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Flashcards in Peds III Deck (60)
1

What is cerebral palsy?

Static motor encephalopathy due to an insult to the developing brain in the first 3 years of life

2

True or false: kids with cerebral palsy usually have other neurologic problems, like seizures, developmental delay, cognitive delay, etc.

True

3

What is the usual presentation of cerebral palsy?

Peripheral hypertonia and axial hypotonia

4

What are the four classifications of cerebral palsy?

-Spastic hemiplegia
-Spastic diplegia
-Spastic quadriplegia
-Dyskinetic CP

5

What is the spastic hemiplegia form of cerebral palsy? Which limbs are usually involved? What causes it?

One side of the body is affected, and usually the arm more than the leg.

Cause by a unilateral perinatal stroke

6

What is the spastic diplegic form of cerebral palsy? What causes it?

Both legs are only affected. Usually seen in premature infants with periventricular leukomalacia

7

What is the spastic quadriplegia form of cerebral palsy?

All extremities are affected

8

What is the dyskinetic form of cerebral palsy? What causes it?

Athetoid
Choreo Athetoid
Dystonic

9

What are the skin problems that can occur with CP kids?

Skin ulcers

10

What are the GI problems that can occur with CP kids?

Constipation
Poor feeding
GERD
FTT

11

What are the orthopedic problems that can occur with CP kids?

Hip dislocation
Scoliosis

12

What are the respiratory problems that can occur with CP kids?

Aspiration pneumonia
Chronic lung disease

13

What are the neurological problems that can occur with CP kids?

Epilepsy
Vision and hearing deficits
-sleep disturbances
-Cognitive delay

14

What is the treatment for CP kids?

Individualized multidisciplinary team

15

What is the incidence of migraines in kids?

5%

16

What gender is usually affected more with migraines in childhood/adolescence?

Females

17

What are the characteristics of migraines?

Recurrent headaches accompanied by n/v and photophobia

18

What aggravates and alleviates the HA with migraines?

-Physical activity worsens
-Sleep cures it

19

Are migraines usually bilateral or unilateral in children?

Bilateral, but progresses unilaterally as they grow older

20

What is the most common type of migraine in children?

Migraine without aura

21

What are the complicated type of migraines? (4)

-Hemiplegic migraine
-Basilar migraine (vertigo)
-Ophthalmoplegic migraine (oculomotor palsy)
-Confusional migraine

22

What should always be obtained with complicated migraines in children?

MRI

23

What is the treatment for migraines in children?

OTC NSAIDs, or triptans if severe

24

What is the antiemetic medication of choice for migraines in children?

Zofran (ondansetron)

25

What is the basilar form of migraine?

Migraine accompanied by vertigo,diplopia, nystagmus and/or dysarthria

26

What is the prophylactic treatment for migraines? (lifestyle + drug)

-Exercise
-Cyproheptadine (antihistamine)

27

What are the side effects of cyproheptadine? (2)

Increased appetite and somnolence

28

Who usually gets topiramate for their migraines?

Obese children and children with epilepsy

29

What are the side effects of topiramate? (3)

-Weight loss
-Renal stones
-Cognitive impairment

30

What are the side effects of amitriptyline?

-Sleepiness
-Cardiac arrhythmia

31

What should be started prior to prescribing amitriptyline for migraine headaches? Why?

EKG
Can cause QT prolongation

32

What is the role of propranolol in treating migraines in kids?

Contraindicated in asthma

33

What are neurocutaneous syndromes?

Group of neurological disorders with skin lesions

34

What are the three most common neurocutaneous syndromes?

-NF
-Tuberous sclerosis
-Sturge Weber syndrome

35

What is the inheritance pattern and cause of Neurofibromatosis?

-AD
-Defect in neurofibromin protein, which normally negatively regulates Ras oncogene signal transduction

36

What are the 7 diagnostic criteria for NF1?

1. 6+ cafe au lait spots
2. axillary or inguinal freckling
3. 2 neurofibromas or 1 complex neurofibroma
4. Optic glioma
5. 2 lisch nodules
6. Sphenoid dysplasia
7. first degree relative with NF1

37

How many cafe au lait spots are needed to diagnose NF1? how big should they be pre and post puberty?

6 or more

More than 5 mm pre
more than 15 mm post

38

How many axillary/inguinal freckling are needed to diagnose NF1?

2 or more

39

How many neurofibromas are needed to diagnose NF1?

2 neurofibromas or 1 complex neurofibroma

40

What is the optic finding that is needed to diagnose NF1?

Optic glioma

41

How many lisch nodules are needed to diagnose NF1?

2

42

What are the bony abnormalities that are diagnostic for NF1? (2)

-Sphenoid dysplasia
-Long bone abnormalities

43

How many first degree relatives are needed to diagnose NF1?

1 first degree

44

How many criteria are needed (of the 7) to diagnose NF1?

2 of the 7

45

What is the inheritance pattern of tuberous sclerosis?

AD, but most patients have de novo mutations

46

What is the genetic defect in tuberous sclerosis? What renal pathology can this cause?

Angiomyolipomas

TSC is caused by a mutation of either of two genes, TSC1 and TSC2, which code for the proteins hamartin and tuberin, respectively. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation

47

What are the skin manifestations of tuberous sclerosis? (2)

-Hypomelanotic macules (ash leaf)
-facial angiofibromas (like facial acne)

48

What are the CNS findings of tuberous sclerosis? (3)

-Subependymal nodules
-Cortical tubers
-Giant cell astrocytomas

49

What are the eye findings of tuberous sclerosis?

Retinal hamartomas

50

What are the heart findings of tuberous sclerosis?

Cardiac rhabdomyomas

51

What are the lungs findings of tuberous sclerosis? When does this usually present?

Lymphangioleiomyomatosis
Presents in adulthood

52

What is Lymphangioleiomyomatosis (LAM)?

A rare, progressive, systemic disease that typically results in cystic lung destruction and predominantly affects women, especially during childbearing years

53

What is the prognosis for kids with cardiac rhabdomyomas 2/2 tuberous sclerosis?

Resolves spontaneously

54

What are the cognitive outcomes of tuberous sclerosis?

Depends on the severity of the seizure disorder--infantile spasms may be associated with worse cognitive prognosis

55

What is the inheritance pattern of sturge-Weber syndrome?

Not inherited

56

What is sturge weber syndrome?

Non-inherited disorder characterized by development of angiomas in the brain, skin, and eyes

57

What is the usual presentation of sturge-weber syndrome? What age?

Port wine stain affecting V1 and V2 unilaterally

Usually obvious at birth

58

What are the CNS findings of sturge-weber syndrome? (4)

-Leptomeningeal angiomas
-Seizures
-hemiparesis
-Cognitive delay of various degrees

59

What are the eye manifestations of Sturge-Weber syndrome?

Unilateral glaucoma

60

What are the two causes of seizures in tuberous sclerosis?

Cortical tubers or giant cell astrocytomas