pediatric neurology

Question 1

definition of epilepsy

Answer 1

occurrence of >2 unprovoked seizures

Question 2

abnormal hyper synchroncous discharge of cortical neurons

Answer 2

epilepsy

Question 3

name that seizure: staring episodes with cessation of activity

Answer 3

absence

Question 4

name that seizure: : brief jerking muscle movements

Answer 4

myoclonic

Question 5

name that seizure: loss of tone

Answer 5

atonic

Question 6

name that seizure: regularly repeated msucles jerking

Answer 6

clonic

Question 7

simple partial is _____alteration in consciousness vs complex partial is ____alteration in consciousness

Answer 7

NO alteration; any alteration

Question 8

staring off for less than 20 seconds withOUT loss of tone, or confusion post starting episode

Answer 8

childhood absence epilepsy

Question 9

what can absence epilepsy be provoked by?

Answer 9

hyperventilation

Question 10

EEG with generalized 3 Hz spike and slow wave discharge

Answer 10

absence seizure

Question 11

tx of choice for absence seizure

Answer 11

ethusoximide

Question 12

peak onset seizures 5-10 yrs, infrequent partial seizures, tingling in mouth, on face, drooling, dysphasia, speech arrest, unilateral UE movement

Answer 12

benign childhood epilepsy with centrotemporal spikes

Question 13

EEG: centrotemporal spikes

Answer 13

rolandic epilepsy

Question 14

usually have seizures that occur during sleep

Answer 14

rolandic epilepsy

Question 15

drug of choice for rolandic epilepsy

Answer 15

carbamazepine or oxcarbazapine

Question 16

EEG: 3-6 Hz polyspikes and wave

Answer 16

juvenile myoclonic epilepsy

Question 17

myoclonic jerks, age of onset 12-18, provoked by photic stimulation, AM wakening, lack of sleep

Answer 17

juvenile myoclonic epilepsy

Question 18

does juvenile myoclonic epilepsy resolve by end of adolescence years or persist?

Answer 18

persists, requires life long tx

Question 19

tx of juvenile myoclonic epilepsy

Answer 19

men: valproic acid
women: levetiracetam, lamotrigine

Question 20

fever + seizure w/o CNS infxn

Answer 20

febrile seizure

Question 21

febrile seizures occur in what ages

Answer 21

3 months–>6 yrs

Question 22

what is the most common seizure in children

Answer 22

febrile seizure

Question 23

simple febrile seizure is defined as generalized, less than ____min long, occurs___in a 24 hr period

Answer 23

15 min long; occurs 1x in 24 hrs

Question 24

tx to stop febrile seizure

Answer 24

benzo

Question 25

Small brief spasm, but one of the most severe epilepsy patterns

Answer 25

infantile spasm

Question 26

EEG: hypsarrhythmia

Answer 26

infantile spasm

Question 27

drug of choice for infantile spasm

Answer 27

ACTH

Question 28

drug of choice for infantile spasm secondary to tuberous sclerosis

Answer 28

vigabatrin

Question 29

cahracterized by different types of seizures, mental retardation

Answer 29

lennox gastaut syndrome

Question 30

EEG: less than 2.5 spike and slow wave discharges

Answer 30

lennox gastaut syndrome

Question 31

neonatal seizures are typically what type of seizure

Answer 31

focal tonic, focal clonic or myoclonic

Question 32

most common etiologies of neonatal seizures (4)

Answer 32

1. hypoxic ischemic encephalopathy 2. CNS infxn 3. intracranial bleeding 4. brain malformation

Question 33

best imaging for neonatal seizre

Answer 33

MRI

Question 34

preferred tx for neonatal seizure

Answer 34

phenobarbital

Question 35

immune mediated acute inflammatory demyelinating polyneuropathy

Answer 35

guillain barre syndrome

Question 36

the most common cause of acute flaccid paralysis in children

Answer 36

guillain barre syndrome

Question 37

campylobacter jejuni infxn, symmetri ascending paralysis

Answer 37

guillain barre syndrome

Question 38

symmetric legs weakness with diminished or absent DTR

Answer 38

guillain barre syndrome

Question 39

incr CSF protein, normal WBC with normal mental exam

Answer 39

guillain barre syndrome

Question 40

what is the most sensitive and specific test for demyelination

Answer 40

nerve conduction study

Question 41

tx of guillain barre syndrome

Answer 41

IVIG 2 g/kg or plasmapharesis

Question 42

calf hypertrophy, gower's sign, x linked recessive, slowly progressive

Answer 42

duchenne muscular dystrophy

Question 43

msot common causes of acute ataxia in children (3)

Answer 43

1. post infectious cerebellar ataxia 2. intoxication 3. guillain barre syndrome

Question 44

most common cause of acute ataxia in children

Answer 44

post infectious cerebellar ataxia

Question 45

rapid onset ataxia that is worst at presentation followed by gradual improvement

Answer 45

post infectious cerebellar ataxia

Question 46

work up of post infectious cerebellar ataxia

Answer 46

brain MRI

Question 47

static motor encephalopathy due to insult to developing brain in first 3 yrs of life

Answer 47

cerebral palsy

Question 48

what is peripheral hypertonia

Answer 48

spasticity and clonus

Question 49

what is axial hypotonia

Answer 49

head lag, truncal hypotonia

Question 50

classic cause of spastic hemiplegia in CP

Answer 50

unilateral perinatal stroke

Question 51

cause of spastic diplegia

Answer 51

premature infants with perventricular leukomalacia

Question 52

recurrent HA with N/V, photophobia

Answer 52

migraine

Question 53

most common type of migraine

Answer 53

migraine w/o aura

Question 54

main med for prevention of migraines in kids

Answer 54

cyproheptadine

Question 55

topiramate is good for tx of mirgrains in what sort of kids

Answer 55

obese, kids with epilepsy

Question 56

what is the main concern associated with tuberous sclerosis

Answer 56

incr risk of epilepsy

Question 57

cardiac rhabdomyoma

Answer 57

tuberous sclerosis

Question 58

non inherited disorder, port wine stain, leptomeningeal angiomas, seizures, cognitive delay, unilateral glaucoma

Answer 58

sturge weber syndrome