Neuro

Question 1

signs of achondrodysplasia in neonates

Answer 1

arrest of femur development at 28 weeks gestation
macrocephaly secondary to foramen magnum stenosis

Question 2

how to distinguish GBS and tranvserve myelitis

Answer 2

GBS is LMN signs
transverse myelitis is UMN signs

Question 3

seizure type and EEG of benign rolandic epilepsy

Answer 3

facial twitching
centrotemporal spikes

Question 4

vomiting before seizure =

Answer 4

panayiotopoulos syndrome

Question 5

calcification pattern in CMV and toxoplasmosis

Answer 5

CMV = periventricular
toxoplasmosis = diffuse

Question 6

features of Sotos syndrome

Answer 6

macrocephaly
large hands and feet
interlectual impairment

Question 7

Features of glutaric aciduria type 1

Answer 7

encephalopathy
subdural effusions

Question 8

features of Landau Kleffner syndrome

Answer 8

normal –> aphasia –> epilepsy

Question 9

features of angelmans syndrome

Answer 9

cognitive impairment
happy
flappy hands
jerky movements
microcephaly
blonde hair

Question 10

angelmans syndrome numbers

Answer 10

15q11.2-q13

Question 11

location of acute viral encephalitis and acute disseminated encephalomyelitis

Answer 11

AVE = grey
ADEM = white

Question 12

friedrich’s ataxia inheritance

Answer 12

autosomal recessive
GAA repeat on FXN gene

Question 13

Friedrich’s ataxia presentation

Answer 13

progressive ataxia
absent reflexes
dysarthria
kyphoscoliosis
HOCM/CCF
DM
optic atrophy

Question 14

meds for tonic clonic

Answer 14

valproate/lamotrigine

Question 15

meds for absence

Answer 15

ethosuximide/valproate

Question 16

meds for myoclonic

Answer 16

valproate/keppra

Question 17

tonic/atonic

Answer 17

valproate/lamotrigine

Question 18

presentation of Lennox Gastaut

Answer 18

frequent status epilepticus

Question 19

Rx neonatal seizures

Answer 19

phenobarbitol

Question 20

Rett syndrome course

Answer 20

normal development until 6-18 months the regression

Question 21

sun setting sign

Answer 21

hydrocephalus

Question 22

communicating hydrocephalus

Answer 22

meningitis

Question 23

non communicating hydrocephalus

Answer 23

aquaduct stenosis
atresia of the outflow of the forth ventricle
chiari malformation
fossa neoplasm or malformation

Question 24

main complication of VP shunt

Answer 24

Obstruction
Staph infection

Question 25

Sturge Weber eye manifestation

Answer 25

glaucoma

Question 26

Ix in all children with seizures

Answer 26

ECG to r/o long QT

Question 27

Seizures in sleep

Answer 27

Benign rolandic or Lennox Gastaut

Question 28

Ix in Bells Palsy

Answer 28

Bp - association with CoA and renal failure

Question 29

Charcot Marie Tooth - inheritence - biopsy - signs

Answer 29

autosomal dominant onion bulb bilateral foot drop

Question 30

meningiocele vs myelomeningocele

Answer 30

meningiocele = meninges bulge, skin covers myelomeningiocele = meninges and cord protrudes, often no skin

Question 31

hip in myelomeningiocele

Answer 31

dislocation due to low tone

Question 32

which lesions cause a facial nerve palsy

Answer 32

pontine

Question 33

night terrors with posturing?

Answer 33

get EEG for ?frontal lobe epilepsy

Question 34

management of night terrors

Answer 34

sleep hygiene

Question 35

which bug is associated with hearing loss in meningitis

Answer 35

HiB

Question 36

how to reduce hearing loss in meningitis

Answer 36

early dex

Question 37

What to do for a first presentation of infantile spasm

Answer 37

paediatric neurologist within 24hrs

Question 38

management of infantile spasms

Answer 38

vigabatrin and prednisolone (unless has tuberus sclerosis) weekly review and urine glucose and bp repeat sleep EEG at 2 weeks other AED or ketogenic diet is second line

Question 39

meningitis with acute hydrocephalus

Answer 39

TB

Question 40

presentation of SSPE

Answer 40

behaviour change jerky movements aphasia dementia

Question 41

Ix for SSPE

Answer 41

EEG Elevated CSF: serum specific IgG

Question 42

4th nerve palsy -nerve name - muscle name - action - presentation of palsy

Answer 42

trochlear superior oblique depresses eye (used for walking down stairs) elevated and lateral position in palsy