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pediatrics > neuroped > Flashcards

neuroped Flashcards

1
Q

most severe form of NTD

A

menigocele(dysraphism)

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2
Q

when does the neural tube close spontaneously

A

bet 3rd and 4th wk AOG

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3
Q

underlying causes of NTD

A

radiation; drugs; malnutrition; genetic determinants

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4
Q

how to prevent myelomeningocele

A

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

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5
Q

location of majority of NTD

A

lumbosacral (75%)

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6
Q

manifestations of NTD

A

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

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7
Q

cause of anencephaly

A

failure of closure of the rostral neuropore

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8
Q

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

A

lissencephaly (agyria)

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9
Q

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

A

Schizencephaly

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10
Q

presence of cysts or cavities within the brain

A

porencephaly

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11
Q

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

A

holoprosencephaly

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12
Q

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

A

obstructive or noncommunicating type

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13
Q

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

A

nonobstructive or communicating type

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14
Q

site of CSF production

A

choroid plexus epithelium within the cerebral ventricles

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15
Q

how is csf reabsorbed

A

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

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16
Q

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

A

setting-sun sign

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17
Q

separation of sutures

A

macewen sign

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18
Q

foreshortened occiput

A

chiari malformation

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19
Q

prominent occiput

A

dandy-walker malformation

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20
Q

major cause of complications of shunting in hydrocephalus

A

Staph epidermidis infxn

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21
Q

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

A

epilepsy

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22
Q

consciousness retained or not in simple partial seizure

A

retained

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23
Q

consciousness retained or not in complex partial seizure

A

impaired

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24
Q

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

A

absence seizure

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25
Q

definition of absence seizure

A

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

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26
Q

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

A

generalized tonic-clonic

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27
Q

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

A

myoclonic seizures

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28
Q

Tx for GTC seizure

A

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

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29
Q

Tx for absence seizure

A

Ethosuximide; Valproic acid

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30
Q

Tx for infantile spasms

A

Vigabatrin

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31
Q

purpose of ketogenic diet in seizures

A

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

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32
Q

most common seizure disorder in childhood

A

febrile seizure

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33
Q

characteristics of febrile seizure

A

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

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34
Q

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

A

complex febrile seizure

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35
Q

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

A

status epilepticus

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36
Q

usual cause of status epilepticus

A

breakthrough seizures; missed doses of anti epileptic drug

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37
Q

DOC for status epilepticus

A

diazepam and phenytoin

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38
Q

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

A

migraine

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39
Q

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

A

migraine without aura

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40
Q

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

A

migraine with aura

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41
Q

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

A

hemiplegic aura

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42
Q

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

A

basilar-type migraine

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43
Q

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

A

abdominal migraine

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44
Q

persistent headache lasting >3days

A

status migrainosus

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45
Q

Tx for status migrainosus

A

Prochorperazine IV 0.15 mkd max 10mg

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46
Q

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

A

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

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47
Q

TSC1 encodes for what protein

A

hamartin

48
Q

TSC2 encodes for what protein

A

tuberin

49
Q

candle-dripping appearance in the subependymal region

A

tuberous sclerosis

50
Q

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

A

tuberous sclerosis

51
Q

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

A

Shagreen patch

52
Q

manifestations of tuberous sclerosis

A

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

53
Q

Management of Tuberous Sclerosis

A

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

54
Q

most prevalent type of Neurofibromatosis

A

NF1

55
Q

Dx of NF1

A

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
Q

Dx of NF2

A

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
Q

hamartomoas located within the iris

A

Lisch nodules

58
Q

Management of NF2

A

genetic counseling and early detection of treatable conditions or complications

59
Q

chromosomal location of NF gene

A

Chromosome 17

60
Q

aka von Recklinghausen dse

A

Neurofibromatosis

61
Q

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

A

GBS; gm(-) enteric bacilli; Listeria monocytogenes

62
Q

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

A

Strep pneumoniae; H. influenzae; N. meningitidis

63
Q

mode of transmission of bacterial meningitis

A

hematogenous from a distant site of infection

64
Q

why is there neck rigidity in meningitis

A

inflammation of spinal nerves and roots produce meningeal signs of irritation

65
Q

complications of meningitis

A

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

66
Q

contraindications to LP

A

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

67
Q

Tx for meningitis due to N. meningitidis

A

IV Penicillin for 5-7 days

68
Q

Tx for meningitis due to S. pneumoniae

A

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

69
Q

rationale of giving dexamethasone IV in HiB meningitis

A

less fever; lower CSF protein; reduced auditory damage

70
Q

CSF analysis in viral meningitis

A

normal glucose; normal or slightly eleveated protein; lymphocytosis

71
Q

where is cerebral abscess commonly located

A

cerebrum (80%); majority are single

72
Q

etiology of cerebral absces

A

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
Q

Causative agent in brain abscess

A

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

74
Q

Dx of cerebral abscess

A

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

75
Q

management of cerebral abscess

A

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

76
Q

Management of cerebral abscess due to head trauma or neurosurgery

A

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

77
Q

Management of cerebral abscess due to CHD

A

Penicillin + Metronidazole

78
Q

Management of cerebral abscess due to infected VP shunt

A

Vancomycin + Ceftazidime

79
Q

Management of cerebral abscess due in immunocompromised px

A

broad spectrum + Amphotericin B

80
Q

Indications for sugery in cerebral abscess

A

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

81
Q

duration of antibiotics in the Tx of cerebral abscess

A

4-6wks

82
Q

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

A

Myasthenia Gravis

83
Q

earliest and most constant signs in Myasthenia Gravis

A

ptosis and some degree of EOM weakness

84
Q

manifestations in MG

A

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
Q

characteristic feature of MG

A

fatigue of muscles

86
Q

what is more affected muscles in MG

A

proximal rather than distal muscle weakness

87
Q

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

A

Tensilon test

88
Q

Dx of MG

A

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

89
Q

Tx of MG

A

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
Q

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

A

Bell palsy

91
Q

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

A

Guillain-Barre syndrome aka demyelinating polyradiculoneuropathy

92
Q

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

A

Miller-Fisher syndrome

93
Q

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

A

Landry ascending paralysis

94
Q

clinical features predictive of poor outcoime with sequela

A

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

95
Q

last function to recover in GBS

A

tendon reflexes and lower extremity weakness

96
Q

Dx of GBS

A

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

97
Q

Management of GBS

A

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
Q

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

A

Cerebral Palsy

99
Q

clinical manifestations of spastic hemiplegia in CP

A

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
Q

clinifcal manifestations in spastic diplega

A

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

101
Q

clinical manifestations in spastic quadriplegia

A

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

102
Q

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

A

brain tumors

103
Q

2 distinct patterns of presentation of brain tumors

A

symptoms and signs of increased ICP and focal neurologic signs

104
Q

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

A

head tilting and nuchal rigidity

105
Q

prominent signs in posterior fossa tumors

A

nystagmus and ataxia

106
Q

more common location of brain tumors

A

infratentorial

107
Q

age at which 2/3 of brain tumors occur

A

2-12yo

108
Q

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

A

Medulloblastoma

109
Q

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

A

Homer-Wright rosettes

110
Q

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

A

cerebellar astrocytoma

111
Q

Tx of Medulloblastoma

A

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

112
Q

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

A

craniopharyngioma

113
Q

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

A

optic nerve glioma

114
Q

diagnostic imaging use to delineate brain tumors

A

cranial MRI

115
Q

term used for implantation of radiation seeds for brain tumors

A

brachytherapy

pediatrics (16 decks)

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