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Neurology Flashcards

1
Q

What embryonic age does primary neurulation occur? Secondary?

A

Primary (brain, spinal cord): 3-4 weeks

Secondary (meningocele, tetheted cord): 4-7 weeks

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2
Q

When would brain midline or cleavage defects occur (holoprosencephaly)?

A

2-3 months

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3
Q

What is the last pathway to myelinate?

A

Association bundle which connects the prefrontal cortex with temporal and parietal lobes (completes ~32 years!!)

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4
Q

What syndrome is associated with encephalocele?

A

Meckel-gruber syndrome (40% of patients with encephaloceles have other anomalies)

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5
Q

What maternal medications are associated with myelomeningoceles?

A

Valproate, carbamazepine

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6
Q

What level of myelomeningocele will make walking unlikely?

A

L2 and above

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7
Q

What are the types of Arnold Chiari malformation? (A primary neurolation defect)

A

Type 1 - only cerebellar tonsiles below foramen magnum (4th ventricle normal)
Type 2 - tonsils, 4th vent, choroid plexus, medulla displaced into spinal canal; associated with hydrocephalus
Type 3 - like a cervical meningocele, cerebellum and lower brainstem into sac

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8
Q

What is outcome of agenesis of corpus callosum?

A

Often normal if isolated. Higher incidence in people with dev delay. Increased risk of seizures later in life. Some assoc anomalies (holoprosencephaly, facial anomalies, cardiac, met disorders like nonketotic hyperglycinemia), trisomies

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9
Q

How to calculate cerebral perfusion pressure?

A

CPP= MAP - intracranial cerebral pressure

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10
Q

What causes decreased cerebral blood flow?

A

Low paCO2, high paO2, high hct, low fetal hemoglobin

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11
Q

What is Mobius syndrome?

A

Bilateral facial paresis. Caused by hypoplasia or absence of cranial nerve nuclei. No treatment or recovery

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12
Q

When is clonus normal?

A

5-10 beats normal in newborns, abnormal after 3 months

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13
Q

When does moro reflex appear? Disappear?

A

Appears: 28-32 weeks
Normal by: 37 weeks
Disappears: 6 months

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14
Q

Palmar grasp reflex

A

Appears: 28 weeks
Disappears: 2-4 months
*persistence is sign of athetoid cerebral palsy

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15
Q

How often is CSF remade?

A

5-7 hours, preterm: 10-30 ml; full term 40 ml. 99% water

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16
Q

What is the major cation in csf?

A

Sodium

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17
Q

What are delta brushes on eeg?

A

Signs of prematurity, appear 29 weeks, disappear at term

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18
Q

When does hypsarrythmia occur?

A

Usually after 2 months, in infantile myoclonic spasms

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19
Q

Features of Dandy Walker malformation

A
  1. Cystic dilation of 4th ventricle
  2. Cerebellar vermis hypoplasia or aplasia
  3. Hydrocephalus (75%), may not occur until months or years later
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20
Q

Symptoms of X-linked aqueductal stenosis

A

Aqueductal stenosis, adducted thumbs, agenesis of corpus callosum, mental deficiency

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21
Q

When HIE occurs are changes more in gray mayter or white matter?

A

White matter

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22
Q

What increases during hypoxia in HIE?

A

Increased:

  1. Cerebral blood flow
  2. Glucose influx to brain
  3. Glycogenolysis and glycolysis (to increase cAMP)
  4. Lacatate and H ions
  5. Excitatory amino acids, intracellular Ca, free radicals
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23
Q

What decreases during hypoxia in HIE?

A

Decreased:

  1. Oxidative phosphorylation
  2. Decreased brain glucose
  3. Phophocreatinine, ATP
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24
Q

What is most common artery for cerebral stroke in full term infant?

A

Left MCA (60%), right mca is 20%

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25
What is the most frequent intracranial hemorrhage?
Subarachnoid (more common in premature than full term). Good prognosis, often asymptomatic, may have early onset refractory seizures
26
When does IVH occur?
50% in first 24 hours, 90% by 72 hours
27
Which side is more common for brachial plexus injury? When does it usually resolve?
1. Right more common | 2. 88% normal by 4 months, 92% by 1 year. If still impaired at 15 months, usually persists
28
Is Erbs or Klumpkes more common
Erbs (90%), nerve roots C5-C7, waiters tip. Intact palmar grasp and hand moro, no should moro. Klumpkes C8-T1 (rare to be isolated)
29
How is SMA type I inherited? What is the location of defect?
Inheritance: autosomal recessive (chr 5) Location: degeneration of anterior horn cell
30
What is onset and life expectancy of SMA type I?
Onset: <6 months. Death < 2 years
31
What does EMG and muscle biopsy show in SMA type I?
EMG: nonspecific denervation, fasciculations, and fibrillations Muscle biopsy: atrophy of motor units
32
What proportion of neonates get acquired transient myasthenia gravis?
10-20%, but recurrence risk is 75%. No correlation with maternal disease severity.
33
How long does acquired neonatal myasthenia gravis last? Treatment?
Mean 18 days | Treatment: anticholinesterase therapy (neostigmine)
34
What is inheritance and location of defect for congenital myasthenia?
Inheritance: autosomal recessive Location: deficiency of endplate acetylcholine receptor
35
Inheritance of congential myotonic dystrophy
Autosomal dominant (chr 19), expanded CTG repeats. Almost exclusively inherited from mother
36
What is pathogenesis of congenital myotonic dystrophy?
Altered protein in muscle -> dysfunctional Na/K channels
37
What are maternal signs of congenital myotonic dystrophy?
Delayed release of hand grip, inability to open eyes completely after tightly shutting
38
What does EMG and muscle biopsy show for congenital mytotonic dystrophy?
EMG: myotonic changes eliciting a dive bomber sound | Muscle biopsy: small and round muscle fibers with large nuclei and sparse myofibrils
39
What is Riley-Day syndrome?
Rare, autosomal recessive. Ashkenazi. Chrom 9. Familial dysautonomia Reduced # of small unmyelinated nerves that control pain temp tast and mediate autonomic functions
40
How is Riley Day syndrome (familial dysautonomia) diagnosed?
No flare with intradermal histamine | Pupil constrictions with metachine eye drops or pilocarpine (normal eye should not restrict)
41
How is Prader Willi syndrome inherited? What chromosome?
Chromosome 15, autosomal recessive (maternal uniparental disomy 25%, 5% methylation). Deleted piece is always of paternal origin.
42
What is most common type and location of congenital intracranial tumors?
Teratomas (more common than neuroepithelial tumors), usually supratentorial (older children often infrarentorial)
43
What are signs of Sturge Weber syndrome)
1. Port wine stain in distribution of 1st trigeminal nerve 2. CNS ipsilateral intracortical calcifications 3. Glaucoma 30% 4. Seizures, mental deficits 5. Hemiparesis contralateral to facial lesion
44
How is Tuberous sclerosis inherited? Signs?
Inheritance: autosomal dominant (chr 9 and 16) Signs: 50% with ashleaf macules (most on trunk and buttocks), CNS tumors, eye symtoms. Seizures, mental deficits, cardiac rhabdomyomas. Enamil pits in teeth.
45
Inheritance and signs of Neurofibromatosis syndrome
Inheritance: autosomal dominant (chr 17) Signs: Cafe au lait spots, many, >1.5cm, usually not present at birth but 80% present by 1 year. Freckles in axilla, inguinal folds, perineum. Seizures, mental deficits. Tumors associated: pheochromocytoma, shwanoma, cutaneous neurofibroma
46
What are signs of McCune Albright syndrome?
Irregular brown pigmentations, fibrous dysplasia of bones, precocious puberty, hyperthyroidism, hyperparathyroidism, pituitary adenomas
47
What is inheritance and signs of Von Hippel Lindau disease?
Inheritance: Autosomal dominant Signs: tumors due to overexpression of transcription factor hypoxia inducible factor. CNS tumors (hemangioblastoma), retinal angiomas, pheochromocytomas
48
When is cerebral palsy typically diagnosed?
6-18 months corrected age
49
What proportion of hearing loss is genetic? Most common inheritance pattern? Most common genetic cause?
1. 50% is genetic 2. Majority autosomal recessive (70%) 3. Most common is mutation in connexin 26 gene, causes 20-30% of congenital hearing loss
50
What is most common cause of nonhereditary sensorineural hearing loss?
Congenital CMV
51
Why is ABR preferred hearing screen for nicu patients?
Ability to detect auditory dyssynchrony (normal canal and cochlea but processing from cochlea to auditory nerve is abnormal)
52
How many infants will fail first hearing screen? How many are actually abnormal?
2-7% will refer after first screen, but only 20% of those will have a hearing deficit
53
What is expected recovery time for facial nerve palsy at birth?
1-3 weeks complete recovery
54
Is SMA a disease of upper or lower motor neuron
Lower (anterior horn cell)
55
How is learning disability defined?
1. Scoring greater than or equal to 1 SD below mean on standardized testing. 2. Discrepeancy of 1 SD between IQ and standardized testing
56
What are disorders of primary neurulation?
``` Anencephaly Encephalocele Myelomeningocele Arnold Chiari Malformation Tethered cord ```
57
What are disorders of prosencephalic development?
Holoprosencephaly Agenesis of corpus callosum Septo optic dysplasia
58
What are disorders of neuronal migration?
Schizencephaly (no cortex) Lissencephaly (smooth brain) Pachygyria Polymicrogyria
59
What are disorders of neuronal organization?
Trisomy 21 Autism Angelman Fragile X
60
What type of stage in oligodendrocyte development is most common <28 weeks and most at risk for damage from PVL?
Pre-oligodendrocyte (then becomes immature oligodendrocytes by term corrected age)

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