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Neuromuscular management > Lecture 13 Pediatrics > Flashcards

Lecture 13 Pediatrics Flashcards

1
Q

When does the nervous system first appear in gestation

On average when does the neural tube form

A

21 days

25 days

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2
Q

What are the 3 primary brain vesicles

A

Prosencephalon, mesencephalon, rhombencephalon

Note: by week 5 there’s 5 vesicles

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3
Q

What part of the fetus brain becomes the cerebral hemispheres

What part becomes the midbrain

A

Telencephalon

Mesencephalon

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4
Q

What nutrient is essential for preventing neural tube defects

A

Folate

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5
Q

What is dysraphism?

What is anencephaly

A

Disorder of neural tube closure

Fatal disorder w/ absence of brain in development.

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6
Q

What are the risk factors of dysraphism

A

Low socioeconomic status, maternal nutritional status, teratogens (alcohol and medications)

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7
Q

What are the 3 types of spina bfidia

Occulta

Meningocele

Myelomeningocele

A

Occulta- vertebral arch defect

Meningocele- dura and arachnoid herniation

Myelomeningocele- herniation of spinal cord

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8
Q

Where is spina bfida occulta most common?

A

L5 to s1

Note: usually asymptomatic

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9
Q

What are the potential complications of Myelomeningocele

A
  • Hydrocephalus
  • Risk of tethered cord syndrome
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10
Q

What is tethered cord syndrome?

What category of motor neuron disorders does it cause?

A

Traction of spinal cord (stuck at one level and unable to move normally)

Causes lower motor neuron disorder

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11
Q

What is Arnold-chiari malformation

A

Hindbrain is sticking out of foramen magnum

Note: sometimes asymptomatic, if it’s over 5mm we are concerned

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12
Q

What are the 3 types of Arnold Chiari malformation

A

Type 1: cerebellar tonsils displaced > 6mm

Type 2: associated with Myelomeningocele

Type 3: associated with encephalocele

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13
Q

What are the symptoms of Arnold chiari malformation

A

Hydrocephalus, headache, cerebellar/medullary/cranial nerve signs

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14
Q

What is dandy walker malformation?

A

Cerebellar vermis developmental anomoly w/ large cyst in posterior fossa (theyre misising their cerebellum)

50% associated w/ mental retardation

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15
Q

How do doctors treat hydrocephalus in children?

A

Ventriculo-peritoneal shunt

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16
Q

Microcephaly

Macrocephaly

Craniostenosis

A

Microcephaly - small head (Causes: idiopathic, chromosomal, toxic)

Macrocephaly - Large head (hydrocephalus, genetic, gigantism)

Craniostenosis- Skull sutures fuse early and alter the shape of the head

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17
Q

How do you classify a global delay?

A

If there is a delay in 2+ of the domains.

-Gross motor
-fine motor
-expressive language
-receptive language
-social/adaptive behavior

There is a delay if a child is less than 70% of the norm

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18
Q

Sitting unsupported should happen at what age?

A

6 months

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19
Q

walking should happen at what age?

A

12 months

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20
Q

tricycle riding should happen at what age?

A

3 years

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21
Q

first words should happen at what age?

A

12 months

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22
Q

putting words together should happen at what age?

A

2 years

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23
Q

What is an important biological measurement that pediatricians measure on kids?

A

head size

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24
Q

child headsize that suddenly increases or decreases ____ percentiles is concerning

A

2 percentiles

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24
Q

What are the 5 normal primitive reflexes

A

Moro reflex
tonic neck reflex
grasp reflex
step reflex
crawl reflex

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24
Q

Red flag: head size ____________ percentiles

A

crossing 2

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25
Q

red flag: __________ to respond to sounds

A

failure

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26
Q

red flag: __________ beyond 3 months

A

fisting

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27
Q

red flag: rolling _______ 3 months

A

before (indicates one side is weak and other is favored)

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28
Q

Red flag: persistent __________ reflex after 6 months

A

MORO reflex (where they abduct their arms when theyre leaned back or when theyre startled)

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29
Q

red flag: persistent ____________________ reflex and not rolling beyond 7 months

A

asymmetric tonic neck reflex

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30
Q

red flag: Not sitting after ____________

A

9 months

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31
Q

red flag:

Delayed smiling ____________

laughing ______________

cooing _____________

first words ____________

A

> 1 month

> 2-3 month

> 3-4 months

> 12 months

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32
Q

red flag: handedness ___________________

A

before 12 months (indicates one side is weak and other is favored)

33
Q

red flag: persistent echolalia after _______

red flag: noticable __________ after 4 years

A

3 years

stutter

At any age: decline in speech ability or vocab

34
Q

How does a delay differ from a regression

A

Regression: acheived milestones are lost

more serious

35
Q

What are predominant causes of speech delays?

A
  • Multilingual family
  • Hearing loss
  • Infantile autism
36
Q

What conditions can mimic regression in a child

A
  • New onset of seizures
  • Misperception of milestones
  • Increased spasticity
  • Movement disorders
  • Hydrocephalus
37
Q

Down syndrome (trisomy 21)
And Fragile X are both examples of what kind of disorder?

Which is the most common cause of intellectual disability of this disorder class?

A

Chromosomal disorders

Fragile X is the most common chromosomal cause of intellectual disability

38
Q

Who does Down syndrome affect more?

Risk increases with advanced ______

2% risk of reoccurrence in next child

A

Men

Advanced maternal age (35+)

39
Q

What are the facial features associated with down syndrome

A

Broad flat face
Slanted eyes
Epicanthic eye fold
Short nose

40
Q

What are the facial features of fragile X syndrome?

What is the average IQ

A

Long face, enlarged ears, high arched palate

IQ 40-55

Note: should be considered in all cases with intellectual disability or autism spectrum disorders

41
Q

What does TORCHS stand for?

A

Infectious causes of developmental delay

  • Toxoplasmosis
  • Other
  • Rubella
  • Cytomegalovirus
  • Herpes or HIV
  • Syphilis
42
Q

What is the most important intervention for developmental delays

A

Refer to specialist if under 3

43
Q

Is cerebral palsy progressive or non progressive

A

Non progressive

CP is damage to the brain one time and then they’re left with permanent deficits but those deficits don’t get worse

44
Q

What can cause CP?

A

Any disturbance to the developing brain in utero or around the time of the delivery

45
Q

What is the most common time CP is caused?

Anenatal (before birth)

Perinatal (during birth)

Post natal

A

Anenatal

46
Q

What are protective factors against CP

A

Magnesium sulfate

Antibiotics

Corticosteroids

47
Q

What are the 4 CP classifications?

Which is most common?

A

Spastic (more common)

Dyskinetic

Ataxic

Mixed

48
Q

What are the classifications of spastic CP?

A

Spastic Hemiparesis

Spastic diplegia

Spastic quadriplegia

Athetoid/extrapyramidal

49
Q

How does spastic Hemiparesis present

A

Arm > leg

Child develops handedness early

18-50% intellectual disability

50
Q

What is damaged in spastic Hemiparesis and what is the common cause

A

Lesion of one corticospinal system in cerebral hemisphere

Stroke in MCA

51
Q

How does spastic diplegia present

A

Weakness in legs more then arms

Scissoring gait (adductor spasm)

Usually normal intelligence

52
Q

What causes spastic diplegia

A

Damage to ACA and PCA

53
Q

What is the most severe form of CP

A

Spastic quadriplegia

54
Q

What causes dyskinetic CP

A

Damage to basal ganglia

Causes chorea, dystonia, athetosis

55
Q

How does dyskinetic CP present

A

Hypotonia in infancy, abnormal movement starting at 1

Unable to walk

56
Q

What is the rarest form of CP

What structure is involved

A

Ataxic CP

Cerebellum

Truncal and gait ataxia that doesn’t respond well to to drugs or PT

57
Q

What is baclofen for

A

Spasticity

58
Q

What is commonly injected to treat CP

A

Botulinum (Botox)

59
Q

What is a selective dorsal rhizotomy

A

Surgery for CP

They cut the nerve causing spasticity

60
Q

Neurofibromatosis and tuberous sclerosis are what

A

Neurocutaneous disorder

61
Q

What are the two forms of neurofibromatosis

Which is more severe?

Which chromosomes are affected?

Peripheral or central?

A

NF1 - chromosome 17
Primarily affects skin and peripheral nerves

NF2- chromosome 22, primarily affects CNS, more severe

62
Q

Neurofibromatosis has what kind of inheritance

A

Autosomal dominant

63
Q

Tuberous sclerosis has what kind of inheritance

A

Autosomal dominant

64
Q

How does tuberous sclerosis present

A

Lesions in nervous system, skin, bones, retina, kidneys

White hard nodules on surface of brain and into ventricals

Calcifications

50% mental retardation seizures

Skin has depigmented macules (look like bad acne).

65
Q

What kind of genetic metabolic disorders are demyelinating

A

Leukodystrophies

66
Q

What kind of inheritance does adrenoleukodystrophy have?

A

recessive X linked (affects only boys)

67
Q

How does adrenoleukodystrophy usually present

A

Normal early development

At about 8 - progressive dementia, seizures, spasticity

68
Q

What is metachromatic leukodystrophy

A

Demyelination of PNS and CNS

can affect any age

Gait failure, mental deterioration, seizures

69
Q

In infants UMN damage does what to tone?

A

Decreases it

Unlike in adults where it increases tone

70
Q

What is duchennes muscular dystrophy?

What muscles is it evident in

A

Muscular dystrophy at 3-5

Hyperlordotic posture, toe walking, ankle dorsiflexor/ hip weakness

Calf pseudohypertrophy

71
Q

What is the prognosis of Duchens muscular dystrophy

A

More severe > BMD

boys will be in wheelchair by 9-12

Life expectancy about 20

1/3 with mental retardation

72
Q

How does Becker muscular dystrophy differ from duchens

A

Ambulatory for longer (30)

IQ normal

73
Q

How are dystrophies diagnosed

A

Phenotype (observation) -> test for high CK -> DNA analysis

74
Q

How does Becker muscular dystrophy present?

A

Myalgia

Isolated quad weakness

Rhabdo

Cardiomyopathy

75
Q

How is spinal muscular atrophy inherited?

How does it present

A

Autosomal recessive

Progressive loss of motor neurons in spinal cord

76
Q

Spinal muscular dystrophy
Type 0

Type 1

A

Type 0- prenatal - death

Type 1- age of onset 6 months.
Never Sit. Dies by 2

77
Q

Spinal muscular dystrophy

2

3

4

A

2- Never Stand (age 6-12 months)
70% survive to 25)

3- never run, age 12 months+ (nearly normal life expectancy)

4- adult onset. Proximal leg weakness

78
Q

What is the cool new 2 million dollar treatment for Spinal muscle atrophy

A

Zolgensma

One time treatment

79
Q

Where does infantile botulism come from

What does it cause

A

Honey and corn syrup

Prevents release of acetylcholine

Acute generalized weakness

80
Q

What nerve roots r most commonly hurt in kids due to brachial plexus injuries

A

C5-C6

Good prognosis

81
Q

Head pushed down away from shoulder causes what palsy

A

Erbs Palsy

C5-c6

Weakness of shoulder abduction, elbow flexion, supination

82
Q

Injury caused by arm pulled upward

A

Klumpke Palsy

C8-T1 damaged

Claw hand

Neuromuscular management (19 decks)

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