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Peds II Flashcards

1
Q

What percent of infantile spasms pts have an identified etiology and/or significant developmental delay?

A

80%

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2
Q

What are the causes of infantile spasms? (4)

A
  • CNS malformation
  • Tuberous sclerosis
  • IEM
  • Congenital infectons
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3
Q

What is the genetic defect in most RCCs?

A

VHL

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4
Q

What are the EEG findings of infantile spasms?

A

hypsarrhythmia–totally erratic with high amplitude slow waves

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5
Q

What is the treatment for infantile spasms?

A

ACTH

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6
Q

What is the prognosis for infantile spasms?

A

If cryptogenic, normal development. Otherwise, developmental delays

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7
Q

What are the side effects of ACTH used in the treatment for infantile spasms?

A

HTN
Hyperglycemia
GI bleed
Immunosuppression

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8
Q

What is the drug of choice for infantile spasms 2/2 tuberous sclerosis?

A

Vigabatrin

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9
Q

What is the major side effect of vigabatrin? What should be followed with this mediation?

A

Visual field restriction that could be permanent

Serial ophthalmologic exams

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10
Q

What is the MOA of vigabatrin?

A

Inhibits the breakdown of GABA

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11
Q

Hypsarrhythmia is associated with what type of seizure?

A

Infantile spasms

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12
Q

What is Lennox-Gastaut syndrome?

A

Epilepsy syndrome characterized by T-C seizures, atypical absence, and complex partial (multiple types of seizures)

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13
Q

What is the prognosis of LGS?

A

Severe MR, with difficult to control seizures

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14
Q

What are the characteristic EEG findings of LGS?

A

Less than 2.5 spikes and slow wave charges

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15
Q

Less than 2.5 spikes and slow wave charges on EEG = ?

A

Lennox-Gastaut syndrome

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16
Q

What are the causes of Lennox-Gastaut syndrome?

A
  • Hypoxic ischemic encephalopathy
  • CNS malformation
  • IEM
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17
Q

What can infantile spasms progress to?

A

Lennox-Gastaut syndrome

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18
Q

What are neonatal seizures?

A

Any seizures in the 1st month of life

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19
Q

Why are generalized seizures not possible in neonate?

A

newborns have incomplete myelination between the hemispheres

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20
Q

What are the characteristics of neonatal seizures?

A

Usually focal tonic, focal clonic, or myoclonic

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21
Q

What are the manifestations of neonatal seizures? (5)

A
  • Repetitive mouth and tongue movements
  • Apnea
  • Pedalic movements
  • Autonomic phenomena
  • Tonic eye deviation
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22
Q

What are the 4 common causes of neonatal seizures?

A
  • Hypoxic ischemic encephalopathy
  • CNS infx
  • Intracranial bleed
  • Brain malformation
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23
Q

What are the two common electrolyte abnormalities that can cause neonatal seizures?

A

Hypocalcemia

Hyponatremia

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24
Q

True or false: hypoglycemia is a common cause of neonatal seizures

A

True

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25
What is the role of brain imaging in the workup for neonatal seizures?
Should be done to r/o congenital brain malformations
26
What is the treatment for neonatal seizures?
Phenobarbital (then fosphenytoin)
27
What is the prognosis for neonatal seizures?
Depends on etiology--but if nothing found, usually good.
28
What are the two key orders for working up neonatal seizures?
MRI of the brain, and CSF analysis
29
How can you differentiate jitteriness from seizures? (3)
- Jitters are tremor like, rather than jerking. - Induced by stimulation - Jitters can be stopped by holding the extremity
30
What is sleep myoclonus?
Common, benign phenomenon that occurs during sleep only, and is not associated with other s/sx
31
What, generally, is Guillain-Barre syndrome?
Immune mediated acute inflammatory demyelinating polyneuropathy
32
What is the most common cause of acute flaccid paralysis in children?
GB syndrome
33
What is the clinical presentation of GB syndrome (onset, progression)?
Sudden onset of symmetric, ascending flaccid paralysis, that begins with paresthesias in both feet
34
What is the bacteria that commonly sets off GB syndrome?
Campylobacter jejuni
35
What are the autonomic dysfunctions that can be seen with GB syndrome? (3)
- Cardiac arrhythmias - hypotension/HTN - urinary retention
36
What are the cranial neuropathies that can occur with GB syndrome?
- Facial diplegia - Drooling - Diplopia
37
What is the most severe autonomic dysfunction that can occur with GB syndrome?
Respiratory muscle failure
38
What are the exam findings with GB syndrome?
- Symmetric leg weakness | - *Diminished/absent reflexes*
39
True or false: sensory deficits are common with GB syndrome
False--sensory exam is usually normal, in spite of sensory complaints
40
True or false: the mental examination of a GB pt is usually normal
True
41
What are the CSF findings with GB syndrome?
Albuminocytological dissociation (increased protein with normal WBCs)
42
What is the most sensitive and specific test for GB syndrome?
Nerve conduction study
43
What are the spinal MRI findings with GB syndrome?
Nerve root enhancement
44
What is the treatment for GB syndrome?
-IVIG or plasmapheresis
45
What is the role of steroids in the treatment of GB syndrome?
Not helpful
46
What is the prognosis for GB syndrome in children?
Generally excellent, with complete recovery in the majority of pediatric patients
47
What is the usual natural h/o GB syndrome with treatment?
Worsening over 2-4 weeks, then plateau for weeks, followed by recovery over months
48
What is the inheritance of pattern of Duchenne muscular dystrophy?
XLR
49
What is the problematic protein in Duchenne muscular dystrophy?
Mutation in the dystrophin gene
50
Why is IVIG used over plasmapheresis in children?
Need central line for plasmapheresis
51
What age does Duchenne muscular dystrophy typically present? How does it present?
3 years old, with proximal muscle weakness affecting the legs before the arms
52
What is the classic sign of Duchenne muscular dystrophy?
Gower's sign
53
What are the calf findings of Duchenne muscular dystrophy?
Hypertrophic
54
What is the natural h/o Duchenne muscular dystrophy?
Slow, progressive weakness, with pts being wheelchair bound by age 12
55
What are the heart problems that can occur with Duchenne muscular dystrophy? (2)
Dilated cardiomyopathy and conduction abnormalities
56
What is the most common cause of death with Duchenne muscular dystrophy? When do they usually die?
Respiratory failure in the 20s
57
What is the treatment for Duchenne muscular dystrophy?
Daily, small dose of prednisone
58
Which is worse: Duchenne's or Becker's muscular dystrophy
Beckers--milder course
59
What is the lab that should be ordered if Duchenne muscular dystrophy is suspected?
Elevated Serum CPK
60
What is the inheritance pattern of Becker's muscular dystrophy?
XLR
61
What is the difference in the pathophysiology of between Duchenne and Becker's muscular dystrophy
Total loss of dystrophin in Duchenne, but only partial loss in Becker's
62
What is the definition of acute ataxia?
Unsteadiness of gait or fine movements of less than 72 hours duration
63
What are the 3 most common causes of acute ataxia in children?
- Postinfectious cerebellar ataxia - Intoxication - GB syndrome
64
What is postinfectious cerebellar ataxia?
rapid onset ataxia that occurs after a recent infection
65
What are the 3 main presenting symptoms of postinfectious cerebellar ataxia?
- Disturbance in balance - Dysarthria - Nystagmus
66
What are the exam findings of postinfectious cerebellar ataxia?
- Unsteady gait | - Truncal ataxia
67
What happens to mental status with postinfectious cerebellar ataxia?
Normal
68
What is the treatment and prognosis for postinfectious cerebellar ataxia?
No specific treatment required--most have complete recovery within a few weeks
69
In which seizure disorder is the EEG treated?
Infantile spasm
70
What is one specific sign that is very specific for a seizure in a neonate?
One sided spasm or eye deviation
71
What is the sign that indicates the need for admission to the ICU with GB?
Cranial neuropathy--although should probably be admitted anyway
72
True or false: an MRI is usually indicated in the workup of acute ataxia
True--need to exclude other causes

Neurology (23 decks)

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