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Pediatrics > Neuromuscular Dysfxn > Flashcards

Neuromuscular Dysfxn Flashcards

1
Q

Cerebral palsy

A

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

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2
Q

Causes of cerebral palsy

A

Shaken baby sx, lack of oxygen from cord wrapping around neck, infection in prenatal period, Rh incompatibility, maternal jaundice—watch closely while preg, stroke

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3
Q

How is cerebral palsy classified

A

By type of abnormality and severity—can be very mild and may not notice or more severe

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4
Q

Best type of walker for cerebral palsy

A

Reverse walker—forces them to stand up straighter

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5
Q

Type of CP and associated area of damage

A

Spastic CP—motor cortex
Dyskinetic CP—basal ganglia
Ataxic CP—cerebellum

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6
Q

Neurological abnormalities in CP brain

A

Faulty brain dev; brain abnormal mvt and posture
- narrower gyri and wider sulci
- small, smooth brain
- neural death from anoxia

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7
Q

Cerebral palsy diagnosis

A
  • 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
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8
Q

CP CM: motor abnormalities and dev milestones

A
  • 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
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9
Q

CP CM: abnormal muscle tone

A
  • 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
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10
Q

Spastic CP

A

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

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11
Q

Ataxic CP

A

Cerebellum prob
Unsteady shaking mvts, wide based gait, poor balance and coordination

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12
Q

Dyskinetic CP

A

Prob with basal ganglia
Chorea often (involuntary jerks), worm, writhing mvts

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13
Q

Mixed CP

A

At least 2 types of CP
- mild, mod, or severe

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14
Q

CP CM: associated impairments

A
  • 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
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15
Q

CP management

A
  • 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
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16
Q

CP NC

A
  • 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
17
Q

Muscular dystrophy

A

Chronic muscle weakness
- Several types (Duchenne’s most common)

18
Q

Duchenne muscular dystrophy transmission and etiology

A
  • 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
19
Q

Duchenne early CM

A
  • 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
20
Q

Duchenne progressive CM

A
  • 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
21
Q

Duchenne diagnosis

A
  • from CM mostly
  • serum enzymes
  • muscle biopsy
  • EMG shows motor changes
22
Q

Duchenne therapeutic management

A

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

23
Q

Duchenne NC

A
  • treat complications
  • nutrition
  • may need trach
  • palliative care for fam and pt
  • modify home environment
24
Q

Spina bifida

A

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)

25
Q

Causes of spina bifida

A

Exact cause unknown
- linked with low folic acid before conception and in first semester
- more common in girls

26
Q

Spina bifida dx

A
  • 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
27
Q

Spina bifida occulta

A

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

28
Q

Spina bifida Cystica (2 types)

A
  • 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
29
Q

Myelomeningocele CM

A
  • 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
30
Q

Myelomeningocele surgery NC

A
  • 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
31
Q

Myelomeningocele NC

A
  • 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
32
Q

Myelominingocele Urinary and bowel NC

A
  • 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

Pediatrics (26 decks)

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