Paeds Neuro

Question 1

Characterise an infantile spasm

Answer 1

Fast, tonic contraction of the trunk and limb muscles with gradual relaxation over 1-2 seconds

Question 2

Describe seizure seen in juvenile myoclonus epilepsy

Answer 2

Brief, bilateral arrhythmic irks typically of upper limb occurring 1-2 hours after waking

Question 3

Do you use carbamezapine to treat juvenile myoclonic epilepsy?

Answer 3

NO! It makes it worse

Question 4

What type of seizure is usually a/w aura

Answer 4

Temporal lobe

Question 5

What eeg finding do you see in absence seizures?

Answer 5

3 second spike-and-waves

Question 6

What are common auras for temporal lobe epilepsy ?

Answer 6

Nausea/ abdo pain/ dejavu/ sense of impending doom.

Question 7

What eeg finding is consistent with benign Rolandic epilepsy?

Answer 7

Centrotemporal spikes

Question 8

What type of epilepsy resolves by adulthood?

Answer 8

Benign Rolandic epilepsy

Question 9

Weight gain, hair loss and gingival hypertrophy a/w what drug?

Answer 9

Sodium valproate

Question 10

What anti-epileptic puts you at risk of stevens-Johnson syndrome? J

Answer 10

Carbamazepine

Question 11

First line tested for generalised tonic-clonic seizures?

Answer 11

Sodium valproate

Question 12

When isn’t sodium valproate first line for tonic-clonic seizures ?

Answer 12

Girls of childbearing potential

Question 13

What is first line treatment for absence seizure (typical or atypical)

Answer 13

Ethosuximide

Question 14

What is second line treatment for tonic or atonic seizures ?

Answer 14

Lamotrigine

Question 15

What anti epileptic can impair effectiveness of oral contraceptives?

Answer 15

Topiramate

Question 16

Define atonic seizure

Answer 16

Partial or complete limp-ness of body. Typically lasts <15 seconds, usually remain conscious

Question 17

What epilepsy syndromes have myoclonus seizures?

Answer 17

Juvenile myoclonic epilepsy
Lennox-Gastaut syndrome
Progressive myoclonic epilepsy

Question 18

What MRI findings are highly suggestive of acute disseminated encephalomyelitis ?

Answer 18

Multi focal diffuse white matter changes

Question 19

What is inheritance of friedreich’s ataxia ?

Answer 19

Autosomal- recessive

Question 20

What non-neurological finding is associated with friedreich’s ataxia?

Answer 20

Hypertrophic cardiomyopathy

Question 21

Progressive Cerebellar symptoms, absent deep tendon reflexes, weakness of distal musculature and preserved intelligence suggests what ?

Answer 21

Friedreich’s ataxia

Question 22

What is characteristic of Landau-kleffner syndrome ?

Answer 22

Acquired aphasia, in previously normal child. + self limiting tonic-clonic seizure

Question 23

How do benign Rolandic epilepsy cases present?

Answer 23

Frequent, short lived, nocturnal seizures with complete recovery afterwards.

Question 24

Big head, hands and feet is what syndrome?

Answer 24

Sotos syndrome

Question 25

Describe infantile spasm

Answer 25

Runs of flexion and extension spasm, with peculiar cry and screaming

Question 26

Normal development until 6-18 months then gradual regression in girls

Answer 26

Rett syndrome

Question 27

Focal seizures occurring during sleep suggests what?

Answer 27

Frontal epilepsy

Question 28

What is first line treatment for West Syndrome?

Answer 28

Vigabatrin

Question 29

What anti-epileptic may cause peripheral visual field loss?

Answer 29

Vigabatrin

Question 30

What is threshold for concern regarding weight centiles?

Answer 30

If on <9th centile then dropping one centile space is bad If 9-91 st centile then a fall across two weight centiles If >91 then fall across three weight centiles

Question 31

What is first line treatment for anorexia nervosa?

Answer 31

Focused family therapy

Question 32

How do proton pump inhibitors work?

Answer 32

Block H+/K+ ATP system in the gastric parietal cells

Question 33

How should a mother who is classed high risk HIV be treated re birth?

Answer 33

IV maternal zidovudine, lower segment CS, baby to have combo antiretroviral therapy

Question 34

Describe sturge-Weber syndrome

Answer 34

Pot wine stain forehead and upper eyelid. Seizures resistant to anticonvulsants. Hemiplagia on controlateral side of port wine stain

Question 35

What is commonest cause of congenital adrenal hypoplasia

Answer 35

21-hydroxy enzyme def

Question 36

What type of cells in Hodgkin’s lymphoma

Answer 36

Reed Sternberg cells

Question 37

Describe Lennox-Gestraut syndrome

Answer 37

Intractable seizures, sever. Tonic and atonic. Development stagnation and regression in-between. Sodium valproate first line