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NEUROMUSCULAR > pediatrics > Flashcards

pediatrics Flashcards

1
Q

when does the nervous system first appear

A

about 21 days of gestation

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2
Q

when does neural tube closer occurs

A

at 23-25 days from anterior to posteior

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3
Q

what are the 3 primary brain vesicles from

A

-prosencephalon
-mesencephalon
- phrobencephalon

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4
Q

by week ___ there are 5 vesicles

A

5

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5
Q

what is another word for neural tube defects

A

dysraphism

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6
Q

what is the diagnosis and prevention for dysraphism (neural tube deficits)

A

Diagnosis: ultrasound, alpha-fetal protein
* Prevention: Folate

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7
Q

is the dysraphism

A

disorders of neural tube closure

anterior neurpore open > cranial dysraphism

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8
Q

what is fatal , absent brain with associated skull defects like dysraphism

A

anencephaly

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9
Q

what is spinal dysraphism

A

spina bifida

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10
Q

what are the 3 different spina bifida

A

-occulta
-meningocele
- myelomeningocele

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11
Q

what type of spina bifida is
- vertebral arch defect only, up to 10% of population

-Dura and arachnoid herniation through vertebral defect

-herniation of spinal cord and meninges through defect

A

occulta

meingocele
myelomeningocele

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12
Q

if a pateint has a turf of hair on their low back what can we maybe suspect

A

spina bifida occulta

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13
Q

what are the symptoms for spina bifida occulta and where is it most common

A

usually asymptomatic
common at L5-S1
skin abnormality may be present over defect

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14
Q

what kind of deficits foes myelomeningocele have

A

myelopathy and/or radiculopathic

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15
Q

what is usually associated with myelomeningocele and what is there a later risk for

A

associated with hydrocephalus and later risk fro tethered cord syndrome

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16
Q

what is a prevention of normal cephalad movement of conus medullaris , child or adult develops progressive neurologic dysfunction due to traction on cord and/or nerve roots and is most common is unilateral lower motor neuron dysfucntion in one leg

A

tethered cord

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17
Q

what symptoms is most common in tethered cord

A

unilateral lower motor neuron dysfucntion in one leg

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18
Q

what can you see in the arnold chiari malformation on the CT/MRI scan

A

you can see the cerebellum going below the skull

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19
Q

what is a Congenital anomaly of hindbrain, downward elongation of brainstem and cerebellum into cervical portion of spinal cord

A

arnold chiari malformation

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20
Q

what is type 1 , 2 and 3 arnold chiari malformation

A

o Type I: cerebellar tonsils displaced > 6mm
o Type II: associated myelomeningocele.
o Type III: associated encephalocele

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21
Q

what are the symptoms for arnold chiari malformation

A
  • early hydrocephalus
    -later with cerebellar , medullary or CN signs
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22
Q

what is the treatment of arnold chiari malformation

A

closure of myelomeningocele and placement of shunt to relieve hydrocephalus

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23
Q

– Cerebellar vermis developmental anomaly with** large cyst** in posterior fossa
– 50% with associated mental retardation
– Hydrocephalus common
– Spastic diplegia from associated malformations

what is this

A

dandy walker malformation

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24
Q

dandy walker malformation .. there is 50% associate with what

A

associated mental retardation

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25
Q

what is common with dandy walker malformation

A

hydrocephalus

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26
Q

Ventriculo-peritoneal shunt for Treatment of _____

A

Hydrocephalus

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27
Q

what is microcephaly

A

small size of head (idiopathic, chromosomal, toxic)

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28
Q

what is Macrocephaly

A

Large head (hyrdrocephalus, genetic, gigantism)

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29
Q

what is Craniostenosis

A

skull sutures fuse early, altering the shape of the head

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30
Q

what are domains for the developmental domains

A

– Gross Motor
– Fine Motor
– Expressive Language
– Receptive Language
– Socialization/Adaptive Behaviors

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31
Q

what is the developmental quotient

A

developmental age/chronological age

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32
Q

what is global developmental delay

A

Delay in 2 or more domains

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33
Q

by 12 months waht shoudl a baby be doing

A

walking and first words

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34
Q

when should a baby be able to sit unsupported

A

6 month

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35
Q

when shoudl a baby be able to put words together and ride a tricycle

A

2 years and 3 years

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36
Q

what is the denver 2 scale used for

A

check milestone in kids

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37
Q

what is considered a vital sign for babies and is very important for measure of brain growth

A

head growth

38
Q

what are the 5 normal primitive reflexes for a bay

A
  • moro reflex (baby puts hands out if pretend like dropping them)
    -tonic neck reflex (baby will turn head to side of stretched out arm)
  • step reflex (baby will act like stepping when held up)
    -crawl reflex (baby will try to crawl when places on tummy
  • grasp reflex (baby will grasp ur finger if placed in palm)
39
Q

what are first 7 red flags for a baby

A
  • Head size crossing two percentiles
  • Failure to respond to sounds
  • Fisting beyond 3 months
  • Rolling before 3 months
  • Persistent MORO** >6months**
  • Persistent asymmetric tonic neck reflex, not rolling > 7 months
  • Not sitting>9months
40
Q

6, red flags continued ….

A
  • Delayed smiling (>1 mo.), laughing (>2-3 mo.), cooing (>3-4 mo.), first words (>12 mo.)
  • Handedness below 12 months
  • No words by 2years
  • Persistent echolalia >3yrs.
  • Noticeable stutter after 4 years
  • At any age: decline in speech ability or vocabulary
41
Q

what does IDEA define this as “significantly sub-average general intellectual functioning, existing concurrently with deficits in adaptive behavior and manifested during the developmental period, that adversely affects a child’s educational performance.”

A

intellectual disability

42
Q

is delay or regression worse and why

A

regression bc that is saying u learned something and then regressed backwards and loss the milestone

43
Q

what are causes of predominant speech delay

A

– Multilingual family
– Hearing loss
– Infantile Autism

44
Q

what are causes of Predominant Motor Delay:

A

– Ataxia
– Hemiplegia
– Hypotonia
– Paraplegia

45
Q

if a kid has 3 copes of trisomy 21 what syndrome do they have

A

down syndrome

46
Q
  • 1:700 live births; Male to Female ratio 3:2
  • Risk increases sharply with advanced maternal age (>age 35)
  • 2% risk of recurrence
  • Associated with:
    – Typical facial features
    – skeletal anomalies
    – infantile hypotonia
    – Intellectual disability
    – Seizures
    – congenital cardiac defects – GI structural defects

what is this

A

down syndrome : trisomy 21

47
Q

what is down syndrome associated with

A
  • typical facial features
  • skeletal anomalies
    -hypotonia
  • intellectual disability
    -seizures
    -cardiac defects
    -GI defects
48
Q

if a kid presents with a shirt stature , stubby finger and toes , increased space between 1st adn 2nd toes, small pelvis , inward curvature of 5th finger and antlatoaxial instability (AAI) of cervical spine what can we seuspect

A

down syndrome

49
Q

what is the most common chromosomal cause of intellectual disability and is MOST common in MALES

A

fragile x syndrome

50
Q

what is Caused by tri-nucleotide repeat expansion in the FMR1 gene and Clinical:
– IQ 40-55, Attention deficit and hyperactivity, seizures
– Long face, enlarged ears, macro-orchidism, hyperextensible joints, low muscle town, high arched palate

A

fragile x syndrome

51
Q

what is Toxic Cause Developmental Delay:

A

fetal alcohol syndrome

52
Q

what are infectious causes of developmental delay : intrauterine infections

A

TORCH(S)

– Toxoplasmosis
– Other (Zika)
– Rubella
– Cytomegalovirus
– Herpes Simplex, HIV – Syphilis

53
Q

what does Congenital Toxoplasmosis causes

A

calcification in the brain adn eye

54
Q

what is the most important intervention from developmental delay

A

referral to specialist if under 3

55
Q

– MRI
– Karyotype (± FISH)
– AST, ALT, NH3, lactate, CBC, Chem 20 – Serum amino acids, urine organic acids

what are these

A

usualt test for developmental delay

56
Q

• A group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to NON PROGRESSIVE disturbances occurring in the developing fetal or infant brain.
• Often accompanied by disturbances of sensation, perception, cognition, communication and behavior, by epilepsy and by secondary musculoskeletal problems

A

cerebral palsy

57
Q

is CP a progressive or non progressive disturbances occurring in the developing fetal or infant brain

A

non

58
Q

those who are born 28 was gestation have higher risk of what

A

CP

59
Q

what are the causes of CP

A

any disturbance of developing a brain in utero or around the time of delivery

hypoxia is 10%

60
Q

what are risk factors of antenatal for development of CP

A
  • pre mature
    -infections
    -multiple gestation
    -complications
61
Q

what are risk factors of perinatal for development of CP

A

-birth asphyxia
- complication

62
Q

what are risk factors of postnatal for development of CP

A
  • non accidental injury (child abuse)
    -head trauma
    -meningitis/ encephalitis
  • cardio arrest
63
Q

what are the 4 classifications of CP

A

-spastic (most common)
-dyskinetic
-ataxic
mixed

64
Q

how would u classify spastic CP

A

-unilateral or bilateral
-velocity dependent increased tone
- hyperreflexia
- UMN signs

65
Q

how would u classify dyskinetic CP

A

-recurrent uncontrolled/incoluntary movement
-tone may be abnormal
-dystonic
- choreoathletotic

66
Q

how would u classify ataxic CP

A

– Hypotonic
– Loss of coordination

67
Q

what is the most common mixed CP

A

Most commonly spastic with dyskinesias

68
Q

where is the lesion for spastic hemiparesis

A

lesionin the CORTICOSPINAL system of ONE cerebral hemisphere

69
Q

what is the most common causes of spastic hemiparesis

A

intrauterine stroke affecting middle cerebral artery

70
Q

does spastic hemiparesis affect the arm or leg more an when may it be apparent

A

arm and when child developed handedness before 18 months

71
Q

if a pt presents with a lesion in the corticopsinal system from an intrauterine stroke affecting the middle cerebral artery and affecting the arms more then the legs so the pt can work and is apparent in a 12 month old what do we think

A

spastic hemiparesis

72
Q

how will a patient with spastic diplegia present

A

spasticity and weakness in their legs

73
Q

what is spastic diplegia casued by

A

-prematurity with bilateral hemorrhages
or
- peri ventricular white matter disease
- perinatal ischemia in border zone of ACA and PCA

74
Q

what kind of gait will a pt with spastic diplegia presents with

A

scissoring gait bc of adductor spams

75
Q

if a patient presents with spasticity and weakness in the left and presents with a SCISSORING gait w normal intelligence what so we suspect

A

spastic diplegia

76
Q

what is the most severe spastic variant of CP

A

spastic quadriplegia

77
Q

what is wrong with teh brain in spastic quadriplegia , what is it associated with and what is frequent

A

diffuse malformation
associated with mod-serve intellectual disability
frequent seizures

78
Q

what are casues of spastic quadriplegia

A

hypoxia -ischemia , infection , CNS malformation

79
Q

where is the lesion for dyskinetic CP and whar does it present with

A

BG lesions leading to chorea , dystonia and athetosis

80
Q

what kind of CP does neonatal hyperbilirubinemia
(kernicterus) and severe anoxia cause

A

dyskinetic CP

81
Q

can children walk with dyskinetic CP n

A

naur

82
Q

kids with dyskinetic CP will present with ____ in infancy , abnormal movements will begin at 1 year old

A

hypotonia

83
Q

what is the rarest form of CP and is the abnormal development of cerebellum or its pathways

A

ataxia CP

84
Q

what kind of ataxia does ataxic CP present with

A

truncal and gait

85
Q

do drugs help ataxic CP

A

NAURRR may improve with age tho

86
Q

what are the oral medication for CP spasticity

A

• baclofen
• Diazepma
• tizanidine

Others: trihexyphenidyl, gabapentin, tetrabenazine)

87
Q

• baclofen
• Diazepma
• tizanidine,
• Others: trihexyphenidyl, gabapentin, tetrabenazine)

what are these medications for

A

CP spasticity

88
Q

what is the injection for CP spasticity

A

botulinum A

89
Q

what is the sx for CP spasticity

A

• Intrathecal baclofen
• selective dorsal rhizotomy
• DBS

90
Q

what is the prognosis for mild to moderate CP

A

normal life

NEUROMUSCULAR (15 decks)

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