Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

Symptoms of neuromuscular disorders?

A

progressive weakness, muscle atrophy, contracture, deformity, and progressive disability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Goals of PT in neuromuscular disorders:

A 
prevent deformity
prolong functional capacity
improve pulmonary function
facilitate development and assistance of family support
control pain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When is the onset of Duchenne, DMD?

A

1-4 years

rapid progression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

When is the loss of walking in Duchenne DMD?

A

9-10 years

death in late teens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Average IQ in Duchenne?

A

average IQ 85, 30% of boys have IQ<70 related to loss of dystrophin in brain.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Onset of Becker, BMD?

A

onset 5-10 years

slowly progressive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Function in Becker, BMD?

A

walking maintained past early teens, survival into 30’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Congenital MD:

A

birth, variable progression, shortened life span

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Congenital myotonic MD:

A

birth, slow progressions, significant intellectual impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What lab tests are evaluated with diagnosis of MD?

A

high serum creatine kinase, CK level

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Primary impairments in MD:

A

insidious muscle weakness secondary to progressive loss of myofibrils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Secondary impairments in MD:

A

contractures and postural malalignment in sitting and standing, scoliosis, decreased respiratory capacity, easily fatigued, occasional obesity; increasing caregiver assistance for ADL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Clinical Presentation

Children with MD

A

Symptom onset 2-5 yrs., diagnosed 5 yrs.

Early proximal muscle weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Posture of children presenting with MD:

A

Increased lordotic posture with mild scapular winging to keep COM behind hip joint for standing stability.
Scoliosis typically develops just before or during adolescence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Pseudohypertrophy

A

enlarged posterior calf due to infiltration of fatty and connective tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Gait of children presenting with MD:

A

broad BOS, waddling

delayed until 18 months- 50% of children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

PT in early symptom management:

A

Baseline strength, ROM data, monitor progression of muscle weakness.
Family support and education.
Address activity level, peer interactions, prognosis, coping support (family-centered care).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Where is muscle weakness in early staged in MD?

A

progressive, proximal muscles weaker early, progress faster

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Where do contractures usually develop in MD?

A

in plantar flexion and inversion of the foot, the hip and knee flexion contractures worsening with w/c use

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Where are early limitation in ROM in early stages of MD?

A

hamstrings, hip flexors, ITBs, and heel cords; contractures of hips and knees with increased time sitting

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Gowers sign

A

use of arms to push on thighs to attain standing from floor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Symptoms in early school age period in MD:

A

initial evidence of disability, functional activities slower.
clumsiness, falling, inability to keep up with peers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Gait in early school age period in MD:

A

mildly atypical gait, increased lateral trunk sway (waddling) that increases with running attempts
increase size of gastrocnemius muscles, ‘pseudohypertrophy’.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

When are children with MD most likely to lose independent ambulation?

A

by 9-10 years

25
Q

Gait deviations of MD:

A

Exaggerated lumbar lordosis – weakness of hip and knee extensors.
Gait deviations: increased BOS, pronounced lateral trunk sway, toe walking, shoulder retraction with lack of reciprocal arm swing

26
Q

Functional Grade 1

A

walks and climbs stairs without assistance

27
Q

Functional Grade 2

A

walks and climbs stairs with the aid of railing

28
Q

Functional Grade 3

A

walks and climbs stairs slowly (>12 sec, 4 stairs) without railing

29
Q

Functional Grade 4:

A

Walks unassisted and rises from chair, cannot climb stairs.

30
Q

Functional Grade 5:

A

Walks unassisted but cannot rise from chair or climb stairs.

31
Q

Functional Grade 6:

A

Walks only with assistance or walks independently with long leg braces

32
Q

Functional Grade 7:

A

Walks in long leg braces, but requires assistance for balance.

33
Q

Functional Grade 8:

A

Stand in long leg braces, but is unable to walk even with assistance

34
Q

Functional Grade 9:

A

uses a wheelchair

35
Q

Functional Grade 10:

A

confined to bed

36
Q

10 Meter walks test greater than 12 seconds:

A

predictive of loss of ambulation within 1 year

37
Q

What do sitting to standing transitions predict?

A

inability to transition predicts LOA within 2 years

38
Q

Muscle groups targeted for strengthening

A

Abdominals
Hip extensors and abductors
Knee extensors

39
Q

Exercises to avoid:

A

Avoid high resistance and eccentric exercise.
Participation in endurance exercises, e.g., cycling and swimming for overall conditioning, standing or walking daily (2-3 hours).

40
Q

Home stretching program:

A

1-2 times daily, slow, pain free stretch 5-10 repetitions, 10 sec hold, 10 minutes.

41
Q

Characteristics of pre-adolescent period:

A

8-10 years of age - increasing falls, c/o fatigue require guarding during walking, stair climbing
wheelchair fitting
difficulty standing

42
Q

Why is there difficulty standing in pre-adolescent period?

A

inability to maintain COG behind hip or in front of knee due to hip extensor and quadriceps weakness.

43
Q

Characteristics of adolescent period:

A

Increased difficulty with transfers; instruct in body mechanics, safety
Significant progression of disability, trunk weakness > loss of sitting balance
loss of walking by 10-12 years

44
Q

Indications for surgery:

A

Ankle plantar flexion contractures >10°
ITB contractures >20° but <45°
Posterior tibialis transfer for equinovarus foot.
Segmental spinal instrumentation early may improve sitting posture and alignment, quality of life

45
Q

Post-surgery PT:

A

standing in casts
gait training as tolerated
conditioning exercises for hips, trunk, UE’s
breathing exercises

46
Q

Sciolosis:

A

50% prevalence in 12-15 year-olds, 90% by 17 years

47
Q

Key muscles to maintain strength for transfers

A

Shoulder depressors, triceps – self-feeding, hygiene.

Shoulder flexors and abductors, elbow flexors

48
Q

Spinal Muscular Atrophy

A

Rare, autosomal recessive genetic defect on chromosome 5q13.
Abnormality of large anterior horn cells in spinal cord, with reduced number and progressive degeneration of remaining cells leading to muscle atrophy

49
Q

Which muscles are affected first in spinal muscular atrophy?

A

Proximal muscles and lung muscles

50
Q

Subtypes of SMA

A

Type I
Type II
Type II
Type IV

51
Q

SMA Type I:

A

0-6 mo

child is very weak, does not learn to sit

52
Q

SMA Type II:

A

(6-18 mo)

child sits but does not walk without AMD.

53
Q

SMA Type III:

A

(>12 mo)

child walks independently

54
Q

SMA Type IV:

A

adult onset

55
Q

Characteristics of SMA Type I:

A

Supine - hips flexed and abducted, elbows supported on surface.
Head lag in pull to sit.
Inability to attain Landau position.
Intolerance for prone position due to respiratory limitations, unable to prop, turn head to sides

56
Q

Characteristics of SMA Type II:

A

Initial presentation late in first year, infant is not pulling to stand.
Proximal weakness and wasting of extremity and trunk musculature.
Prone, quadruped difficult due to long lever arm.
Transitions to and from sitting difficult.
Delayed acquisition of motor skills, 90% sit by one year of age, 75% remain independent sitters until age 7, 50% until age 14.
Decline of abilities in second year.
Fine resting tremor, tongue fasciculations.

57
Q

Characteristic SMA Type III:

A

Progressive weakness, muscle wasting, onset toddler years into adulthood.
Proximal muscles affected first, may be confused with MD.
Absent reflexes and fasciculations.
Contractures and progressive spinal deformities are uncommon when ambulatory.
Symptom onset prior to age 2 have poorer prognosis, stop walking after 12 years.
PT to maintain function and flexibility, bracing for standing, walking

58
Q

Charcot-Marie-Tooth Disease

A

Hereditary motor and sensory neuropathy, HMSN.
Slowly progressive neuropathy affecting peripheral nerves.
Sensory loss, weakness, muscle wasting in distal muscles of feet, lower leg, hands, and forearms

Pediatrics (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions