Pediatric Hypotonia

Question 1

Passive resistance to muscle movement

Answer 1

Tone

Question 2

Tone is maintained by _______, ________, and ________.

Answer 2

cerebellum at the red nucleus, basal ganglia, and motor strip

Question 3

Loss of strength

Answer 3

Weakness

Question 4

Possible causes of infantile hypotonia

Answer 4

Pathology of CNS, motor neurons, peripheral nerves, or muscles

Question 5

What three histories are most important to obtain during assessment of a hypotonic infant?

Answer 5

Prenatal, perinatal, and familial

TORCH exposure, term delivery, APGAR score, and milestones

Question 6

Components of APGAR score

Answer 6

Activity
Pulse
Grimace
Appearance
Respiration

Question 7

How high does the APGAR score go?

Answer 7

10

Question 8

Macroglossia, eye folds, palmar crease, hypotonia, congenital heart defects

Answer 8

Down’s syndrome (Trisomy 21)

Question 9

Severe hypotonia, thin upper lip, almond shapes eyes, short stature, hyperphagia, developmental delay

Answer 9

Prader Willi syndrome

Question 10

Motor milestone at 3 months

Answer 10

Holds head at 90 degrees in vertral suspension

Question 11

Fine motor and vision milestone at 3 months

Answer 11

Holds an object placed in the hand

Question 12

Hearing, speech, and language milestone at 3 months

Answer 12

Turns to sound

Question 13

Social, emotional, and behavioral milestones at 3 months

Answer 13

Hand regard, laughs, squeals

Question 14

Milestone red flags at 3 months

Answer 14

Lack of social response or vocalization

Question 15

Motor milestones at 6 months

Answer 15

No head lag on pull to sit; sits with support; lifts up on forearms in prone position

Question 16

Fine motor and vision milestones at 6 months

Answer 16

Palmar grasp of objects; transfers objects hand to hand

Question 17

Hearing, speech, and language milestone at 6 months

Answer 17

Vocalizations

Question 18

Social, emotional, and behavioral milestone at 6 months

Answer 18

May finger feed self

Question 19

Milestone red flags at 6 months

Answer 19

Poor head control; floppiness; not reaching

Question 20

Motor milestones at 12 months

Answer 20

Pulls to stand; cruises; may stand alone briefly; may walk alone

Question 21

Fine motor and vision milestones at 12 months

Answer 21

Puts block in cup; casts about

Question 22

Hearing, speech, and language milestones at 12 months

Answer 22

One or two words; imitates adult sounds

Question 23

Social, emotional, and behavioral milestones at 12 months

Answer 23

Imitates actives; object permanence established; stranger anxiety established; points to indicate want

Question 24

Milestone red flags at 12 months

Answer 24

Not communicating by gestures such as pointing; not weight bearing through legs

Question 25

Inability to process phospholipids or bile acids, hypotonia, seizures, diminished or absent tendon reflexes, usually die within 1 year

Answer 25

Zellweger syndrome

Question 26

Autosomal recessive; normal development initially then obvious hypotonia, weakness, and decreased movement; respiratory distress; cardiomegaly and hepatomegaly; absent DTRs; usually die by 2 years old

Answer 26

Pompe's disease

Question 27

Constipation, hypotonia, ophthalmoplegia, bulbar signs, descending paralysis, stool culture for diagnosis

Answer 27

Infantile botulism

Question 28

Severe hypotonia, difficulties with sucking and swallowing, respiratory problems develop, normal sensation and IQ, no reflexes, degeneration of alpha motor neurons

Answer 28

Spinal muscle atrophy

Question 29

Ptosis, fatigue with feeding, decreased strength with repeated stimulus, diagnosed with EMG

Answer 29

Myasthenia Gravis

Question 30

Passive resistance to movement of the extremities; rapid contraction in response to high intensity stretch

Answer 30

Phasic tone

Question 31

Resistance to passive movement of the axial muscles; prolonged contraction of antigravity muscles in response to low intensity stretch of gravity

Answer 31

Postural tone

Question 32

Most common cause of pediatric hypotonia

Answer 32

Hypoxia induced encephalopathy (HIE)

Question 33

Throw arms out then cry after sudden loss of support (until 6 months)

Answer 33

Moro reflex

Question 34

Turn head and observe tonic contracture on that side (until 6-7 months)

Answer 34

Tonic reflex (fencing reflex)

Question 35

Touch roof of mouth and elicit response

Answer 35

Suck reflex

Question 36

Touch side of mouth and infant turns head toward touch

Answer 36

Root reflex

Question 37

Place something in hand and infant grasps it (finger in hand)

Answer 37

Grasp reflex

Question 38

Lift child with one arm under chest and abdomen (face down, parallel with floor)

Answer 38

Horizontal suspension

Question 39

What does a normal (negative) horizontal suspension test involve?

Answer 39

Some flexion of arms and legs, attempt to lift head

Question 40

Most sensitive measure of postural tone, grasp supine infant's hands and pull to sitting position

Answer 40

Traction response

Question 41

What does a normal (negative) traction response test involve?

Answer 41

Head lag (minimal by 2 months), flexion of arms and legs, infant tries to help

Question 42

Pick up infant and hold between hands

Answer 42

Vertical suspension

Question 43

What does a normal (negative) vertical suspension test involve?

Answer 43

Head erect in midline with flexion at knee, hip, and ankle

Question 44

Arms held in flexion against the chest for a few seconds then quickly extended and released

Answer 44

Arm recoil

Question 45

What does a normal (negative) arm recoil test involve?

Answer 45

Arms should spring back to flexed position