Hypotonic infant Flashcards

1
Q

Tone

A

passive resistance to muscle movement- cannot be changed by voluntary control or exercise

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2
Q

Weakness

A

power of strength

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3
Q

Hypotonia

A

lowered resistance to passive movement when alert but not stimulated

differs from strength (weakness)

differs from fatigability

weak infants are always hypotonic
hypotonic infants may have normal strength

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4
Q

Causes of hypotonia

A

central nervous abnormalities

  • chromosome disorders
  • metabolic diseases- sepsis
  • spinal cord injuries
  • cerebral dysgenesis
  • hypoxic-ischemic injuries- obtained during birth

motor neuron- anterior horn cells

  • spinal muscular atrophies
  • traumatic myelopathy

nerve

  • no myelin or degeneration
  • preservation of intelligence- no cognitive, just motor

muscle

  • congenital myopathy
  • muscular dystrophy
  • central core disease/fibre myopathies

neuromuscular junction

  • infantile botulism
  • congenital and transient myasthenia gravis
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5
Q

History for hypotonia includes:

A

history of pregnancy

  • term
  • TORCH exposure or infections
  • hx of spontaneous abortions

delivery

  • hx of hypoxia of mother
  • abnormal physical exam at birth (dysmorphic, arthrogryposis)
  • APGAR

post natal period

  • milestones- Hx of motor delay with normal socialization decreases likelihood of CNS
  • clinical course (increase in tone vs. progressive weakness)
  • ability to feed

family hx
premature death

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6
Q

APGAR scoring system

A

Activity (tone)
0- absent
1- flexed arms and legs
2- active

Pulse
0- absent
1- bellow 100
2- over 100

Grimace (reflexes)
0- floppy
1- minimal response
2- prompt response to stimulation

Appearance (color)
0- blue, pale
1- pink body, blue extremities
2- pink

Respiration
0- absent
1- slow and irregular
2- vigorous cry

taken at 5, 10, & 30 min
score out of 10

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7
Q

Motor milestones

A

6 w- smiles

2/3 m- head lifted RED- lack of social response or vocalization

5-6- roll over
6- sit, finger feed self RED- poor head control, floppiness, not reaching

9m- crawls- sits steadily when unsupported and pivots around

11-12- stand up without assistance- RED not communicating by gestures (pointing) and not bearing weight on legs

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8
Q

Frog leg posture

A

abnormal at any age

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9
Q

Tone is maintained by

A

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

damage to cerebellar and motor strip = hypotonia
damage to red nucleus and basal ganglia= hypertonia

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10
Q

Primitive reflex

A

Moro- startle- head back, arms out then cry
6m

Tonic neck- turn head tonic contracture on that side
6-7m

Suck- touch root of mouth

Root- touch side of mouth

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11
Q

Horizontal suspension

A

attempt to lift head
flexes arms and legs

abnormal= draped like cloth

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12
Q

Vertical suspension

A

no pull through

flexion at knee, hip, ankle

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13
Q

Traction response

A

flex of arm
holds head
infant tries to help

measure of postural tone

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14
Q

Down syndrome- trisomy 21

A
microgenia (small chin) 
macroglossia (large tongue) 
simian crease (hand) 
eye folds 
thin upper lip 
railroad track ears 
upturned nose 
flat nasal bridge 
congenital heart defects 
otitis media 
short stature
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15
Q

Prader-Willi- deletion

A
narrow temple distance and nasal bridge 
almond shaped eyes 
thin upper lip 
overweight 
developmental delay
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16
Q

TORCH

A

toxoplasmosis, others (syphilis), rubella, CMV, Herpes

FISH (fluorescence in situ hybridization)
= blood test

17
Q

Central indicators

A

History of HIE (hypoxic ischemic event)
micro/macromegaly
abnormal eye movement

18
Q

Hypoxic ischemic encephalopathy

A

APGAR <7 at 5 minutes

life long disability

19
Q

Zellweger syndrome

A

autosomal recessive
inability to process phospholipids or bile acids

diminished or absent tendon reflexes
die within 1 yr of life

(Lorenzo’s oil)

20
Q

Pompe’s disease (glycogen storage disease)

A

autosomal recessive

normal development initially then obvious hypotonia, weakness, decreased movement

21
Q

Infantile botulism

A

constipation, hypotonia, ophthalmoplegia, bulbar signs

toxin mediated botulism blocks release of ACh at the NMJ –> descending paralysis

stool culture for dx

22
Q

Myasthenia gravis

A

ptosis, fatigue with feeding

diagnose with EMG