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Neurology Flashcards

1
Q

When does primary/secondary neurulation occur?

Dorsal induction

A

Primary: 3-4 weeks

Secondary: 4-7 weeks

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2
Q

What anomalies are associated with abnormal primary neurulation?

A 
Anencephaly
Myeloschisis
Encephalocele
Myelomeningocele
Arnold-Chiari malformation
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3
Q

What anomalies are associated with abnormal secondary neurulation?

A 
Spinal cord abnormalities
Lower sacral segments
Spinal cysts
Tethered cord
Lipoma
Teratoma
Myelocystocele
Meningocele,
Lipomeningocele
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4
Q

When does prosencephalic development occur?

Ventral induction

A

2-3 months

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5
Q

What anomalies are associated with prosencephalic development?

A

Formation: Aprosencephaly

Cleavage: Holoprosencephaly

Midline:
Agenesis of the corpus callosum
Agenesis of the septum pellucidum
Septo-optic dysplasia

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6
Q

When does neural and glial proliferation occur?

A

3-4 months

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7
Q

What anomalies occur during neural and glial proliferation?

A

Micrencephaly

Macrencephaly

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8
Q

When does neuronal migration occur?

A

3-5 months

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9
Q

What anomalies happen during neuronal migration?

A

Schizencephaly (no cortex)
Lissencephaly (smooth brain)
Pachygyria (broad gyri)
Polymicrogyria

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10
Q

When does neuronal organization occur?

A

Axonal proliferation: 3 months-birth

Dendritic and synapse: 6 months to 1 year

Synaptic rearrangements: birth to years

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11
Q

What disorders are association with abnormal neuronal organization?

A 
Mental deficiency
Trisomy 21
Fragile X syndrome
Autism
Angelman syndrome
Prematurity
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12
Q

When does myelination occur?

A

Birth to years

Corticospinal tract: 38 weeks to 2 years

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13
Q

Which neuronal pathway is last to myelinate? When does it complete?

A

Association bundle connecting prefrontal cortex to temporal and parietal lobes

32 years

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14
Q

What disorders are associated with abnormal myelination?

A

Cerebral white matter hypoplasia
Prematurity
Malnutrition

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15
Q

What causes anencephaly and when does it occur?

A

Abnormal primary neurulation

First 26 days of gestation

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16
Q

What is the incidence and epidemiology of anencephaly?

A

0.2-3 in 1000 births
Female
Hispanic women

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17
Q

What factors increase the risk of anencephaly?

A 
Maternal hyperthermia
Maternal deficiencies of
- Folate
- Copper
- Zinc
Previous anencephaly (2-5% recurrence)
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18
Q

What anomalies are associated with anencephaly?

A

13-33%:

  • CHD
  • CDH
  • Renal malformation
  • Hypoplastic adrenal glands
  • Omphalocele
  • Trisomy 13
  • Trisomy 18
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19
Q

Anencephaly is identified clinically through findings of

A 
Elevated AFP
Fetal ultrasound at 14 to 15 weeks
Karyotype
Polyhramnios
65% with spontaneous abortion
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20
Q

A primary neurulation defect that result in failed closure the rostral neural tube with herniation of meminges and brain is

A

Encephalocele

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21
Q

The percentage of encephaloceles that are associated with other anomalies

A

40%

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22
Q

70% of encephaloceles are located in the _____ region

A

Occipital

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23
Q

In encephalocele, AFP is usually

A

Normal

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24
Q

Prognosis of encephalocele is determined by

A

Amount of brain tissue within the sac
Presence of hydrocephalus, microcephaly, other anomalies
A frontal encephalocele has a better prognosis

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25
Q

Abnormal neurulation resulting in failure of posterior neural tube closure with open defects not covered by the skin is a

A

Myelomeningocele

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26
Q

In the United States myelomeningoceles occur most in the

A

East and south regions

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27
Q

Folic acid supplementation decreases the risk of neural tube defects

A

60 to 70%

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28
Q

The difference between a meningocele and a myelomeningocele is

A

Meningocele only meninges herniate through bony abnormality. Myelomeningocele both spinal cord and meninges herniate.

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29
Q

Myelomeningocele lesions below the level of ______ impact ambulation abilities

A

L3-4

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30
Q

In myelomeningocele reflexes are absent if the lesion is at level _____ or higher

A

L2

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31
Q

How often does hydrocephalus occur in the setting of myelomeningocele?

A

80%

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32
Q

A primary neurulation defect that causes displacement of the cerebellum tonsils through the foramen magnesium is

A

Arnold chiari malformation

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33
Q

Three types of Arnold Chiari malformation are marked by

A

Type 1- caudal displacement of tonsils

Type 2- elongation and displacement, hydrocephalus

Type 3- cerebellum and lower brainstem displaced to sac

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34
Q

Absence of telencephalon and diencephalon due to abnormal development in the prosencephalic stage is

A

Aprosencephaly

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35
Q

Clinical signs of Aprosencephaly can include

A

Intact skull and hair but small brain volume
Cyclopia or absence of eyes
Genitalia and limb anomalies

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36
Q

Abnormal development in the prosencephalic stage causing primary defect in cleavage is

A

Holoprosencephaly

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37
Q

Recurrence risk of holoprosencephaly is

A

6%

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38
Q

____% of holoprosencephaly is associated with a chromosomal abnormality.

A

40%

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39
Q

A maternal condition that increases the risk of holoprosencephaly

A

Maternal diabetes

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40
Q

Abnormal prosencephalic development leading to a single cerebral structure with a large central ventricle, absent corpus collosum, optic nerve hypoplasia, and dysmorphic features is

A

Holoprosencephaly

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41
Q

The outcome of holoprosencephaly is

A

Extremely poor with high rate of fetal demise

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42
Q

Extracranial abnormalities are present in holoprosencephaly _____%

A

50%

Myelomeningocele
Renal malformations
CHD
Polydactyly

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43
Q

Consequences of Neuro malfunctions in holoprosencephaly are

A

Apnea
Seizures
Hypothalamic dysfunction
DI/SIADH

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44
Q

A defect in midline development during the prosencephalic stage is

A

Agenesis of the corpus collosum

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45
Q

Associated abnormalities with agenesis of the corpus collosum

A 
Dandy Walker
Holoprosencephaly
Dysmorphic facies
CHD
Nonketotic hyperglycemia
Pyruvate dehydrogenase deficiency
Tri 8,13,18
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46
Q

Diagnostic confirmation of agenesis of the corpus collodum is with

A

MRI

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47
Q

Developmental outcome in agenesis of the corpus collosum is

A

Normal if no other anomalies
Increased rush if other neuro malformations
Increased risk of seizures

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48
Q

Medial, frontal and parietal lobes vascular supply is from the

A

Anterior cerebral artery

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49
Q

Lateral hemispheres receive vascular supply from

A

Middle cerebral artery

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50
Q

Midbrain, occipital lobes, and inferior temporal lobes receive vascular supply from

A

Posterior cerebral artery

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51
Q

The internal carotid artery supplies the

A

Anterior and middle cerebral arteries

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52
Q

The basilar artery supplies the

A

Posterior cerebral artery

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53
Q

Cerebral perfusion pressure is calculated:

A

MAP - ICP

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54
Q

Hypoglycemia and seizures can cause _______ cerebral blood flow

A

Increased

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55
Q

Anemia causes _____ cerebral blood flow

A

Decreased

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56
Q

Head circumference growth is approximately

A

1cm/week after 3rd week of life

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57
Q

Chromosomal abnormalities associated with microcephaly are

A 
Tri 13,18,21
Deletion 13q
CHARGE
Meckel Gruber
Smith Lemli Opitz
Infection
Maternal substance use 
Radiation
(Maternal) phenylketonuria
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58
Q

50% of macrocephaly is due to

A

Familial- benign familial macrocephaly (50%, male> female)

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59
Q

Chromosomal abnormalities associated with macrocephaly are

A 
Beckwith-Wiedeman
Neurofibromatosis
Soto syndrome
Fragile x
Achondroplasia
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60
Q

Craniosynostosis usually presents by

A

6 months

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61
Q

Syndromes known to be associated with craniosynostosis are

A

Crouzon

Apert

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62
Q

Metabolic causes of craniosynostosis

A

Hypophosphatemia
Rickets
Hypercalcemia

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63
Q

Goals of surgical intervention for craniosynostosis are

A

Prevent ICP
Allow brain growth
Prevent visual/auditory compromise
Skull/facial appearance

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64
Q

The most common form of craniosynostosis is

A

Scaphocephaly or dolichocephaly

56%

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65
Q

The least common type of craniosynostosis is

A

Occipital plagiocephaly (2%)

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66
Q

Sagittal suture craniosynostosis causes

A

Elongated skull
Scaphocephaly/dolichocephaly

Normal brain growth
Males»females

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67
Q

The most common craniosynostosis in Crouzon or Apert syndromes

A

Frontal plagiocephaly

Unilateral coronal suture closure
Females&raquo_space; males
High rate of neurodevelopment impacts

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68
Q

Craniosynostosis associated with carpenter syndrome is

A

Brachycephaly (13%)

Bilateral coronal suture closure

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69
Q

Craniosynostosis that cause hypotelorism is

A

Trigonocephaly (4%)

Metopic suture closure

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70
Q

The highest risk for ICP and mental deficiency from craniosynostosis is with

A

Multiple suture involvement

Surgery indicated as early as possible

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71
Q

Normal closure of the anterior fontanelle occurs by

A

2 years

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72
Q

An enlarged anterior fontanelle can indicate

A 
Hydrocephalus
CNS infection
Hypothyroidism
Tri 13, 18, 21
Zellweger syndrome
hypophosphatemia
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73
Q

The cranial nerve responsible for smell

A

One

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74
Q

The cranial nerve responsible for functions of the eye and movement are

A

2, 3, 4, 6

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75
Q

The cranial nerves responsible for facial sensation and movement

A

5, 7

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76
Q

Cranial nerve responsible for hearing

A

8

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77
Q

Sucking, swallowing, and tongue movement are responsibilities of cranial nerves:

A

5, 7, 9, 10, 12

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78
Q

Cranial nerves responsible for taste are

A

7, 9

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79
Q

Mobius syndrome is

A

Bilateral facial paresis due to hypoplasia or absence of cranial nerve nuclei

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80
Q

Pathologic hypertonia is due to

A

Corticospinal tract or extrapyramidal systems injury

HIE
Meningeal inflammation
Hemorrhage
Bilateral cerebral injury
Basal ganglia injury
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81
Q

An abnormal Dubowitz exam indicates

A

Neonatal hypotonia

Infant slips through hand when held under armpits

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82
Q

Syndromes commonly with hypotonia

A

Tri 21
Prader-Willi
Angelman

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83
Q

Metabolic disorders with hypotonia

A

Urea cycle defects
Isovaleric acidemia
Hypothyroidism
Hypermagnesemia

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84
Q

Contralateral hemiparesis, eye deviation, and gradient weakness in upper or lower extremities is suggestive of

A

Focal cerebral injury

Term: upper»> lower weakness

Preterm: lower&raquo_space;» upper weakness

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85
Q

Weakness in the proximal limbs, upper greater than lower is from

A

Parasagittal cerebral injury

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86
Q

Symmetric weakness in the lower greater than the upper extremity is from

A

Periventricular bilateral cerebral injury

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87
Q

Flaccid weakness in all extremities that evolves to spasticity is due to

A

Spinal cord injury

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88
Q

Flaccid weakness in all extremities associated with fasciculations is

A

Lower motor neuron injury

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89
Q

Focal weakness in specific patterns is due to

A

Nerve root injury

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90
Q

Generalized weakness is due to

A

Peripheral nerve injury

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91
Q

Generalized weakness with hypotonia is due to

A

Neuromuscular junction injury

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92
Q

Generalized weakness with hypotonia that may include the face and affects proximal muscles more than distal indicates

A

An injury at the level of the affected muscle

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93
Q

DTRs that appear normal at birth but become progressively more brisk are due to

A

Upper motor neuron lesion

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94
Q

DTRs that are absent with an asymmetric plantar response is due to

A

Lower motor neuron lesion

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95
Q

Unaffected DTRs with significant muscle weakness is due to

A

Neuromuscular junction injury

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96
Q

Decreased muscle strength with intact DTRs is due to

A

Muscle injury

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97
Q

Clonus is abnormal if present past ____ age

A

3 months

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98
Q

Moro reflex should disappear by ____ age

A

6 months

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99
Q

Asymmetric Morro reflex suggests

A

Peripheral nerve injury

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100
Q

Palmar reflex should disappear by _____

A

2-4 months

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101
Q

Persistent palmar grasp suggests

A

Athetoid cerebral palsy

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102
Q

Tonic neck reflex should disappear by ____

A

6 months

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103
Q

Persistent tonic neck reflex suggests

A

Focal cerebral abnormalities

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104
Q

Rooting reflex should disappear by

A

4 months

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105
Q

Crossed extensor reflex should disappear by

A

2 months

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106
Q

CSF is produced by the _______ at a rate of _____

A

Choroid plexus

0.4ml/min

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107
Q

CSF flows from the choroid plexus to the ______, ______, ______, ________, _______ , _________
MSF
LMS

A 
foramen of MONRO
cerebral aqueduct of SYLVIUS
FOURTH ventricle
lateral foramen of LUSCHKA and MAGENDIE
SUBARACHNOID space
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108
Q

The structure responsible for producing and regulating pressure of CSF

A

Choroid plexus

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109
Q

A neuroepithelial fold containing CSF and debris is a

A

Choroid plexus cyst

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110
Q

Most choroid plexus cysts appear at _____ and disappear by _____

A

11 weeks

26 weeks

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111
Q

A small number of choroid plexus cyst are associated with

A

Trisomy 18

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112
Q

CSF is recycled every

A

5-7 hours

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113
Q

A major electrolyte component of CSF is

A

Sodium

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114
Q

CSF concerning for IVH is

A

Xanthochromic with increased RBC and protein

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115
Q

A discontinuous EEG is find in infants age

A

27-30 weeks

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5
Perfectly
116
Q

EEG synchrony begins at ____ weeks during ______

A

31 sleep

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117
Q

EEG responds to external stimuli beginning at ____ weeks

A

34 weeks

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118
Q

Delta brushes on EEG are first seen at ____ weeks and disappear at _____

A

29 weeks

Term

119
Q

Theta bursts on EEG are first seen at ____ weeks and disappear at _____

A

29 weeks

35 weeks

120
Q

Abnormal progression of EEG pattern by more than ____ suggests

A

3 weeks

Underlying disorder

121
Q

Neuro insults, HIE, meningitis, encephalitis and metabolic disorders have ______ on EEG.

A

Background depression

122
Q

A lack of response to external stimuli appears as ______ on EEG

A

Burst suppression

Poor prognosis

123
Q

Cerebral cortical death is marked by ________ on EEG

A

Electrocerebral silence >72h

124
Q

________ are a typical finding on EEG in preterm infants. In term infants it came indicate ________

A

Periodic discharges

Middle cerebral artery infarction

125
Q

A prolonged high voltage on EEG is shown as ______

A

Multifocal sharp waves

Multiple etiologies

126
Q

PVL shows up on EEG as

A

Central positive sharp waves

127
Q

Hypsarrhythmia is characteristic of _____ and shows up after ______

A

Infantile myoclonic spasms

2 months

128
Q

Cranial US is least useful for lesions located ______

A

Posterior fossa

129
Q

Cranial CT is most useful for

A 
Intracranial calcifications >>>MRI
Parenchymal processes
Fluid collections
Posterior fossa lesions
Avoiding sedation (vs MRI)
130
Q

Brain MRI is better than CT for

A 
Avoiding radiation
Migration and myelination disorders
AV malformations
Ischemia
PVL
Spinal cord
131
Q

Most common cause of obstructive hydrocephalus is

A

IVH (post hemorrhagic)

132
Q

Aqueductal stenosis causes hydrocephalus by

A

Obstruction of the aqueduct causing 3rd and lateral ventricle dilation

1/3 of parents with hydrocephalus

133
Q

Causes of aqueductal stenosis are

A

Viral (mumps, rubella, parainfluenza)
Arnold Chiari
X-linked: adducted thumbs, agenesis of the corpus collosum, mental deficiency
AR with VACTERL

134
Q

A cystic dilation of the 4th ventricle, cerebellar vermis hypoplasia/aplasia with hydrocephalus is most likely

A

Dandy Walker malformation

135
Q

Hydrocephalus is due to Dandy Walker ____%

A

5-10%

136
Q

Typical management of Dandy Walker includes a

A

VP shunt

137
Q

Dandy Walker prognosis is related to

A

Age at onset (earlier is worse)

Severity of malformation

138
Q

Congenital hydrocephalus can be caused by

A

DW - T^2 I^2 G^2 - M:

Dandy Walker
Teratogens - Tumors
Infection- IVH
Genetics- vein of Galen
Maternal malnutrition
139
Q

Causes of communicating hydrocephalus

A

AA- CEGI- LL

Arnold chiari - Acquired hydrocephalus
Congenital absence of arachnoid granulation
GBS ventriculitis
IVH
Leptomeningeal inflammation -Lissencephaly

140
Q

What two forms of neuromuscular impairment are required for a diagnosis of HIE?

A

Spastic quadriplegia
OR
Dyskinetic CP

141
Q

Following hypoxia there is an increase in

A 
Cerebral blood flow
Glucose to the brain
Glycogenolysis
Glycolysis
Lactate, hydrogen ions
Excitatory amino acids, calcium, free radicals
142
Q

Hypoxia decreases

A

Oxidative phosphorylation
Brain glucose
Phosphocreatine
ATP

143
Q

Stage 1 Sarnat HIE can include

A

Hyperalert
Mild distal flexion
Weak suck reflex
Tachycardia

144
Q

Seizures are most likely part of stage ____ Sarnat HIE

A

Stage 2

145
Q

Lethargy, weak suck and Moro reflexes and mild hypotonia are consistent with stage _____ Sarnat HIE

A

Stage 2

146
Q

The diving reflex shifts ____

A

Blood flow preferentially to brain, heart, and adrenal glands

147
Q

HIE causes seizures that are _________ in about ____% of patients

A

Subtle, focal, multifocal or myoclonic

50%

148
Q

The time of hyperventilation in HIE is

A

Not recommended; may be detrimental

149
Q

In moderate and severe HIE, ____% and ____% of parents will have significant deficits

A

20% -higher risk for symptoms > 5-7d

100%

150
Q

The most common cerebral injury after HIE is

A

Selective neuronal necrosis

151
Q

Selective neuronal necrosis occurs ____ after injury due to ____

A

24-36h

Excitatory amino acids

152
Q

The area of the brain most likely impacted by selective neuronal necrosis is

A

Diffuse
Deep nuclear structures of the cerebral cortex
Deep nuclear structures (basal ganglia, thalamus, globus pallidus)

153
Q

In term infants with systemic hypotension, hypoxemia, acidosis, the likely injury is

A

Parasagittal cerebral injury

154
Q

In parasagittal cerebral injury, location of necrosis is marked by

A

Border areas of anterior, middle and posterior cerebral arteries
Typically bilateral and symmetrical

155
Q

Clinically parasagittal cerebral injury presents with

A

Proximal limbs weakness, upper» lower

Shoulder girdle weakness

156
Q

In parasagittal cerebral injury, deficits of the auditory, visual, spatial and language abilities suggests vascular involvement of the

A

Posterior cerebral artery

157
Q

Ischemia that is unilateral, left and most commonly involving the MCA is called

A

Focal or multifocal ischemia

158
Q

The etiology of focal or multifocal ischemia is most often

A

Unknown (50%)

Perinatal asphyxia (30%)

Other etiologies related to increased clotting risk:
Polycythemia
Protein C or S
Antithrombin 3 deficiency
Anti phospholipid antibodies
159
Q

Papillary response and oculomotor response in focal or multifocal ischemia are

A

Normal

160
Q

Periventricular hemorrhagic infarction is associated with __________ in 80% of cases

A

Large asymmetric IVH

161
Q

IVH leads to periventricular hemorrhagic infarction sure to

A

Obstructed blood flow in terminal vein from IVH causing venous infarction to medullary veins

162
Q

Most periventricular hemorrhagic infarction is located

A

Dorsal and lateral to lateral ventricle external angle

163
Q

Periventricular hemorrhagic infarction usually leads to

A

Spastic hemiparesis or asymmetric quadriparesis

Upper== lower extremities

164
Q

In contrast to periventricular hemorrhagic infarction, periventricular leukomalacia usually affects

A

Lower extremities more than upper extremities

Spastic diplegia

165
Q

Focal injury and necrosis of the periventricular white matter causes

A

PVL

166
Q

Anatomic factors that worsen risk for PVL in preterm infants are

A

Poor cerebral autoregulation
Actively differentiating or myelinating glial cells
Insults that lead to oligodendroglial cell death and myelin deficiency

167
Q

Best imaging and timing for PVL is

A

US at 1 month of age or more

168
Q

Outcome of PVL can include

A

Spastic diplegia

Cognitive and visual deficits

169
Q

Hemorrhage in term infants caused by trauma and testing of veins and venous sinuses is

A

Subdural hemorrhage

170
Q

Location of subdural hemorrhage can be

A

Infratentorial
Or
Over convexities

171
Q

Which location of subdural hemorrhage had a worsened severity and outcome?

A

Infratentorial

172
Q

Both infratentorial and convexity subdural hemorrhage can have an onset of

A

Delayed from injury several days (infratentorial) to months (over convexity)

173
Q

Convexity subdural may initially present with

A

Increasing head circumference

174
Q

Likelihood of hydrocephalus requiring VP shunt in subdural hemorrhage is

A

10-15%

175
Q

The most common type of intracranial bleed in infants is

A

Subarachnoid

176
Q

An uncommon type of intracranial bleed in infants causing brainstem compression is

A

Cerebellar hemorrhage

177
Q

A common outcome of cerebellar hemorrhage in infants is

A

Long term neurodevelopment deficits

178
Q

Diagnosis of cerebellar hemorrhage is best by

A

CT

179
Q

In VLBW infants, incidence of IVH ranges from

A

8% (1250-1500g) to 32% (<750g)

180
Q

Location of IVH are typically in the

A

Germinal matrix and subependymal germinal matrix

181
Q

___% of IVH occur in the first 72h of life

A

90%

182
Q

IVH can lead to

A

Posthemorrhagic hydrocephalus

PVL

183
Q

IVH occurs in ___% of healthy term newborn’s

A

2%

184
Q

In term infants, IVH results in ____% normal neuro outcome, ___% severe deficits, _____ requiring shunt, and ___% mortality

A

55% normal
40% severe deficits
50% shunt
5% mortality

185
Q

From most superficial to deep, rank subgaleal, cephalohematoma, caput, and extradural hemorrhage

A 
Captut
Subgaleal
Cephalohematoma
Extradural
Subdural
186
Q

Subgaleal hemorrhage can extravasate ___% of an infant’s blood volume

A

40%

187
Q

A rare bleed associated with birth trauma in the superiosteal space/inner surface of the skull caused by injury to the middle cerebral artery or veins is

A

Extradural hemorrhage

188
Q

Extradural hemorrhage management often includes

A

Evacuation to alleviate ICP

189
Q

Extradural hemorrhage can be associated with

A

Cephalohematoma

Linear skull fracture

190
Q

Brachial plexus injuries are most likely to involve ______ nerve roots on the _____ side

A

Upper

Right (90% unilateral)

191
Q

Recovery of brachial plexus injury is recovery of ____% by 4 months and ____% by 1 year.

A

88%

92%

192
Q

Full recovery of brachial plexus injury occurs with improvement at _______ and recovery by ______

A

2 weeks

6 months

193
Q

After brachial plexus injury, deficits remaining at ______ do not resolve

A

15 months

194
Q

Nerve roots affected in Erb-Duchenne palsy:

A

C5-7

195
Q

Nerve roots affected in Klumpke’s palsy:

A

C8-T1

196
Q

In brachial plexus injury, ______ palsy is most common

A

Erb’s (90%)

197
Q

Both Erb’s palsy and Klumpke’s palsy have an absent ________ reflex.

A

Biceps reflex

198
Q

Absent biceps reflex and shoulder moro, intact palmar grasp with waiters tip positioning is

A

Erb’s palsy

199
Q

Absent biceps, moro, and grasp reflex with weak hand flexors is

A

Klumpke’s palsy

200
Q

Miosis, ptosis, anhidrosis, and decreased iris pigmentation is consistent with injury to nerve root:

A

T1 (Horner’s syndrome)

201
Q

Flaccid weakness of the lower>upper extremities, atonic anal sphincter, abdominal muscle paralysis, and bladder distention results from

A

Spinal cord injury from traction/rotation injury during delivery

202
Q

Most common cranial nerve injury during birth is

A

Facial nerve

203
Q

Facial nerve palsy causes

A 
Facial muscle weakness
Asymmetric cry
Incomplete eyelid closure
Flat nasolabial fold
Left (75%)>>>right
204
Q

Expected recovery of facial nerve palsy is

A

1-3 weeks

205
Q

Lower motor neuron disorders:

A

CANMM:

Congenital myopathy
Anterior horn cell
Neuromuscular junction
Muscular dystrophy
Metabolic/multi-system disease
206
Q

Spinal muscle atrophy is an example of

A

Anterior horn cell, lower motor neuron disorders

207
Q

Spinal muscle atrophy genetics are

A

Autosomal recessive

Chromosome 5

208
Q

Presentation of SMA is

A 
Generalized hypotonia
Frog leg position
Jug handle arm position
Areflexia
Tongue fasciculations
Abdominal breathing
209
Q

SMA labs

A

Normal CPK
Denervation, fasciculations and fibrillations on EMG
Normal nerve conduction velocity
Atrophied motor units on muscle biopsy

210
Q

Acquired transient neonatal myasthenia gravis is a disorder of the

A

Neuromuscular junction

211
Q

Neonatal myasthenia gravis occurs in _____% of infants of mother’s with myasthenia gravis

A

10-20%

212
Q

Recurrence risk of neonatal myasthenia gravis is

A

75%

213
Q

Neonatal myasthenia gravis is caused by

A

Anti acetylcholine receptor antibodies

214
Q

Hypotonia, polyhramnios, pulmonary hypoplasia, arthrogryposis, respiratory failure and feeding difficulties occur with

A

Anti acetylcholine receptor antibodies,

Neonatal myasthenia gravis

215
Q

Transient neonatal myasthenia gravis usually lasts ______ and is treated with ___________

A

18 days

Anticholinesterase therapy

216
Q

If maternal history isn’t diagnostic, neonatal myasthenia gravis is diagnosed with

A

Normal CPK, muscle biopsy, nerve conduction velocity
EMG: progressive amplitude decline with repetitive nerve stimulation
Responsive to anti-cholinesterace treatment

217
Q

Congenital myasthenia gravis is in two forms:

A

Congenital myasthenia

Familial infantile myasthenia

218
Q

Genetic inheritance of birth forms of congenital myasthenia gravis is

A

Autosomal recessive

219
Q

Congenital myasthenia is caused by _________

A

Endplate acetylcholine receptor deficiency

220
Q

Familial infantile myasthenia is caused by _______

A

Acetylcholine synthesis or vesicle packaging deficiency

221
Q

Congenital myasthenia is __________ (more/less) severe than familial infantile myasthenia.

A

Less severe

222
Q

Congenital myasthenia presents with

A

Hypotonia: ptosis, ophthalmoplegia, facial weakness, poor suck and cry

223
Q

Familial infantile myasthenia presents with

A

Hypotonia, respiratory failure, apnea, severe feeding difficulties, facial weakness, ptosis

224
Q

EMG results for transient, congenital and familial myasthenia

A

Show progressive weakness with prolonged stimulation

225
Q

Congenital myotonic dystrophy is a form of muscular dystrophy inherited

A

Autosomal dominant
Chromosome 19
Expanding CTG repeats
Almost exclusive maternal inheritance

226
Q

Congenital myotonic dystrophy is caused by

A

Abnormal protein leading to dysfunctional sodium and potassium channels

227
Q

Polyhydramnios, prolonged labor, maternal difficulty opening eyes and releasing hang grip are consistent with

A

Congenital myotonic dystrophy

228
Q

Infants with congenital myotonic dystrophy present with

A 
Facial diplegia (tent-shaped mouth), poor feeding, respiratory failure, arthrogryposis, areflexia, muscle atrophy
Mental deficiency
Mortality in 40%
229
Q

Congenital myotonic dystrophy diagnostics show

A

Normal CPK and nerve conduction velocity
EMG: myotonic changes, “dive-bomber” sound
Muscle biopsy: small, round muscle fibers, large nuclei, sparse myofibrils

230
Q

Riley-Day, Prader-Willi, and arthrogryposis multiplex congenita are all disorders marked by

A

Hypotonia and muscle weakeness

231
Q

9q31-33 defective gene that transmits an autosomal recessive hypotonia disorder in Ashkenazi Jewish families is

A

Riley-Day

232
Q

Familial dysautonomia is diagnosed by

A

Riley-Day:

Pupil constriction with metacholine drops/pilocarpine
No flare with intradermal histamine
Genetics: 9q31-33 fever defect, AR

233
Q

The effects of familial dysautonomia/Riley-Day syndrome are due to:

A

Peripheral nervous system disorder:

  • Reduced number off small unmyelinated nerves for pain, temperature, taste, autonomic function mediation
  • Reduced large myelinated afferent nerve fibers
234
Q

Familial hypotonia with presentation by 1 year, absent corneal reflexes, decreased or absent DTR’s, and decreased tongue papillae is consistent with

A

Riley -Day or familial dysautonomia

235
Q

Deletion of 15q 11q13/maternal uniparental disomy/or maternal methylation causes a syndrome markers by hypotonia called

A

Prader-Willi

236
Q

In Prader-Willi, the deleted gene material at 15q11-13 is ________ origin

A

Paternal–> maternal uniparental disomy

237
Q

An infant with hypotonia, almond shaped eyes, cryptorchidism, and FTT followed by obesity is most likely

A

Prader-Willi

238
Q

Subtle seizures are marked by

A

Oral, facial, ocular activity
Swimming/pedaling movements
Vital sign changes or apnea

most frequent neonatal seizure type

239
Q

Multifocal tonic seizures are notable for

A

Clonic activity and movements of one limb that migrates to other parts of the body
“Jitteriness”
Minimal apnea or ocular involvement

Usually in term infants

240
Q

Focal clonic seizures are

A

Localized (one limb/area)
Don’t impact consciousness
Focal disease>metabolic disorders
More common in term infants

241
Q

Tonic and myoclonic seizures are more common in

A

Preterm infants

242
Q

Tonic seizures include

A

Posturing

Vital sign changes

243
Q

Myoclonic seizures appear like

A

Rapid jerks of extremities

244
Q

A deficiency that can cause and should be considered in treatment of seizures is

A

Pyridoxine

245
Q

A deficiency that can cause and should be considered in treatment of seizures is

A

Pyridoxine

246
Q

Half life of phenobarbital is

A

~100h

247
Q

Infants receiving phenytoin should be monitored for

A

Cardiac arrhythmias

248
Q

A prosencephalic vein malformation can lead to

A

Vein of Galen malformation

249
Q

Brain atrophy with a cranial bruit, hemorrhagic infarction and central ischemia is concerning for

A

Vein of Galen malformation

250
Q

The mechanism of injury from vein of Galen malformation is primarily

A

Intracranial steal

251
Q

Secondary effects of intracranial steal due to vein of Galen malformation are

A

Veinous thrombosis–> hemorrhagic infarction

High output CHF–> cerebral ischemia

Cranial compression–> brain atrophy

252
Q

CHF in vein of Galen malformation presents

A

Early: day 0-3
Refractory to medical management
Cranial bruit

253
Q

Outcomes of vein of Galen malformation are ______ and dependent on ________

A

High morbidity and mortality

Dependent on severity of CHF and success of management of CHF and embolization

254
Q

Most intracranial tumors in neonates are

A

Teratomas
Supratentorial
Present with increased FOC
Very poor prognosis

255
Q

Neurocutaneous syndromes are

A

STNVM :

Sturge-Weber
Tuberous sclerosis
Neurofibromatosis syndrome
Von Hippel-Lindau
McCune-Albright
256
Q

CP affects ____% of infants <1500g

A

5-20%

257
Q

Athetoid cerebral palsy is increased in

A

Bilirubin encephalopathy

258
Q

Apgar scores of 0-3 at 20 minutes increase risk of CP to

A

57%

259
Q

Most infants with CP have _____ apgar scores

A

Normal

260
Q

Most common type of CP is

A

Spastic

261
Q

CP with increased tone, DTR’s, normal cognition, and gross motor but not fine motor effects is characteristic of

A

Spastic CP

262
Q

A mixed tone CP involving gross and fine motor deficits, hearing and speech abnormalities is

A

Athetoid cerebral palsy

263
Q

The least common form of CP is

A

Ataxic

264
Q

CP most likely to have severe cognitive delay with decreased tone, coordination, and reflexes is

A

Ataxic CP

265
Q

Findings concerning for CP are

A

Hypotonia
Inability to suck
Weak cry
— >24h of life

266
Q

CP is typically diagnosed at ______ age and is non-progressive/progressive

A

6 to 18 months

Non-progressive

267
Q

Mental deficiency two are more standard deviations below the main IQ effects ______% of the population

A

3%

268
Q

The most common onset of etiologies causing mental deficiency are ________

A

Prenatal, 60 to 80%

269
Q

Prenatal causes of mental deficiency can include

A 
Trauma
Perinatal depression
Metabolic causes, characteristics, severe neonatal hypoglycemia
Infection, meningitis
Intracranial hemorrhage, stroke
270
Q

Postnatal and perinatal causes of mental deficiency each contribute approximately _____%

A

10%

271
Q

Profound mental deficiency is defined as an IQ of less than

A

<20

272
Q

An IQ of less than ______ defines mild mental deficiency

A

52-68

273
Q

New IQ classifications are based on

A

Level of support needed for daily activities of living

274
Q

Term infants hearing loss occurs at a rate of _____ as compared with premature infants of less than 32 weeks with a rate of _____

A

1/1000- profound, bilateral
2/1000- mild-moderate
1/1000- unilateral

Preterm: 2-4/100

275
Q

Most common cause of hearing loss is

A

Genetic, 50%

276
Q

Inheritance pattern of hearing loss is
_____% autosomal recessive
_____% autosomal dominant
_____% other

A

70%
15%
15%

277
Q

The most common gene defect implicated in genetic hearing loss is

A 
Connexin 26 (Cx26)
Accounts for 20 to 30% of congenital hearing loss
278
Q

Syndromes associated with hearing loss are

A 
Alport
CHARGE
Klippel-Feil
P-PR: Pendred, Pierre-Robin
Stickler
Treacher-collins
Tri 8/21
Usher
Waardenburg
279
Q

Acquired causes of hearing loss account for _____%

A

25%

280
Q

The most common cause of acquired hearing loss is

A

Congenital CMV infection

281
Q

Hearing loss is defined as inability to hear at_______ decibels

A

<21

282
Q

Normal hearing range is

A

-10 - 20

283
Q

Profound hearing loss is ______ decibels

A

> 90

284
Q

Sound transmission interference from the external auditory canal to the inner ear is

A

Conductive hearing loss

285
Q

Abnormal development or damage of cochlear hair cells or auditory nerve are

A

Sensorineural hearing loss

286
Q

Most common cause of conductive hearing loss is

A

Fluid in the middle ear

287
Q

____% cases should be detected in the newborn hearing screening

A

90%

288
Q

____ hearing screening is more likely to have a false positive due to excess debris or fluid in the external auditory canal

A

EOAE

289
Q

EOAE screening works

A

Measuring acoustic feedback from the cochlea

290
Q

ABR screening works by

A

Measuring the EEG waves generated in response to clicks

291
Q

Rescreening for infants who fail the EOAE screening should be done

A

Within 10 days using ABR

292
Q

Evaluation and management for failed hearing loss in the newborn period should include

A 
ENT consult
Genetics
Early intervention
Ophthalmology
Early use of amplification system, cochlear implant
293
Q

Of the ___% of infants it fail initial hearing screening, ___% will have a true hearing deficit

A

5%

20%

Neo Boards (15 decks)

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