocele: The SAC May be fine membrane Prone to leakage of CSF, easily ruptured May be covered with dura, meninges, or skin Rapid epithelialization

Myelomeningocele (cont’d) : 

Myelomeningocele (cont’d) 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 : 

Initial Management of Myelomeningocele 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 : 

Latex Allergy 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 : 

Allergic Reactions to Latex Range from urticaria, wheezing, rash, to anaphylaxis Reactions tend to increase in severity when latex comes in contact with mucous membranes, wet skin, bloodstream, or airway Cross-reactions with foods: banana, avocado, kiwi, chestnuts

Populations at Risk for Latex Allergy : 

Populations at Risk for Latex Allergy Patients with urogenital anomalies Patients with multiple surgeries Healthcare workers

Goals : 

Goals To reduce exposure to latex for SB clients who are at risk of developing allergy To create a latex-safe environment for allergic individuals

Progressive Infantile Spinal Muscular Atrophy : 

Progressive Infantile Spinal Muscular Atrophy AKA Werding-Hoffmann disease Autosomal recessive trait Most common paralytic form of “floppy infant syndrome,” AKA congenital hypotonia

Progressive Infantile Spinal Muscular Atrophy: Characteristics : 

Progressive Infantile Spinal Muscular Atrophy: Characteristics Progressive weakness and wasting of skeletal muscles Degeneration occurs in spinal cord and brainstem, resulting in atrophy of skeletal muscles Age of onset variable; earlier onset has poorest prognosis

Therapeutic Management : 

Therapeutic Management Diagnosed by electromyography and muscle biopsy Treatment is symptomatic Prevent infection/treat infection vigorously Treat orthopedic problems (especially scoliosis) Prognosis varies with age of onset

Werding-Hoffmann Disease (SMA Group 1) : 

Werding-Hoffmann Disease (SMA Group 1) Manifest within first 2 months of life Infant lies in frog position, weak cry, and cough, generalized weakness Active movement usually limited to fingers and toes Normal sensation and intellect Death usually by age 4 yrs

Intermediate SMA (Group 2) : 

Intermediate SMA (Group 2) Manifest between 2-12 months of age First have weakness of arms and legs, later generalized weakness Prominent pectus excavatum Movements absent during relaxation/ sleep Life span 7 months to 7 years

Kugelberg-Welander Syndrome (Group 3) : 

Kugelberg-Welander Syndrome (Group 3) Onset in first year of life Normal head control, can sit by age 6-8 mos Thigh and hip muscles weak Some will learn to walk Slowly progressive course Normal life expectancy

Muscular Dystrophies (MDs) : 

Muscular Dystrophies (MDs) 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) : 

Duchenne Muscular Dystrophy (DMD) 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 : 

Characteristics of DMD 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 : 

Diagnostic Evaluation of DMD 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 : 

Clinical Manifestations of DMD 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 : 

Therapeutic Management of DMD 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 : 

Nursing Considerations of DMD 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.)

Guillain-Barré Syndrome (GBS) : 

Guillain-Barré Syndrome (GBS) AKA infectious polyneuritis Acute demyelinating polyneuropathy with progressive paralysis Children less often affected than adults Occurrence in children most often between age 4-10 yrs

Pathophysiology of GBS : 

Pathophysiology of GBS Immune-mediated disease Often associated with viral or bacterial infection or administration of vaccines Inflammation and edema in spinal and cranial nerves progresses to impaired nerve conduction, then partial or complete paralysis of involved muscles

Diagnostic Evaluation of GBS : 

Diagnostic Evaluation of GBS Based on paralytic manifestation and/or EMG findings CSF may have increased protein concentration, other labs WNL Symmetric paralysis is part of differential diagnosis

Clinical Manifestations of GBS : 

Clinical Manifestations of GBS Initially: muscle tenderness, paresthesia, muscle weakness Paralysis rapidly ascends from lower extremities; may involve trunk, arms, and face Flaccid paralysis, loss of reflexes Intercostal and phrenic nerve involvement Frequently have urinary incontinency or retention and constipation

Therapeutic Management of GBS : 

Therapeutic Management of GBS Treatment is symptomatic Possibly steroids, IV immunoglobulin, and plasmapheresis Respiratory support

GBS (cont’d) : 

GBS (cont’d) Prognosis: better outcomes associated with younger ages; most patients have complete recovery Most patients have muscle function begin to return 2 days to 2 weeks after onset of symptoms, but prolonged period to complete recovery Most deaths due to respiratory failure

Nursing Considerations of GBS : 

Nursing Considerations of GBS Supportive care Observe for early signs of respiratory distress/difficulty swallowing Focus on prevention of complications Support for child and family

Tetanus : 

Tetanus AKA “lockjaw” An acute, preventable, and often fatal disease Caused by exotoxin of Clostridium tetani Characterized by muscle rigidity involving the masseter and neck muscles

Four Requirements for Developing “Lockjaw” : 

Four Requirements for Developing “Lockjaw” Presence of tetanus spores or vegetative forms of the bacillus Injury to tissues Wound conditions that encourage multiplication of the organism Susceptible host

Tetanus (cont’d) : 

Tetanus (cont’d) Spores are found in soil, dust, and GI tract of humans and animals Bacteria enter body through wound, especially puncture or crush wound or burn May enter through scratch, bee sting, thorn or needle prick Exposure greater during outdoor activities

Pathophysiology of Tetanus : 

Pathophysiology of Tetanus Exotoxin spreads from wound to CNS by way of neurons or bloodstream Toxin becomes fixed on nerve cells of brainstem and spinal cord Toxin produces muscle stiffness

Clinical Manifestations of Tetanus : 

Clinical Manifestations of Tetanus Initially: progressive stiffness and tenderness of neck and jaw muscles; difficulty in opening of the mouth; facial muscle spasm Progressive: opisthotonos, difficulty swallowing, laryngospasm, and tetany of respiratory muscles Rigidity of abdominal and limb muscles

Clinical Manifestations of Tetanus (cont’d) : 

Clinical Manifestations of Tetanus (cont’d) Respiratory: accumulated secretions, atelectasis, pneumonia, respiratory arrest Pt anxious but alert; mentation unaffected Rapid HR, diaphoresis, mild or absent fever Incubation: 3-10 days Mortality approximately 30%, usually fatal in newborn

Therapeutic Management of Tetanus : 

Therapeutic Management of Tetanus Prevention by tetanus toxoid or tetanus antitoxin after exposure Treatment of wounds contaminated with dirt, feces, soil, saliva, puncture wounds, avulsions, crushing, burns, and frostbite should include tetanus immune globulin if patient inadequately immunized

Therapeutic Management of Tetanus (cont’d) : 

Therapeutic Management of Tetanus (cont’d) ICU for constant observation and respiratory support availability Monitor fluid and electrolyte status Tetanus immune globulin therapy to neutralize toxins Wound care to decrease proliferation of organisms Muscle relaxants, sedatives, Pavulon

Nursing Considerations : 

Nursing Considerations Control environmental stimuli Careful monitoring of respiratory status Attempt to reduce anxiety of child and family

Botulism : 

Botulism Food poisoning resulting from ingestion of toxin produced by anaerobic bacillus Clostridium botulinum Sources: improperly sterilized home canned foods for older children; infant sources: honey and light or dark corn syrup

Clinical Manifestations of Botulism : 

Clinical Manifestations of Botulism CNS symptoms appear abruptly 12-36 hrs after ingestion General signs Weakness, dizziness, headache, diplopia, speech difficulties Vomiting Progressive, life-threatening respiratory paralysis

Treatment of Botulism : 

Treatment of Botulism IV botulism antitoxin Supportive measures—respiratory support Continue therapy until paralysis abates

Infant Botulism : 

Infant Botulism Caused by ingestion of spores or vegetative cells of C. botulinum and subsequent release of toxin Source of botulism in infants: honey and light or dark corn syrup Wide variation in severity of disease: mild constipation to respiratory failure

Infant Botulism (cont’d) : 

Infant Botulism (cont’d) Often constipation is present symptom Generalized weakness, decreased movement, diminished deep tendon reflexes Loss of head control, feeding difficulty, weak cry, diminished gag reflex, hypotonia Do not treat infants with botulism antitoxin Prognosis generally good but very slow (weeks to months)

Spinal Cord Injuries : 

Spinal Cord Injuries 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 : 

Levels of Spinal Cord Injuries Higher injury—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 diaphragm→vent dependent

Spinal Cord Injury : 

Spinal Cord Injury 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