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Flashcards in Peds II Deck (72)
1

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

80%

2

What are the causes of infantile spasms? (4)

-CNS malformation
-Tuberous sclerosis
-IEM
-Congenital infectons

3

What is the genetic defect in most RCCs?

VHL

4

What are the EEG findings of infantile spasms?

hypsarrhythmia--totally erratic with high amplitude slow waves

5

What is the treatment for infantile spasms?

ACTH

6

What is the prognosis for infantile spasms?

If cryptogenic, normal development. Otherwise, developmental delays

7

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

HTN
Hyperglycemia
GI bleed
Immunosuppression

8

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

Vigabatrin

9

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

Visual field restriction that could be permanent

Serial ophthalmologic exams

10

What is the MOA of vigabatrin?

Inhibits the breakdown of GABA

11

Hypsarrhythmia is associated with what type of seizure?

Infantile spasms

12

What is Lennox-Gastaut syndrome?

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

13

What is the prognosis of LGS?

Severe MR, with difficult to control seizures

14

What are the characteristic EEG findings of LGS?

Less than 2.5 spikes and slow wave charges

15

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

Lennox-Gastaut syndrome

16

What are the causes of Lennox-Gastaut syndrome?

-Hypoxic ischemic encephalopathy
-CNS malformation
-IEM

17

What can infantile spasms progress to?

Lennox-Gastaut syndrome

18

What are neonatal seizures?

Any seizures in the 1st month of life

19

Why are generalized seizures not possible in neonate?

newborns have incomplete myelination between the hemispheres

20

What are the characteristics of neonatal seizures?

Usually focal tonic, focal clonic, or myoclonic

21

What are the manifestations of neonatal seizures? (5)

-Repetitive mouth and tongue movements
-Apnea
-Pedalic movements
-Autonomic phenomena
-Tonic eye deviation

22

What are the 4 common causes of neonatal seizures?

-Hypoxic ischemic encephalopathy
-CNS infx
-Intracranial bleed
-Brain malformation

23

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

Hypocalcemia
Hyponatremia

24

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

True

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