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Flashcards in neuroped Deck (115):
1

most severe form of NTD

menigocele(dysraphism)

2

when does the neural tube close spontaneously

bet 3rd and 4th wk AOG

3

underlying causes of NTD

radiation; drugs; malnutrition; genetic determinants

4

how to prevent myelomeningocele

maternal periconceptual use of folic acid reduces its incidence by 50%(started before conception until at least 12wks AOG); 0.4mg/day

5

location of majority of NTD

lumbosacral (75%)

6

manifestations of NTD

flaccid paralysis of LE; absent DTR's; lack of response to pain and touch; hip subluxation; clubfeet; bowel and bladder incontinence; associated with type II chiari hydrocephalus

7

cause of anencephaly

failure of closure of the rostral neuropore

8

absent cerebral convolutions; poorly developed sylvian fissure; failure to thrive; mircrocephaly; developmental delay; seizures; faulty neuroblast migration

lissencephaly (agyria)

9

uni or bilateral clefts within the cerebral hemispheres; severe MR; seizures; microcephaly; spastic quadriparesis

Schizencephaly

10

presence of cysts or cavities within the brain

porencephaly

11

due to defective cleavage of prosencephalon and inadequate induction of the forebrain structures; evidence of noncleaved midline brain structures

holoprosencephaly

12

type of hydrocephalus where obstruction is within the ventricular system; abnormality of the aqueduct or a lesion in the 4th ventricle

obstructive or noncommunicating type

13

type of hydrocephalus where there is obliteration of the subarachnoid cisterns and malfunction of the arachnoid villi; follows a subarachnoid hemorrhage; leukemic infiltrates

nonobstructive or communicating type

14

site of CSF production

choroid plexus epithelium within the cerebral ventricles

15

how is csf reabsorbed

arachnoid villus cells; which are located in the superior sagittal sinus return CSF to the blood stream within the vacuoles(pinocytosis)

16

eyes may deviate downward due to impingement of the dilated suprapineal recess on the tectum

setting-sun sign

17

separation of sutures

macewen sign

18

foreshortened occiput

chiari malformation

19

prominent occiput

dandy-walker malformation

20

major cause of complications of shunting in hydrocephalus

Staph epidermidis infxn

21

present when 2 or more unprovoke seizures occur at an interval greater than 24hrs apart

epilepsy

22

consciousness retained or not in simple partial seizure

retained

23

consciousness retained or not in complex partial seizure

impaired

24

type of seizure where there is sudden cessation of motor activity or speech with a blank facial expression and flickering of the eyelids

absence seizure

25

definition of absence seizure

never associated with an aura; rare in <5yo; not associated with postictal state; rarely persist longer than 30s; no loss of body tone

26

seizure associated with an aura; sudden loss of consciousness and patient may become cyanotic and apneic; there is loss of sphincteric control

generalized tonic-clonic

27

repititive seizures consisting of brief often symmetric muscular contraction with loss of body tone and falling or slumping forward

myoclonic seizures

28

Tx for GTC seizure

PPCaLaVaG; Phenobarbital; Phenytoin; Carbamazepine; LAMOTRIGINE; Valproic Acid; Gabapentin

29

Tx for absence seizure

Ethosuximide; Valproic acid

30

Tx for infantile spasms

Vigabatrin

31

purpose of ketogenic diet in seizures

anticonvulsant effect due to increased beta hydroxybutyrate and acetoacetate resulting from ketosis

32

most common seizure disorder in childhood

febrile seizure

33

characteristics of febrile seizure

rare before 9mos and after 5yrs old; peaks at 14-18mos; normal EEG; (+) family history

34

febrile seizure with duration >15mins; repeated convulsions occur within 24hrs; focal seizure activity

complex febrile seizure

35

one seizure lasting 30mins or multiple seizures during 30mins without regaining consciousness

status epilepticus

36

usual cause of status epilepticus

breakthrough seizures; missed doses of anti epileptic drug

37

DOC for status epilepticus

diazepam and phenytoin

38

recurrent headache with symptom-free intervals and at least 3 of the following 1Family history 2Relief ff sleep 3Unilateral location 4Associated aura 5Abdominal pain 6Nausea and Vomiting 7Throbbing in character

migraine

39

most prevalent type of migraine; throbing or pounding; bifrontal or temporal areas; intense nausea and vomiting; (+) family hx in 90% usually maternal

migraine without aura

40

migraine with sensory symptoms; fortification spectra (brillian white zigzag lines); Alice in Wonderland syndrome

migraine with aura

41

migraine that has unilateral sensory or motor signs that may persist for days; good prognosis in older child or adolescent

hemiplegic aura

42

migraine associated with vasoconstriction of basilar and posterior cerebral arteries; girl<4yo at risk

basilar-type migraine

43

mid-abdominal pain with pain-free periods between attacks; at least 2 of the ff symptoms: anorexia; nausea; vomiting; pallor; pain may persist from 1-72hrs

abdominal migraine

44

persistent headache lasting >3days

status migrainosus

45

Tx for status migrainosus

Prochorperazine IV 0.15 mkd max 10mg

46

Prophylactic treatment for migraine (more than 2-4 severe episodes monthly)

propranolol 10-20mg TID for >7yo; Flunarizine 5mg at bedtime

47

TSC1 encodes for what protein

hamartin

48

TSC2 encodes for what protein

tuberin

49

candle-dripping appearance in the subependymal region

tuberous sclerosis

50

heterogeneous dse with wide clinical spectrum varying from severe MR and incapacitating seizures to normal intelligence and a lack of seizures; often within the same family

tuberous sclerosis

51

roughened; raised lesion with an orange-peel consistency primarily in the lumbosacral region in tuberous sclerosis

Shagreen patch

52

manifestations of tuberous sclerosis

infantile spasms; hypopigmented ASH leaf lesions; calcified tubers in the periventricular areas; subungual/periungual fibroma;mulberry tumors; rhabdomyosarcoma of the heart; bilateral angiomyolipomas

53

Management of Tuberous Sclerosis

seizure control and baseline 2D echo; CXR; renal UTZ

54

most prevalent type of Neurofibromatosis

NF1

55

Dx of NF1

2 of the ff: 1. 6 or more cafe au lait macules>5mm in prepubertals and >15mm in postpubertal individuals with sparing of the face; 2. 2 or more Lisch nodules; 3. 2 or more neurofibromas or one plexiform neurofibroma; 4. distinctive osseus lesion; 5. Optic glioma; 6. 1st degree relative with NF1; 7. high incidence of learning disabilities; 8. majority occurs in the paternal germline

56

Dx of NF2

1 of the ff: 1. bilateral acoustic neuroma; 2. parent; sibling; or child with NF2 and either unilateral acoustic neuroma or any 2 of the ff: 1. neurofibroma; 2. meningioma; 3.glioma; 4. schwannoma

57

hamartomoas located within the iris

Lisch nodules

58

Management of NF2

genetic counseling and early detection of treatable conditions or complications

59

chromosomal location of NF gene

Chromosome 17

60

aka von Recklinghausen dse

Neurofibromatosis

61

most common cause of bacterial meningitis in 1st 2mos of life

GBS; gm(-) enteric bacilli; Listeria monocytogenes

62

most common cause of bacterial meningitis in 2-12mos of life

Strep pneumoniae; H. influenzae; N. meningitidis

63

mode of transmission of bacterial meningitis

hematogenous from a distant site of infection

64

why is there neck rigidity in meningitis

inflammation of spinal nerves and roots produce meningeal signs of irritation

65

complications of meningitis

hydrocephalus; subdural effusions; SIADH may exacerbate edema leading to hyponatremic seizures

66

contraindications to LP

evidence of increased ICP; severe cardiopulmonary compromise; infection of the skin overlying the site

67

Tx for meningitis due to N. meningitidis

IV Penicillin for 5-7 days

68

Tx for meningitis due to S. pneumoniae

3rd gen Cephalosporin or IV Penicillin for 10-14 days

69

rationale of giving dexamethasone IV in HiB meningitis

less fever; lower CSF protein; reduced auditory damage

70

CSF analysis in viral meningitis

normal glucose; normal or slightly eleveated protein; lymphocytosis

71

where is cerebral abscess commonly located

cerebrum (80%); majority are single

72

etiology of cerebral absces

embolization due to RL shunt; meningitis; chronic OM or mastoiditis; face and scalp infections; orbital cellulitis; dental infections; penetrating head injuries; VP shunt infections

73

Causative agent in brain abscess

S.aureus; Strep; anaerobes; gm(-) aerobic bacilli(Proteus; Pseudomonas; Haemophilus; Citrobacter)

74

Dx of cerebral abscess

(+) blood culture in 10%; CSF not done due to herniation; cranial CT and MRI (most reliable methodS)

75

management of cerebral abscess

empiric antibiotics depend on the probable pathogens; unknown cause-3rd gen Cephalosporin + Metronidazole

76

Management of cerebral abscess due to head trauma or neurosurgery

Nafcillin or Vancomycin with 3rd gen Cep + Metronidazole or Meropenem as monotherapy

77

Management of cerebral abscess due to CHD

Penicillin + Metronidazole

78

Management of cerebral abscess due to infected VP shunt

Vancomycin + Ceftazidime

79

Management of cerebral abscess due in immunocompromised px

broad spectrum + Amphotericin B

80

Indications for sugery in cerebral abscess

1(+) gas in the abscess; 2Multiloculated abscess; 3Posterior fossa location; 4Fungal Cause; 5Associated infections like mastoiditis; periorbital abscess; sinusitis

81

duration of antibiotics in the Tx of cerebral abscess

4-6wks

82

dse characterized by immune-mediated neuromuscular blockade whre postsynaptic muscle membrane or motor endplate is less responsive than normal in releasing Ach

Myasthenia Gravis

83

earliest and most constant signs in Myasthenia Gravis

ptosis and some degree of EOM weakness

84

manifestations in MG

dysphagia and facial weakness; poor head control due to weak neck flexors; weakness involves limb-girdle and distal muscle of the hands; (fasciculations; myalgias and sensory symptoms do not occur); diminished tendon stretch reflexes

85

characteristic feature of MG

fatigue of muscles

86

what is more affected muscles in MG

proximal rather than distal muscle weakness

87

test where ptosis and ophtalmoplegia improve within a few seconds after administration of Edrophonium chloride IV in MG

Tensilon test

88

Dx of MG

EMG is more spefically diagnostic thatn muscle biopsy (decremental response is seen as a result of repititive nerve stimulation

89

Tx of MG

Neostigmine 0.4mg/kg IM q4-6hrs; Atropine to treat cholinergic crisis; Prednisone; Thymectomy when there is high titers of anti-Ach receptors and who are symptomatic for <2yrs; plasmapharesis to steroid nonresponders

90

acute unilateral facial nerve palsy not associated with other cranial neuropathies; usually develops abruptly about 2wks after a systemic viral infxn

Bell palsy

91

disease characterized by an autoimmune exn that develops in response to a previous infection that leads to aberrant demyelination of peripheral nerves and ventral motor nerve roots

Guillain-Barre syndrome aka demyelinating polyradiculoneuropathy

92

syndrome associated with Guillain-Barre that presents as acute opthalmoplegia; ataxia; and areflexia

Miller-Fisher syndrome

93

type of paralysis characterized by weakening beginning in the lower extremities and progressively involves the trunk; upper limbs; and bulbar muscles seen in Guillain-Barre

Landry ascending paralysis

94

clinical features predictive of poor outcoime with sequela

CN involvement; need for intubation; maximum disability at time of presentation

95

last function to recover in GBS

tendon reflexes and lower extremity weakness

96

Dx of GBS

albuminocytologic dissociation(high CSF protein with lack of cellular response); reduced motor NCV

97

Management of GBS

admit for observation bec ascending paralysis may occur within 24hrs; if rapidly progressive ascending paralysis: IVIG for 5 consecutive days; high dose pulse methylprednisolone IV for relapses

98

noprogressive disorder of posture and mov't often associated with epilepsy and abnormalities of speech; vision; and intellect resulting from a defect or lesion of the developing brain

Cerebral Palsy

99

clinical manifestations of spastic hemiplegia in CP

arms>legs; difficulty in hand manipulation obvious by 1yo; delayed wlaking or walks on tiptoes; spasticity apparent specially in the ankles; seizures and cognitive impairment

100

clinifcal manifestations in spastic diplega

bilateral spasticity of the leg; commando crawl; increased DTR's +Babinski; normal intellect

101

clinical manifestations in spastic quadriplegia

most severe form of CP due to marked motor impairment of all extremities and high association with MR and seizures; swallowing difficulties

102

2nd most commmon malignancy in childhood; most common solid tumors in childhood

brain tumors

103

2 distinct patterns of presentation of brain tumors

symptoms and signs of increased ICP and focal neurologic signs

104

signs in px with brain tumors that may indicate herniation of cerebellar tonsils

head tilting and nuchal rigidity

105

prominent signs in posterior fossa tumors

nystagmus and ataxia

106

more common location of brain tumors

infratentorial

107

age at which 2/3 of brain tumors occur

2-12yo

108

malignant brain tumor most often found in the cerebellum; M>F; heterogeneous enhancements in the cerebellum often invading the 4th ventricle and can cause obstructive hydrocephalus

Medulloblastoma

109

circular patterns of tumor cells surrounding a center of neutrophils found in Medulloblastoma

Homer-Wright rosettes

110

most common primary brain tumor; best prognosis; causes hydrocephalus; resection with 90% 5year survival rate

cerebellar astrocytoma

111

Tx of Medulloblastoma

surgery + irradiation; 80-90% 5year survival rate

112

brain tumor that is characterized by solid and cystic areas that tend to calcify; short stature; pressure to optic chiasm may cause bitemporal hemianopsia

craniopharyngioma

113

brain tumor that causes decreased visual acuity and pallor of the discs; 25% have NF; hyperalert and euphoric despite being emaciated; invasion of the hypothalamus leads to obesity or DI

optic nerve glioma

114

diagnostic imaging use to delineate brain tumors

cranial MRI

115

term used for implantation of radiation seeds for brain tumors

brachytherapy