Exam 2 Flashcards
(128 cards)
1
Q
What is the main type of MD that only affects’ Boys
A
Duchenne
2
Q
Why Does Duchenne MD only affect Boys
A
It is a recessive gene tied to the X chromosome, boys only get 1 x so it is always expressed even though it is recessive
3
Q
What is the pathophysiology of MD
A
Absence of dystrophin leads to breakdown of muscle fibers
4
Q
When is MD usually diagnosed in boys
A
3-7 years
5
Q
Describe the pattern of weakness in MD
A
Neck flexors
Abdominals
Pelvic girdle
Proximal to distal UE musculature
6
Q
Describe pseudohypertrophy as seen in MD
A
Larger muscles due to increased fat and connective tissue content
Not actually stronger
7
Q
What percent of MD patients experience intellectual or behavioral problems
A
30%
8
Q
What is the primary impairment of MD
A
Insidious weakness
9
Q
What are secondary impairments of MD
A
Contractures
Postural malalignment
Osteopenia
decreased respiratory and cardiac capacity, GI motility issues
10
Q
How old are MD patients when they first need a wheelchair
A
8-12
11
Q
What 5 factors predict loss of ambulatory ability
A
50% reduction in leg strength
MMT <3 for hip extensors and <4 for ankle DF
Inability to climb stairs
More than 9 sec 10 meter time and inability to rise from the floor, predicts loss of walking within 2 years
10 meter time more than 12 seconds predicts ambulation loss within 1 year
12
Q
What is the primary goal for MD treatment
A
Function and participation, prolonged standing and ambulation
13
Q
What are some things to avoid with MD
A
Immobilization
aggressive strengthening
14
Q
What is the rough survival rate of children with cancer and why
A
83%
increased efficacy if interventions
15
Q
Survivor of cancer are ag great risk or what additional medical problems
A
Cognitive deficits
Functional impairments
Cardiovascular and pulmonary disease
16
Q
Treatment of pediatric cancer in the CNS alone can cause what late effects
A
Cognitive, hearing and visual deficits
17
Q
Treatment of pediatric cancer in the CNS, head, neck and gonads can cause what late effects
A
Endocrine abnormalities such as short stature, hypothyroidism, delayed secondary sexual development
18
Q
Treatment of pediatric cancer in the MSK system alone can cause what late effects
A
Scoliosis and spinal shortening
19
Q
What risk comes with receiving chemo or radiation as a child
A
10x greater chance of developing second malignancy
20
Q
Describe acute lymphoblastic leukemia (ALL)
A
80% of all pediatric cases
Commonly occurs in 2-5 years of age
Chemo for 2-3 years
over 90% survival rate
21
Q
Describe acute myelocytic leukemia (AML)
A
Most frequent in 0-2 years old
Survival rate of 63%
22
Q
What is the second most common type of cancer in pediatrics and third in adolecents
A
Brain and CNS tumors
23
Q
Name some brain and CNS tumors
A
Astrocytomas
Medullablastmas
Ependyomas
24
Q
Describe posterior fossa sydrome
A
Ataxia
cranial nerve involvement
Decreased cervical AROM
Cerebellar mutism
Headaches
Drowsiness
Irritability
25
Name some bone and soft tissue tumors
Osteoma
Ewing Sarcoma
26
What are some treatment strategies for bone and soft tissue tumors
Chemo
Surgery to remove
27
What are some SS of pediatric leukemias
Enlarged lymph nodes
Enlarged liver or spleen
Fever
Easy bleeding or bruising
Night sweats
Weight loss
28
What are some SS of pediatric lymphomas
Painless enlargement of lymph nodes
Night sweats
Persistent fatigue
Fever
Chills
Unexplained Weight loss
Anorexia
Pruritus
29
What are some SS of pediatric sarcomas
Interment pain that worsens at night
Swelling
Decreased ROM and altered gait
30
What are some SS of brain and CNS tumors
Headache
Vomiting
Vision, speech, hearing changes
Worsening balance
Poor gait
Unusual weakness or sleepiness
31
What are some acute side effects of chemotherapy
Anemia, fatigue, tiredness, reduced endurance, headaches, dizziness, damage to bone marrow, immune suppression, increased bruising, loss of appetite
32
What are some late effects of chemotherapy
Pulmonary, cardiac, endocrine and reproductive dysfunction
Osteoporosis and neurocognitive / neurosensory loss
33
What are some acute side effects of radiation therapy
N/V, Diarrhea, hair loss, mucositis, fatigue, skin changes
34
What are some late effects of radiation therapy
Fibrosis and tissue injury
35
During the conditioning phase before a bone marrow transplant what are patients most at risk for
Infection
Bruising
Fatigue
36
Describe the engraftment period of bone marrow transplant
In hospital for 28-35 days after treatment
Waiting to see if immune system begins to recover
Looking for absolute neutrophil count to increase by more than 500 cell/microliter 2 days in a row
37
What are some physical limitations caused by lymphoma or leukemia
Decreased ankle dorsiflexion strength and ROM and handgrip strength
Poor balance and postural control
Peripheral neuropathy
38
What are some physical limitations caused by osteosarcomas or Ewing's sarcoma
Increased effort with locomotion
Decreased sensation
Neuropathic and nociceptive pain from tumor
39
What are some physical limitations caused by central and peripheral nervous system tumors
Poor motor control
Abnormal muscle tone
Decreased Strength and ROM
40
What are some symptoms of anemia
Fatigue
Reduced endurance
Headaches
Dizziness
41
What are some treatment considerations for Leukemia
Bone pain should subside
May have peripheral neuropathy
Encourage aerobics
Rick of osteonecrosis present
42
What are some treatment considerations for Lower extremity sarcomas
Gait training with an AD
Avoid high impact and high torsion activities
43
What are some treatment considerations for brain tumors
Risk of posterior fossa syndrome
44
Describe spinal muscular atrophy
Degeneration of anterior horn cells with subsequent progressive weakness
45
Describe the clinical presentation of SMA
Symmetrical weakness of skeletal muscles
Progressive wasting
Hypotonia
Normal intellect and sensation
46
Describe SMA onset type 1
Infantile onset
Onset at 0-4 months
Rapidly progressive
Severe weakness
Mortality dependent on disease aggression and medial support
47
Describe SMA onset type 2
childhood onset
Onset at 6-12 months
Initial progression that becomes slowly progresive over years
Moderate to severe weakness
48
Describe SMA onset type 3
Juvenile onset
Onset 1-10 years
Slowly progressive
Mild impairment
49
Describe SMA function type 1
Non-sitter
Infantile onset
Significant issues with breathing and swallowing
Do not attain ability to sit without assistance
Muscle fasciculations of tongue are common
50
Describe SMA function type 2
Sitter
Onet around 18 months
Delayed milestones
Variable course - stable over long period of time
Fatigue is significant
May stand with support but will not ambulate without support or bracing
51
Describe SMA function type 3A
Walker
Diagnosed before 2 years old
50% retained the ability to walk past the age of 12
Tongue fasciculations in about 50%
Proximal LE weakness and fatigue are most common impairments
52
Describe SMA function type 3B
Walker
Diagnosed after 2 years old
50% retained the ability to walk pas 44th birthday
Tongue fasciculations in about 50%
Proximal LE weakness and fatigue are most common impairments
53
Describe intervention strategies of SMA type 1
ROM and strengthening, positioning to maintain flexibility, wedge for positioning, respiratory care, developmental activities like supported sitting with focus on head control, adaptive equipment / technology
54
Describe intervention strategies of SMA type 2
ROM and stretching, sitting posture, developmental activities aquatics, encouraging standing by 12-18 months, early fitting for KAFOs for standing, supported walking, mobility, respiratory care and fatigue management, managing scoliosis with corset until ages 10-12
55
Describe intervention strategies of SMA type 3
Exercise and AROM, strengthening and endurance, fatigue and overhead lifting, adaptive equipment, transfers and mobility training
56
What types of treatments are emerging in the evidence as potentially valuable for SMA
Gene replacement therapy
Spiranza
57
What is the most common cause of pediatric SCI
MVA
Sports for adolescents
58
What are some preventative measures that can be taken to avoid SCI
Seatbelts, car and booster seats
Rear facing car seats until 2 years of age
Water safety
Physicals before sports participation
59
Describe anterior cord syndrome
Motor paralysis
Decreased pain and temperature sensation
Poor prognosis
60
Describe the presentations of hemorrhages in the central part of the cervical spinal cord
Flaccid UE
Spastic LE
ambulatory, bowel and bladder intact
61
Describe posterior cord lesions
Loss of proprioception
Preserved motor function
Ambulation unlikely
62
Describe a brown sequard lesion
Ipsilateral paralysis and loss of proprioception
Contralateral loss of pain and temperature
Ambulation and bowel and bladder control likely
63
Describe cauda equina syndrome
Injury to L5 nerve roots
Areflexia of LEs and bladder
64
What are some treatment options for kids with SCI
Steroids
Hypothermia - may have neuroprotective effect
Stem cell transplants
Surgical stabilization
Cervical traction
Halo traction
Orthotics
65
Describe autonomic dysreflexia
Noxious stimulus
Elevate, remove compression stockings and abdominal binder or manage bowels
66
What are some OMs for SCI patients
FIM, WeeFIM
Spinal Cord Independence Measure
PEDI
Pediatric Neuromuscular Recovery Scale
67
Describe mobility device needs for various levels of injury
C5 and up - power chair
C6 - manual chair at home, power in community
C7-T1 Manual
68
Define spina bifida
Failure of neural tube to close primarily in the thoracolumbar region within the 1st 30 days post-conception
69
What are some causes of spina bifida
Genetic predisposition
Too little folic acid in mother
Maternal use of valproic acid
70
Describe spina bifida occulta
Asymptomatic
Found on x-ray
Tuft of hair or hyperpigmentation
May have tethered cord
May have urinary tract problems
71
Describe meningocele
Protruding sac
Only contains meninges and CSF
No motor or sensory problems
72
Describe Lipomeningocele
Fatty mass in lumbosacral region
Bowel and bladder dysfunction
Lower leg / foot disorders due to tethered cord syndrome
73
What are some surgical managements of spina bifida
Correction post Nataly
Shunt for hydrocephalus
Orthopedic repairs
74
What are some common impairments associated with spina bifida
Decreased strength and function below level of lesion
Decreased sensation below lesion
Hip sub and dislocations
Talipes equinovarus
hydrocephalus
Tethered cored syndrome
Bowel and bladder issues
75
What is the prevalence of hydrocephalus in spina bifida patients and what are the SS
90%
sunsetting eyes, bulging anterior fontanelle
76
What are some SS of shunt malfunction
Redness along shunt site
Headaches
Vomiting
Sunsetting eyes
77
What is a Arnold-chiari malformations
Cerebellum and brainstem displaced distally through foramen magnum
78
Describe tethered cord syndrome
Spinal cord adheres to the site of occlusion
Scoliosis
Gait issues
Bowel and bladder dysfunction
Decreased strength or sensation
79
How do you treat tethered cord syndrome
Dx with myelogram
Rx with surgical release
80
What group of people are at high risk of having a latex alergy
Spina bifida kids
81
What are some causes of TBI
Trauma
Anoxia
Non-traumatic event
82
Describe some injuries with shaken baby syndrome
Retinal swelling
Subdural hematoma
Retinal hemorrhaging
83
What are some secondary injuries associated with TBI
Hypoxia
Seizures
Hemorrhage
Brain Swelling
Extracranial causes
84
Describe dry drowning
Aspirating water
Laryngospasm cuts off wind pipe
Neurologic damage 33% of the time
85
Describe some behavioral and cognitive impairments seen in TBI
Emotional issues
Attention deficits
Distractibility
Impulsivity
Aggression
Decreased memory and learning difficulties
Language issues
86
Describe RLCF 3 treatment
Side Lying positioning
Contracture prevention
Prevent pressure ulcers
PROM
Avoid supine due to reflexes
Elevate head above heart to prevent increased intracranial pressure
Tilt table - 45 - 60 mins
87
Describe RLCF 2 treatment
Constant supervision
Constant daily routine
Repetition of tasks including orientation x3
Child can become frustrated, bores so trips to park or playground are helpful
88
Describe RLCF 1 treatment
Strengthening
Endurance
Locomotor training
CIMT
Gait and transfer
Stair climbing
Cardio fitness
89
Define a concussion
Complex physiologic process affecting the brain, induced by traumatic biomechanical forces
May or may not involve loss of consciousness
Functional rather than structural brain injury
90
What are the conditions to diagnose a concussion
Period of loss or decreased level of consciousness
Loss of memory either before or after the event
Any alteration in MS at the time of injury
91
Describe the stages of rehabilitation for concussion
No activity - complete rest
Light aerobic - <70% intensity no resistance
Sport specific exercise - Sports related drills, no head contact
Noncontact training drills - more complex drills, resistance training may begin
Full contact practice - normal activities after medical clearance
Return to play - normal play
92
How long is typical recovery after a concussion
2-4 weeks
93
Define intellectual disability
Significant limitations in both intellectual functioning and in adaptive behavior
< 70-75 IQ
94
Describe down syndrome
Genetic disorder resulting from trisomy 21
95
Describe how down syndrome is diagnosed
Amniocentesis at 13-14 weeks gestation
Chorionic villus sampling at 9-12 weeks
Alpha fetoprotein screening
Physical characteristics, extra skin on the back of the neck
96
What are some characteristics of down syndrome patients
Intellectual disability
Stereotypical facial features
Hypotonia
Ligamentous laxity
Joint hypermobility
Gross motor delay
Decreased strength
97
What are some comorbidities associated with down syndrome
GI tract anomalies
Thyroid issues
Visual and hearing impairments
Alzheimer's and dementia
Diabetes and obesity
98
When do down syndrome kids walk by
74% by 30 months
92% by 36
99
What are some common exam findings of down syndrome kids
Poor head control
Head lag with pull into sitting
Scapular winging
Genu recurvatum
Excessive flexibility
Slipping through sensation when held
Open mouth posture with tongue thrust
100
What findings at the ankle are to be expected with down syndrome kids
Pes planus
Calcaneal valgus
101
What are some intervention strategies for hypotonia
Stimulate co-contraction
Strengthen antigravity muscles
Prevent splits during transfers
102
What are some appropriate activities for down syndrome kids
bike riding
Sports
Aerobics
Swimming
Special Olympics
103
Describe DCD
Developmental coordination disorder
Lifetime condition
5-6% of kids
Boys more than girls
increased incidence with kids of low birth weight
104
What are some characteristics of DCD
Clumsy
Poor proprioception and motor planning
Difficulty adapting to environmental contexts
105
How is DCD diagnosed
DSM-5
difficulty with age appropriate activities even after extensive coaching
Absence of other explanatory diagnosis
106
What are some intervention strategies for DCD
Part task training
Process oriented approach
Perceptual motor training
Promote repetition
107
Define CP
Group of non-progressive motor disorders affecting movement and posture
108
What other types of disorders accompany CP
sensation, perception, cognition, communication, behavior, epilepsy and MSK problems
109
What group of kids have the highest incidence of developing CP
born before 28 weeks
Low birth weight, 2.2-2.3 lbs
110
What are some genetic causes of CP
Premature birth
Placenta abruption
Preeclampsia
111
Describe Periventricular leukomalacia
Developed in 80% of CP kids
Caused by hypoxic ischemic encephalopathy
112
Describe outcomes of PVL and IVH grades
1-2 likely to walk by age 2 and minimal risk of neurological impairment
3-4 only 10% walk and high risk of neurological impairment
113
What is a common risk factor of CP
Hypoxia or ischemia
114
What is the average age of diagnosis of CP
19 months
115
What are the 5 major atributes of gait
Sability in stance
Sufficient foot clearance in swing
Appropriate prepositioning of the foot for initial contact
Adequate step length
Energy conservation
116
Describe gait at 1 year
Wide BOS
High guard arm position
Absent reciprocal arm swing
Flat food initial contact
Exaggerated hip rotation
117
Describe gait at 1.5 year
Heel strike
High guard arm position
Reciprocal arm swing in most
171 steps per minute on average
Exaggerated hip rotation
118
Describe gait at 2 years
100% heel strike
Narrow BOS
Decreased hip rotation
Increased step length
119
Describe gait at 2.5 years
Reciprocal arm swing in nearly all
Mature control of hip abduction and adduction
Average cadence now 156
120
Describe gait at 3 years
Adult like BOS
All joint rotations smooth
Reciprocal arm swing 100%
121
Describe gait at 4 years
Reciprocal arm swing
All joint rotations look adult like
Adult like BOS
122
Describe gait at 5 years
Reciprocal arm swing
All joint rotations look adult like
Adult like base of support
Increased stride length
Increased velocity 1.08m/sec
Cadence now 154 steps/ min
123
Describe gait at 6 years
Cadence now 146 steps/min
124
Describe gait at 7 years
Increasing stride length and gait velocity (1.14m/sec)
Cadence now 143 steps/min
125
What can cause Trendelenburg gait
weak hip abductors and decreased pelvic stability
126
What can cause crouch in gait
poor hip and knee extension, increased pronation
127
What can cause foot drop in gait
weak ankle DFs
128
What can cause toe walking in gait
increased tone onto ankle PFs, tissue tightness on ankle PFs
