The hypotonic infant Flashcards

1
Q

What’s the difference between hypotonia and muscle weakness?

A
  • Tone: passive resistance to muscle movement. Cannot be changed by voluntary control or exercise.
  • Hypotonia: lowered resistance to passive movement when an infant is alert, but not stimulated.
  • Weakness: decreased max muscle power than can be generated

***Weak infants are always hypotonic while hypotonic infants may have normal strength

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2
Q

What’s the difference between phasic and postural tone?

A
  • Phasic tone: The passive resistance to movement of the extremities/appendicular structures
  • Postural: The resistance to passive movement of the axial muscles (neck, back, trunk, “core”). The prolonged contraction of antigravity muscles in response to low intensity gravity stretch of gravity.
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3
Q

If you have rapid contraction of a muscle in response to high intensity stretch, this is mediated by which type of motor neurons?

A

Alpha motor neurons

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4
Q

The prolonged contraction of antigravity muscles in response to low intensity stretch of gravity is mediated by _________ and ________ motor neurons.

A

The prolonged contraction of antigravity muscles in response to low intensity stretch of gravity is mediated by gamma and alpha motor neurons.

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5
Q

The cerbellum is a muscle tone (facilitator or inhibitor). Therefore, damage to the cerebellum results in (hypertonia or hypotonia)

A

The cerebellum is a muscle tone facilitator. Therefore, damage to the cerebellum results in hypotonia

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6
Q

Midline cerebellum facilitates (axial or appendicular) muscle tone, while lateral cerebellum facilitates (axial or appendicular) muscle tone.

A
  • Midline cerebellum facilitates axial muscle tone
  • Lateral cerebellum facilitates appendicular muscle tone.
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7
Q

Red nucleus is a muscle tone (facilitator or inhibitor). Therefore, damage to red nucleus results in (hypertonia or hypotonia)

A

Red nucleus is a muscle tone inhibitor. Therefore, damage to red nucleus results in hypertonia

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8
Q

Basal ganglia/striatum is a muscle tone (facilitator or inhibitor). Therefore, damage to basal ganglia/striatum results in (hypertonia or hypotonia).

A

Basal ganglia/striatum is a muscle tone inhibitor. Therefore, damage to basal ganglia/striatum results in hypertonia.

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9
Q

Damage to the motor strip initially causes (hypertonia or hypotonia), followed by ___________.

A

Damage to the motor strip initially caused hypotonia followed by spasticity.

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10
Q

Tone is passive resistance to muscle tone maintained by which 4 brain structures?

A
  • Cerebellum
  • Red nucleus
  • Basal ganglia
  • Motor strip
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11
Q

When thinking about hypotonia in infants, it may have a cause due to ________, ________, ________, or ________.

A

When thinking about hypotonia in infants, it may have a cause due to:

  • CNS: chromosome disorder (i.e. Prader-Willi, Down syndrome), metabolic dz, spinal cord injury, hypoxic-ischemic injuries (usually during birth)
  • Motor neuron
  • Peripheral nerve: congenital hypomyelinating neuropathy, familiar dysautonomia, infantile neuraxonal degeneration
  • Muscle/NMJ: Muscular dystrophies, myopathies, congenital myasthenia gravis, ifantile botulism
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12
Q

Your history must include TORCH exposure. What does TORCH stand for?

A

Toxic plasmosis

Other: syphilis, varicella

Rubella

Cytomegalovirus (CMV)

Herpes

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13
Q

Another word for joint fixation at birth

A

Arthrogryposis. Seen in Zellweger syndrome.

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14
Q

Primitive reflex: what is the moro reflex?

A

While supporting infant’s neck, quickly lower its head/back to the table and should notice 3 distinct components:

  • Spreading out the arms (abductions)
  • Unspread the arms (adduction)
  • Crying (usually)

*Present up to 6 months

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15
Q

Primitive reflex: You turn the infant’s head to the side and you observe tonic contracture of the arm on the ipsilateral side. This is called a normal ______________ reflex.

A

Tonic neck fencing reflex. Should be present up to 6-7 months.

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16
Q

Primitive reflex: Describe the suck reflex.

A

Anything touches the roof of the mouth causing sucking. Present up to 4 months.

17
Q

Primitive reflex: describe rooting

A

Anything toucheing side of infant’s mouth and he turns to suck. Present up to 4 months.

18
Q

Primitive reflex: how long is the grasp reflex present?

A

Up to 2 months

19
Q

At what time intervals is the APGAR score assessed?

A

1 minute, 5 minute, and sometimes 10 mins. Should be a progression from very low score at 1 minute to very high score (hopefully 10) at 5 mins.

20
Q

What does APGAR stand for and how do you score points?

A
  • Activity:
    • 0: absent
    • 1: flexed arms and legs
    • 2: active
  • Pulse:
    • 0: absent
    • 1: <100 bpm
    • 2: >100 bpm
  • Grimace:
    • 0: floppy
    • 1: minimal response to stimulation
    • 2: prompt response to stimulation
  • Appearance:
    • 0: blue/pale
    • 1: pink body, blue extremities
    • 2: pink
  • Respiration:
    • 0: absent
    • 1: slow and irregular
    • 2: vigorous cry
21
Q

Pediatric milestones at age 3 months include…..

A
  • Holds head at 90º in ventral suspension
  • Holds an object placed in the hand
  • Turns to sound
  • Hand regard, laughs, and squeals

***Red flags: lack of social response or vocalization

22
Q

Pediatric milestones at age 6 months include….

A
  • No head lag on pull to sit; sits with support; in prone position lifts up on forearms
  • Palmar grasp of objects; transfers objects hand to hand
  • Vocalizations
  • May finger feed self

***Red flags: poor head control, floppiness, not reaching

23
Q

Pediatric milestones at age 12 months include….

A
  • Pulls to stand; ruises; may stand alone briefly; may walk alone
  • Puts block in cup, casts about
  • 1-2 words imitates adult sounds
  • Imitates activities; object permanence established; stranger anxiety established; points to indicate wants

***Red flags: not communicating by gestures, such as pointing; not weight bearing through legs

24
Q

Describe the traction response test

A
  • Most sensitive measure of postural tone
  • Grasp supine infants hands and pull to sitting position
  • Normal: Flexion of arms and legs is notable. Head lags, but minimal by 2 months.
  • Present at 33 weeks
  • Positive traction test is bad: child does not grasp hands or apply resistance with arms, head flops back.
25
Q

Describe the vertical suspension test

A
  • AKA axillary suspension
  • Pick up infant and hold between hands
  • Normal: head erect in midline with flexion at knee, hip, ankle
  • Hypotonia: infant slips through hands; also can check head control (falls forward)
26
Q

Describe the horizontal suspension reflex test

A
  • AKA ventral suspension
  • Lift infant with one arm under chest and abdomen
  • Assessing nuchal and truncal tones
  • Normal: some flexion of arms and legs; attempt to lift head
  • Abnormal: draped like cloth; no attempt to lift head; no flexion of legs
27
Q

Characteristic traits of Down syndrome include….

A
  • Trisomy 21
  • Epidemiology: 1/800 births (higher risk with older age of parents >35)
  • Microgenia
  • Macroglossia
  • Epicanthic eye folds
  • Palmar crease
  • Hypotonia
  • Congenital heart defects ***need cardiology eval and f/u
  • Upturned nose
  • Flat nasal bridge
28
Q

Characteristic traits of Prader Willi include….

A
  • Genetic: paternally derived (usually a deletion)
  • Clinical features:
    • severe hypotonia
    • thin upper lip
    • almond shaped eyes
    • hyperphagia beginning 1-6 y/o
    • Developmental delay - some do, some don’t
    • Decreased movement in-utero, weak cry, poor reflexes
29
Q

Hypoxic-ischemic encephalopathy is defined as APGAR of _____ at 5 minutes

A

Hyopoxic-ischemic encephalopathy is defined as APGAR of <7 at 5 minutes

30
Q

Describe Zellweger Syndrome

A
  • Genetic dz (autosomal recessive)
  • Inability to process phospholipids or bile acids
  • Presents with sever hypotonia, seizures, dysmorphic with high forehead, arthrogryposis
  • Diminished or absent tendon reflexes
  • Usually die within 1 yr of life
  • Lorenzo’s oil
31
Q

Describe Pompe’s Disease

A
  • Autosomal recessive
  • Glycogen storage dz
  • Nl development initially, then obvious hypotonia, weakness, decreased movement, poor swallow, pool secretions, absent DTRs
  • No tx - pts die by 2 y/o
32
Q

What are the two kinds of infantile myasthenia gravis?

A
  • Neonatal: maternal antibodies to ACh receptors, resolves with supportive care
  • Congenital: difficulty with packaging, delivery or recognition of ACh. Does not go away.