PEADS 3

Question 1

febrile seizure in who

Answer 1

18m commonest

Question 2

atypical febrile seizures

ix

Answer 2

> 15min
focal
multiple in 24 hours

EEG, brain CT/MRI

Question 3

treatment if continues seizure

Answer 3

rectal diazepam 1m-2y 5mg
2-12y 10mg
buccal midaz 0.3-0.5mg/kg

Question 4

causes of cerebral palsy antenatal
intrapartum
post natal

Answer 4

rubella, toxoplasmosis, CMV
birth asphyxia/truma
IVH, meningitis, head trauma

Question 5

spastic CP

Answer 5

70%
increased tone
increased reflexes
decreased power

Question 6

dyskintetic

Answer 6

involuntary movement

chorea

Question 7

ataxic

Answer 7

decreased tone
wide ataxic gat
nystagmus
intention tremor

Question 8

ix for CP

rx

Answer 8

MRI brain
MDT
spascticity baclofen, oral diaz, botox, ortho surgery

Question 9

amencephaly

Answer 9

brain does not develop

Question 10

encephalocele

Answer 10

extrusion of brain through midline skull defect

Question 11

myelomeningocele

Answer 11

abnormal cord and exposed defect

Question 12

meningocele

Answer 12

normal spinal cord. defect covered with skin

Question 13

occulta SB

Answer 13

cord covered with bone and skin with overlying skin lesion like lipoma/sinus/hair

Question 14

ix and rx for SB

Answer 14

MRI

early surgical closure

Question 15

communicating causes of hydrocephalus

noncomm

Answer 15

failure to reabsorb CSF postbleed/infection, arnold chairi, choroid plexus tumour - overproduction

obstruction to CSF flow - tumour, malformation

Question 16

symptoms of hydrocephalus
ix
rx

Answer 16

irritability, poor feeding, headaches, nom, seizures, big head, bulging fontanelle, sunsetting eyes, papilloedema

cranial USS/CT/MRI

ventriculoperitoneal shunt

Question 17

what is spinal muscular atrophy
genetics
type 1 is what

Answer 17

degeneration of anterior horn cells AR

type 1 is the worst

Question 18

symptoms of SMA
ix
prognosis

Answer 18

progressive proximal weakness, decreased fatal movements, frog leg posture, respiratory distress, bell shaped chest
EMG, genetic testing
death by 18 months in type 1

Question 19

tuberous sclerosis genetics
signs and ix
death by what

Answer 19

ADom but 80% sporadic mutation
development delay, infantile spasms, LD, behavioural difficulties, ash leaf spots

skull xr - snail track calcification

epilepsy

Question 20

ataxia telengectasia genetics 
symptoms
ix
cx
prognosis

Answer 20

AR dx of DNA repair affecting cerebellum in thymus

poor co ordination. telengectasia

increased AFP, EBC, chromosome fragility

infections, ALL

death in 20s due to immunodef

Question 21

friedecia ataxia genetics
symptoms
ix
rx

Answer 21

AR 1/800
sensory and cerebellar ataxia intention tremor
ECHO, EEG, NCS, gene studies
MDT

Question 22

muscular dystrophy is what 
which is more common 
symptoms 
ix
progosis

Answer 22

inherited x linked - prog muscle weakness
duchenne > becker
speech and motor delay, waddling gait, CM in backers, prox weakness
Gowers, increased CK, abnomarma EMG and NCS, muscle biopsy, DNA testing, MRI muscles

DMD death in early in 20s
BMB slower progression