Neuromuscular Dysfxn Flashcards
Cerebral palsy
Permanent dx of mvt and posture causing activity limitation due to nonprogressive disturbance that occurred in fetal or infant brain; defect of brain, not muscles
Causes of cerebral palsy
Shaken baby sx, lack of oxygen from cord wrapping around neck, infection in prenatal period, Rh incompatibility, maternal jaundice—watch closely while preg, stroke
How is cerebral palsy classified
By type of abnormality and severity—can be very mild and may not notice or more severe
Best type of walker for cerebral palsy
Reverse walker—forces them to stand up straighter
Type of CP and associated area of damage
Spastic CP—motor cortex
Dyskinetic CP—basal ganglia
Ataxic CP—cerebellum
Neurological abnormalities in CP brain
Faulty brain dev; brain abnormal mvt and posture
- narrower gyri and wider sulci
- small, smooth brain
- neural death from anoxia
Cerebral palsy diagnosis
- thru neuro exam and prenatal, delivery, postnatal hx
- persistent infantile reflexes are big sign (usually first sign)
- hx of preterm labor
- maternal infx
- failure to meet dev milestones on time (esp 6-12M)
- imaging and testing to confirm brain chx assoc with CP and rule out other lesions—CT, MRI
- diagnosis not confirmed until 2 YEARS—give time for brain to catch up and confirm
CP CM: motor abnormalities and dev milestones
- can’t sit up unassisted by 8M
- hypotonic
- very stiff arms and legs
- only crawling with one side (hand preference shouldn’t be shown until about 4Y)
- failure to smile by 2M
- feeding prob (gag, poor suck, choke, tongue thrust after 6M)
- extreme cry or irritability
- uncoor of involuntary mvt
- writhing mvts (with dystaxic CP)
- ataxia—uncoordinated; poor balance
CP CM: abnormal muscle tone
- Persistent Moro, tonic neck, grasp reflex past 6M
- hypotonic up to 1Y
- hypertonic after 1Y—resistance to passive ROM, hips higher than trunk when prone, inc deep tendon reflex, spine deformity w/ opisthotonic posture
- contractures—place rolled up washcloth in hand and might help
Spastic CP
Motor cortex prob
Upper motor neuron weakness; muscles are weak and moving lots, hypertonic, walk on toes and scissoring—walk with knees together
Most common type
Ataxic CP
Cerebellum prob
Unsteady shaking mvts, wide based gait, poor balance and coordination
Dyskinetic CP
Prob with basal ganglia
Chorea often (involuntary jerks), worm, writhing mvts
Mixed CP
At least 2 types of CP
- mild, mod, or severe
CP CM: associated impairments
- mental impairment (50-60% in normal limits)
- seizures
- ADHD
- incontinence
- non-ambulatory causes constipation, orthopedic probs, skin b/d, resp infx (asp pneumonia)
- feeding probs (may need a device)
- chewing, talking, swallowing prob, cavities, gingivitis
- impaired vision—nystagmus and lazy eye
- impaired hearing
CP management
- PT, OT, Speech
- assistive devices for ADLs and mobility
- surgery to release tendons and improve function
- meds for assoc sx—seizures, Botox, muscle relaxant, incontinence
- support at home, edu at school
- edu on meds, prevent skin b/d and resp infx
- need inc calories for Dyskinetic CP
- offer toys to affected side to encourage mvt
- safety
CP NC
- early ID
- assessment
- help improve all muscle tone and control
- freq rest
- nutritional needs—G tube, supp feedings
- routine skin assess
- immunizations
- safety (ex—modified car seat)
- family support—lifts for larger kids, assistive devices, therapy often, respite care—kids can even be brought into hospital if parents need break, positive reinforcement
Muscular dystrophy
Chronic muscle weakness
- Several types (Duchenne’s most common)
Duchenne muscular dystrophy transmission and etiology
- X-linked dx; mom passes defective gene to male offspring OR new mutation
- mutation in gene that encodes dystrophin (protein in muscles); absence causes muscle degeneration
- mostly affects males
Duchenne early CM
- onset btwn 3-5Y
- fine until then, normal dev, but weakness begins 3-5
- rapid progressive muscular degeneration after initial dev
- waddling gait
- lordosis
- positive Gower’s sign—need to use hands to push torso up to stand
Duchenne progressive CM
- pseudohypertrophy from fatty infiltration, not muscle growth
- muscular atrophy
- ambulation lost 10-12Y
- contractures from uneven loss of muscle tone
- obesity from inactivity—does not affect quality of life much
- facial and rest muscles atrophy
- cardiac or respiratory failure from weak muscles (lead to infx, especially respiratory)
- mild—brain mostly intact
- median age 27 with mech vent
Duchenne diagnosis
- from CM mostly
- serum enzymes
- muscle biopsy
- EMG shows motor changes
Duchenne therapeutic management
Maintain fxn in unaffected muscles as long as possible
- ROM, bracing, ADLs, surgery to release contractures
- genetic counseling of parents
- resp failure (cough assist, vent/trach, vax to prevent lung infx)
- cardiac failure (digoxin, diuretics)
- treat with experimental/newer drugs like transferase and steroids
Duchenne NC
- treat complications
- nutrition
- may need trach
- palliative care for fam and pt
- modify home environment
Spina bifida
Spine malformation
- posterior portion of lamina of vertebrae fails to close; neural tube fails to close by end of 4th week (issue bc many ppl don’t know they are pregnant yet)
Causes of spina bifida
Exact cause unknown
- linked with low folic acid before conception and in first semester
- more common in girls
Spina bifida dx
- most dx post-natal bc some types don’t cause probs
- if open sac on outside bod can diagnose prenatal
- based on imaging of meningeal sac
- neuro evaluation ongoing bc CM may not be present until child is dev delayed
Spina bifida occulta
Defect only in vertebrae; spinal cord and meninges normal
- missing a vertebrae so may see dimple or tuft of hair in area
- no sx; fine
- no neuro impairment
Spina bifida Cystica (2 types)
- Meningocele SBC—external sac encases meninges and spinal fluid but not associated with neuro defects
- Myelomeningocele SBC—external sac encases meninges, spinal fluid, and nerves; most in lumbar or lumbosacral area but higher defect on spine=more sx
Myelomeningocele CM
- sac-like protrusion at birth
- 90-95% have hydrocephalus (accum fluid in ventricle—big head due to Arnold-Chiari malformation)
- varying degrees of sensory and neuro dysfunction
- poor muscle tone in bladder and rectum
- flexion or extension contractures
Myelomeningocele surgery NC
- surgery to close sac w/i 24-72h (best w/i 12-18)
- cover sac with sterile, nonadherent dressing and leave pt on belly
- prevent infx is main goal
- avoid rectal temps bc poor anal sphincter tone which can cause rectal prolapse or lack of bowel control
- post-surgery VS
- watch incision
- latex free enviro bc kids who have surg at early age are more at risk
Myelomeningocele NC
- let parents hold and attach, feed—attachment may be delayed bc parents can’t hold right away
- keep prone for a bit
- may need orthopedic devices, prob need wheelchair
- prevent skin b/d and ulcers
- measure head circumference and fontanelles
- teach parents s/s infection
- adequate nutrition and hydration
- promote normal development
- emo and edu support, modified devices, good skincare
Myelominingocele Urinary and bowel NC
- tx urinary incontinence with in/out Cath (can learn by age 6)—don’t need sterile Cath but need great hygiene bc high risk infx
- vesicotomy/Mitranoff procedure can be done if child can maintain periods of dryness—make reservoir to hold urine
- tx bowel incontinence with bowel training, prevent constipation, high fiber and water, lax, digital stimulation, enemas
- antegrade continence enema procedure—appendix or ileum used to make catheterizable channel thru a stoma. Enema solution instilled straight into colon every 1-2 days, child has BM 20-30 min later
- need good hygiene
