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Neurology > Peds II > Flashcards

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
Q

What is the role of brain imaging in the workup for neonatal seizures?

A

Should be done to r/o congenital brain malformations

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26
Q

What is the treatment for neonatal seizures?

A

Phenobarbital (then fosphenytoin)

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27
Q

What is the prognosis for neonatal seizures?

A

Depends on etiology–but if nothing found, usually good.

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28
Q

What are the two key orders for working up neonatal seizures?

A

MRI of the brain, and CSF analysis

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29
Q

How can you differentiate jitteriness from seizures? (3)

A
  • Jitters are tremor like, rather than jerking.
  • Induced by stimulation
  • Jitters can be stopped by holding the extremity
30
Q

What is sleep myoclonus?

A

Common, benign phenomenon that occurs during sleep only, and is not associated with other s/sx

31
Q

What, generally, is Guillain-Barre syndrome?

A

Immune mediated acute inflammatory demyelinating polyneuropathy

32
Q

What is the most common cause of acute flaccid paralysis in children?

A

GB syndrome

33
Q

What is the clinical presentation of GB syndrome (onset, progression)?

A

Sudden onset of symmetric, ascending flaccid paralysis, that begins with paresthesias in both feet

34
Q

What is the bacteria that commonly sets off GB syndrome?

A

Campylobacter jejuni

35
Q

What are the autonomic dysfunctions that can be seen with GB syndrome? (3)

A
  • Cardiac arrhythmias
  • hypotension/HTN
  • urinary retention
36
Q

What are the cranial neuropathies that can occur with GB syndrome?

A
  • Facial diplegia
  • Drooling
  • Diplopia
37
Q

What is the most severe autonomic dysfunction that can occur with GB syndrome?

A

Respiratory muscle failure

38
Q

What are the exam findings with GB syndrome?

A
  • Symmetric leg weakness

- Diminished/absent reflexes

39
Q

True or false: sensory deficits are common with GB syndrome

A

False–sensory exam is usually normal, in spite of sensory complaints

40
Q

True or false: the mental examination of a GB pt is usually normal

A

True

41
Q

What are the CSF findings with GB syndrome?

A

Albuminocytological dissociation (increased protein with normal WBCs)

42
Q

What is the most sensitive and specific test for GB syndrome?

A

Nerve conduction study

43
Q

What are the spinal MRI findings with GB syndrome?

A

Nerve root enhancement

44
Q

What is the treatment for GB syndrome?

A

-IVIG or plasmapheresis

45
Q

What is the role of steroids in the treatment of GB syndrome?

A

Not helpful

46
Q

What is the prognosis for GB syndrome in children?

A

Generally excellent, with complete recovery in the majority of pediatric patients

47
Q

What is the usual natural h/o GB syndrome with treatment?

A

Worsening over 2-4 weeks, then plateau for weeks, followed by recovery over months

48
Q

What is the inheritance of pattern of Duchenne muscular dystrophy?

A

XLR

49
Q

What is the problematic protein in Duchenne muscular dystrophy?

A

Mutation in the dystrophin gene

50
Q

Why is IVIG used over plasmapheresis in children?

A

Need central line for plasmapheresis

51
Q

What age does Duchenne muscular dystrophy typically present? How does it present?

A

3 years old, with proximal muscle weakness affecting the legs before the arms

52
Q

What is the classic sign of Duchenne muscular dystrophy?

A

Gower’s sign

53
Q

What are the calf findings of Duchenne muscular dystrophy?

A

Hypertrophic

54
Q

What is the natural h/o Duchenne muscular dystrophy?

A

Slow, progressive weakness, with pts being wheelchair bound by age 12

55
Q

What are the heart problems that can occur with Duchenne muscular dystrophy? (2)

A

Dilated cardiomyopathy and conduction abnormalities

56
Q

What is the most common cause of death with Duchenne muscular dystrophy? When do they usually die?

A

Respiratory failure in the 20s

57
Q

What is the treatment for Duchenne muscular dystrophy?

A

Daily, small dose of prednisone

58
Q

Which is worse: Duchenne’s or Becker’s muscular dystrophy

A

Beckers–milder course

59
Q

What is the lab that should be ordered if Duchenne muscular dystrophy is suspected?

A

Elevated Serum CPK

60
Q

What is the inheritance pattern of Becker’s muscular dystrophy?

A

XLR

61
Q

What is the difference in the pathophysiology of between Duchenne and Becker’s muscular dystrophy

A

Total loss of dystrophin in Duchenne, but only partial loss in Becker’s

62
Q

What is the definition of acute ataxia?

A

Unsteadiness of gait or fine movements of less than 72 hours duration

63
Q

What are the 3 most common causes of acute ataxia in children?

A
  • Postinfectious cerebellar ataxia
  • Intoxication
  • GB syndrome
64
Q

What is postinfectious cerebellar ataxia?

A

rapid onset ataxia that occurs after a recent infection

65
Q

What are the 3 main presenting symptoms of postinfectious cerebellar ataxia?

A
  • Disturbance in balance
  • Dysarthria
  • Nystagmus
66
Q

What are the exam findings of postinfectious cerebellar ataxia?

A
  • Unsteady gait

- Truncal ataxia

67
Q

What happens to mental status with postinfectious cerebellar ataxia?

A

Normal

68
Q

What is the treatment and prognosis for postinfectious cerebellar ataxia?

A

No specific treatment required–most have complete recovery within a few weeks

69
Q

In which seizure disorder is the EEG treated?

A

Infantile spasm

70
Q

What is one specific sign that is very specific for a seizure in a neonate?

A

One sided spasm or eye deviation

71
Q

What is the sign that indicates the need for admission to the ICU with GB?

A

Cranial neuropathy–although should probably be admitted anyway

72
Q

True or false: an MRI is usually indicated in the workup of acute ataxia

A

True–need to exclude other causes

Neurology (23 decks)

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