Neurology Flashcards

1
Q

Most common cause of CP in term neonates in the US?

A

HIE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Most common neurologic complication of prematurity?

A

IVH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Most sensitive time for MRI for hypoxic ischemic brain injury?

A

24-96 hours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

MRI at what time shows full extent of brain injury (HIE)?

A

10 days (between 7-21 days)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Gold standard for diagnosing seizures?

A

Continuous EEG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Are all neonatal seizures correlated clinically?

A

No, 80-90% of seizures have NO clinical correlation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

When does edema peak in HUS for HIE?

A

24 hours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

When can you see diffusion changes on MRI in HIE?

A

Present 7-10 days, then pseudonormalize

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is basal ganglia injury predictive of?

A

Severity of motor injury (Cerebral palsy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is posterior limb, internal capsule injury predictive of?

A

Ability to walk at 2 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Most common type of neonatal stroke?

A

Arterial ischemic stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Most common presenting sign of stroke?

A

Seizures (>75%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Name factors that decrease cerebral blood flow

A

Hypocarbia, hypotension, hypothermia and polycythemia decrease cerebral blood flow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

An abnormality at which stage leads to anencephaly, and failure of what causes it?

A

Failure of the anterior tube closure during primary neurulation within first 26 days (weeks 3-4)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Maternal risk factors for anencephaly

A

Maternal hyperthermia, copper, zinc and folate deficiencies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Prenatal diagnostic factors for anencephaly

A

Elevated alpha fetoprotein
Polyhydramnios
Other anomalies: CDH, CHD, Trisomy 13 and 18, omphalocele
Detected on ultrasound at week 14-15

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What abnormality leads to encephalocele and when does it occur?

A

Failure of closure of rostral tube during primary neurulation (weeks 3-4)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Most common location for encephalocele

A

Occipital

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the prognosis of an encephalocele depend on?

A

The amount of brain tissue in the sac

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Why do myelomeningoceles happen? (defect)

A

Failure of posterior neural tube

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

In which location are most meningomyeloceles?

A

Lumbar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What does prognosis of a myelomeningocele depend on?

A

Level of lesion (lower the lesion, better the outcome), also presence of other anomalies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is an Arnold Chiari Type 1 malformation associated with?

A

Syringomyelia, hydromelia, skeletal anomalies (scolisos most common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is an Arnold Chiari Type 2 malformation associated with?

A

Myelomeningocele and hydrocephalus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Familial features associated with holoprosencephaly

A

Single maxillary central incisor
Ocular hypertelorism
Midfacial abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Aprosencephaly occurs due to a defect in?

A

Formation during the prosencephalic stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Holoprosencephaly occurs due to a defect in?

A

Cleavage during prosencephalic stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What changes in paCO2 and paO2 lead to increased cerebral blood flow?

A

Increase in paCO2 and decrease in paO2 cause increased CBF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Formula to calculate cerebral perfusion pressure

A

Mean arterial pressure - intracranial cerebral pressure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Changes in hemoglobin concentration and proportion of fetal hemoglobin leading to increased cerebral blood flow?

A

Decreased hemoglobin concentration

Increased proportion of fetal hemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Most common type of craniosynostosis? Caused by closure of which suture?

A

Dolicocephaly or scaphocephaly

Closure of saggital suture

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Craniosynostosis that is associated with Crouzon and Apert syndrome

A

Frontal plagiocephaly

Unilateral closure of coronal suture

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Craniosynostosis associated with Carpenter syndrome

A

Brachycephaly

Closure of bilateral coronal sutures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

At what age does the anterior fontanel completely close by

A

Age 2 years

35
Q

Syndrome with bilateral facial paresis?

A

Mobius syndrome

Secondary to hypoplasia or absence of cranial nerve nuclei

36
Q

Cause of hypertonia (injury to which structure)

A

Corticospinal tract or extrapyramidal system

37
Q

Persistence of palmar grasp is characteristic of

A

athetoid cerebral palsy

Normally disappears by 2-4 months

38
Q

Where is the choroid plexus located

A

Atrium of lateral ventricles

39
Q

When do choroid plexus cysts appear and disappear by? What syndrome can they be associated with?

A

Appear by week 11, disappear by week 26. Can be associated with trisomy 18

40
Q

Major cation in CSF?

A

Sodium

41
Q

At which gestational age do delta brushes appear and disappear on EEG?

A

Appear at weeks 29-30

Disappear at week 36-37 from central region and weeks 38-40 from occipital region

42
Q

What is hypsarrhythmia seen with?

A

Infantile myoclonic spasms

43
Q

What are central positive sharp waves seen with?

A

Premature infants with PVL

44
Q

Most common pattern of cerebral injury seen with term HIE

A

Selective neuronal necrosis

45
Q

Which artery is the most common site for focal cerebral ischemia

A

Left MCA. Left hemisphere is most commonly affected

46
Q

Most common clinical sequela of PVL?

A

Spastic diplegia (lower extremities more affected than upper extremities)

47
Q

Most common location of neuronal injury in hypoglycemia?

A

Bilateral, posterior occipital cortex

48
Q

Which nerve roots are damaged in Erb-Duchenne

A

C5-C7

49
Q

Which nerve roots are damaged in Klumpke’s

A

C8-T1

50
Q

Where is the defect/pathogenesis in SMA type 1?

A

Lower motor neuron - degeneration of anterior horn cell

51
Q

Inheritance pattern of SMA type 1

A

Autosomal recessive, chromosome 5

52
Q

EMG features in neonatal myasthenia gravis

A

Progressive decline in amplitude with repetitive nerve stimulation, returns to baseline after a period of rest or administration of neostigmine

53
Q

Inheritance pattern for myotonic dystrophy

A

Autosomal dominant, chromosome 19. Expanded repetition of CTG
Inherited almost entirely from mother

54
Q

What determines the severity of congenital myotonic dystrophy

A

Number of CTG repeats

55
Q

Muscle biopsy in congenital myotonic dystrophy

A

Abnormal: small and round muscle fibers, large nuclei, sparse myofibrils

56
Q

EMG in congenital myotonic dystrophy

A

Myotonic changes eliciting a dive bomber sound

57
Q

Prader Willi genetics

A

Deletion of 15q 11q13, some caused by maternal uniparental disomy
Deleted piece always of paternal origin

58
Q

Prader Willi clinical triad

A

Hypotonia, cryptorchidism, poor feeding

59
Q

Weight and growth pattern in Prader Willi

A

Failure to thrive during infancy, followed by obesity that presents between 6 months to 6 years

60
Q

Most common location for intracranial tumor

A

Supratentorial

61
Q

Most common presenting sign for an intracranial tumor

A

Increasing head circumference with bulging fontanel

62
Q

Features of Sturge Weber

A

Port wine stain, facial hemangioma (distribution of 1st division of trigeminal nerve). Present at birth
Glaucoma
CNS ipsilateral tramline intracortical calcifications
Seizures
Hemiparesis

63
Q

Features of tuberous sclerosis

A
Autosomal dominant
Hypopigmented ash leaf macules, greatest on trunk or buttocks
Cardiac rhabdomyomas
CNS tumors
Enamel pits in teeth
Seizures
64
Q

Features of neurofibromatosis

A

Autosomal dominant, chromosome 17
Cafe au lait spots, dont cross midline, have sharp borders, multiple
Freckling
Associated tumors: cutaneous neurofibromas, schwannoma, pheochromocytoma
Seizures

65
Q

Features of McCune Albright

A
Irregular brown pigmentation
Fibrous dysplasia of bones
Precocious puberty
Hyperthyroid
Hyperparathyroid
Pituitarty adenomas
66
Q

Features of von Hippel Lindau

A
Autosomal dominant, chromosome 3
Increased tumor growth
CNS tumor (hemangioblastoma, most commonly in cerebellum)
Multiple systemic hemangiomata
Retinal angiomas
Pheochromocytomas
67
Q

Most common type of CP with extreme prematurity

A

Spastic diplegia

68
Q

Most common type of CP with bilirubin encephalopathy

A

Athetoid CP

69
Q

Most common cause of multiple congenital contractures?

A

Amyoplasia

70
Q

Cause of benign familial neonatal seizures

A

Potassium channel

Positive family hx

71
Q

Gold standard for diagnosing neonatal seizures

A

Continuous EEG

72
Q

Normal EEG pattern for premies?

A

Discontinuous, switches to continuous ~31 weeks

Delta brushes start disappearing ~36 weeks

73
Q

Persistence of which reflex after 2-4 months is characteristic of athetoid CP?

A

Palmar grasp

Appears 28 weeks, well established 32 weeks, disappears by 2-4 months

74
Q

What is an asymmetric Moro suggestive of?

A

Peripheral nerve injury

75
Q

Persistence of tonic neck reflex after 6 months is suggestive of?

A

Focal cerebral abnormalities

76
Q

Falx laceration that leads to subdural hemorrhage is a result of rupture of which vessel?

A

Inferior saggital sinus

77
Q

Most common birth injury (excluding head stuff)?

A

Clavicle fracture

78
Q

Horner’s syndrome is a result of injury to which nerve root?

A

T1

79
Q

Most common traumatic nerve injury?

A

Facial nerve injury

80
Q

How do you bypass flexor predominance in the neonatal period during an examination?

A

By assessing both pronation and supination

81
Q

Signs of upper motor neuron dysfunction?

A

Hyperreflexia

82
Q

HIE cooling temperature?

A

33.5°C to 34.5°C

83
Q

Which anti-epileptic is most teratogenic?

A

Valproate

84
Q

Which anti-epileptic has the most effect on cognition?

A

Valproate