Chapter 24: child w/ neuromuscular dysfxn

Question 1

Upper vs lower motor neuron lesions

Answer 1

-Upper: weakness, spasticity, increased DTRs, abnormal superficial reflexes, primarily causes CP (cerebral palsy)
-Lower: weakness, atrophy of skeletal muscles, hypotonia, usually symmetric, gradual or sudden onset indicates causation

Question 2

Dx tools

Answer 2

-Neuro exam
-Electromyogram (EMG): measures electrical activity, topical anesthetic, nerve conduction studies
-Muscle biopsy: procedural sedation, serum enzymes

Question 3

CP

Answer 3

-Nonprogressive impairment of motor fxn
-Abnormal perception and sensation, visual hearing and speech impairments, seizures, cognitive disabilities
-Attributed to disturbances that occurred in developing fetal or infant brain
-Most common permanent physical disability in childhood

Question 4

Etiology of CP

Answer 4

-Prenatal, perinatal, postnatal risk factors
-Brain abnormalities, cerebral infections, head trauma (shaken baby syndrome), anoxic brain injury
-Premature or multiple births, VLBW infants
-Placental insufficiency
-Intrapartum asphyxia (traumatic birth or shoulder dystocia)
-Anoxia is most common cause of brain damage whenever it occurs

Question 5

CP clinical classification systems

Answer 5

-ICF, GMFCS, MACS, CFCS, HINE

Question 6

CP types

Answer 6

-Spastic (most common)
-Dyskinetic
-Ataxic
-Mixed

Question 7

CP s/s

Answer 7

-Motor: poor head control after 3, stiff or rigid limbs, arching, floppy tone, unable to sit w/o support at 8 m, clenched fists after 3 m
-Behavioral: excessive irritability, no smiling by 3 m, feeding difficulties

Question 8

CP dx

Answer 8

-Early infancy
-Parents describe concern w/ development
-Neuroimaging: MRI is strong predictor when performed at term
-Metabolic and genetic testing

Question 9

CP tx and mgmt

Answer 9

-Braces, splints, adaptive equipment
-PT, OT, speech therapy
-Botulinum toxin type A (botox) reduces muscle stiffness
-Baclofen pump (intrathecal) decreases rigidity in tone
-Dantrolene sodium (dantrium) is a muscle relaxant
-Diazepam (valium) can treat muscle spasms
-Surgery for progressive deformities
-Dental care

Question 10

CP complications and mgmt

Answer 10

-Aspiration: keep head elevated, keep suction available, correct feeding techniques, CPR education
-Injury: raise bedrails, pad side rails, secure child in wheelchairs, frequent rest periods, use of helmets

Question 11

CP intellect

Answer 11

-Little more than half have normal IQ
-Difficult to assess
-Rigid, atonic, quadriparetic CP have highest incidence of profound impairment

Question 12

Neural tube defects

Answer 12

-Largest group of congenital anomalies w/ multifactorial inheritance
-Can involve entirety or small area of neural tube
-More common in women
-Failed closure of neural tube
-Prenatal prevention: folic acid 0.4 mg/day
-Alpha fetoprotein test at 6-18 weeks gestation can detect
-Ultrasound imaging

Question 13

Anencephaly

Answer 13

-Most serious NTD –> both cerebral hemispheres are missing
-Many are stillborn
-Provide comfort but no resuscitation efforts

Question 14

Spina bifida

Answer 14

-Failure of osseous spine to close
-Types: SB occulta (not visible externally), SB cystica (visible w/ external sac, includes meningocele and myelomeningocele)

Question 15

SB occulta

Answer 15

-L5 to S1
-Skin s/s: sacral dimple, sacral angioma or port wine nevus, sacral tufts of dark hair, sacral lipoma
-Dx: prenatal –> amniocentesis, chorionic villus sampling, postpartum –> MRI, US, CT, X-ray

Question 16

SB cystica

Answer 16

-Visible w/ external sac
-Meningocele: sac contains meninges and spinal fluid but no neural elements
-Myelomeningocele: sac contains meninges, spinal fluid, nerves

Question 17

Myelomeningocele

Answer 17

-If defect is below L2: flaccid paralysis of lower extremities, incontinence, sensory deficit
-Protection of site from trauma (prone position)
-Keep sac from dying out w/ saline-soaked sterile gauze
-Assess for hydrocephalus
-Risk of latex allergy d/t to exposure from catheterizations and surgeries
-s/s: urine dribbling, overflow incontinence, poor anal sphincter tone

Question 18

Hypotonia

Answer 18

-Decreased muscle tone in neonatal period
-Muscle atrophy, marked head leg, poor sucking, frog-leg posture
-AKA floppy infant syndrome
-From cerebral trauma or perinatal hypoxia
-Dx: nerve conduction velocity, EMG, muscle biopsy

Question 19

SMA

Answer 19

-Spinal muscular atrophy
-Type 1 –> werdnig-hoffman disease
-Autosomal recessive
-Progressive weakness and wasting of skeletal muscles
-Seen before age 2 y, progresses to death from RF
-Tx: no cure

Question 20

GBS

Answer 20

-Guillain-barre syndrome
-Infectious polyneuritis
-Acute demyelinating polyneuropathy w/ progressive paralysis
-Immune-mediated disease
-Occurs 10 days after nonspecific viral infection
-Associated w/ vax
-Children less affected then adults
-Better outcomes w/ younger children
-Phases: acute (s/s), plateau (little change), recovery (most recovery done by 4 weeks)
-Good prognosis, muscle fxn returns 2 days - 2 weeks after onset of s/s

Question 21

GBS s/s

Answer 21

-Muscle tenderness, paresthesia, muscle weakness
-Paralysis ascends from legs to head
-Flaccid paralysis, loss of reflexes
-Urinary complications

Question 22

GBS dx

Answer 22

-EMG, assess health of muscles and motor neurons
-CSF may have increased protein concentrations
-WNL labs
-Symmetric paralysis

Question 23

GBS mgmt

Answer 23

-Steroids, IV immunoglobulins, plasmapheresis
-Heparin, gabapentin, analgesics
-Respiratory support

Question 24

Botulism

Answer 24

-Food poisoning from clostridium botulinum
-Causes: improperly sterilized home-canned foods for older children (honey and corn syrup is common, not given if < 1 y), ingestion of farm soil

Question 25

Botulism s/s

Answer 25

-CNS s/s appear abruptly 12-36 hrs after ingestion
-Weakness, dizziness, headache, diplopia, speech problems, vomiting, progressive, respiratory paralysis, life-threatening

Question 26

Botulism mgmt

Answer 26

-IV botulism immunoglobulin (BIG-IV aka babyBIG) for infants
-Trivalent equine botulinum antitoxin and bivalent antitoxin for older children
-Respiratory support
-Therapy until paralysis abates

Question 27

Spinal cord injuries

Answer 27

-Generally result of indirect trauma (sudden hyperflexion or extension of neck)
-Especially in MVAs w/o child restraints
-Sports injuries
-Vertebral compression from blow to head or buttocks (diving, surfing, falling from horses)
-Birth injuries from traction force on spinal cord during breech delivery
-Higher injury –> more extensive the damage

Question 28

Spinal cord injury s/s

Answer 28

-Spinal shock syndrome: sudden disruption of CNS and ANS
-Absence of reflexes w/ flaccidity or limpness
-Loss of sensation or motor fxn
-Autonomic dysfxn: hypotension, low or high body temp, loss of bladder or bowel control
-Neurogenic shcok

Question 29

Spinal cord injuries mgmt

Answer 29

-Stabilization and transport to pediatric trauma center: ABCs, manage spinal shock
-IV steroids for children and adolescents, surgery for decompression
-Major complication: autonomic dysreflexia, neurogenic shock

Question 30

Autonomic dysreflexia

Answer 30

-Life threatening
-Caused by bladder distention
-HTN, headache, brady, diaphoresis, cardiac arrhythmias, flushing, piloerection, blurred vision, nasal congestion, anxiety, visual spots
-Tx: remove cause, IV antihypertensives, antispasmodics

Question 31

MDs

Answer 31

-Muscular dystrophies
-Largest group of muscular diseases in children
-Genetic origin
-Gradual degeneration of muscle fibers, progressive weakness, wasting of skeletal muscle
-Duchenne is common: increasing disability and deformity w/ loss of strength

Question 32

DMD

Answer 32

-Duchenne muscular dystrophy
-AKA pseudohypertrophic muscular dystrophy
-Most severe and common of muscular dystrophies of childhood
-X-linked recessive
-Positive fhx
-Fatal, life expectancy into early adulthood

Question 33

DMD s/s

Answer 33

-Appropriate developmental milestones early in life or subtle delays (onset 3-5 yrs)
-Progressive muscle weakness, wasting, contractures
-Waddling, gait, frequent falls, gower sign (pt uses hands to walk up their body from a kneeling position), lordosis
-Progressive generalized weakness in adolescence
-Profound muscular atrophy in later stages
-Death from RF or HF