Paeds Neuro Flashcards

1
Q

Characterise an infantile spasm

A

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

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2
Q

Describe seizure seen in juvenile myoclonus epilepsy

A

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

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3
Q

Do you use carbamezapine to treat juvenile myoclonic epilepsy?

A

NO! It makes it worse

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4
Q

What type of seizure is usually a/w aura

A

Temporal lobe

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5
Q

What eeg finding do you see in absence seizures?

A

3 second spike-and-waves

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6
Q

What are common auras for temporal lobe epilepsy ?

A

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

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7
Q

What eeg finding is consistent with benign Rolandic epilepsy?

A

Centrotemporal spikes

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8
Q

What type of epilepsy resolves by adulthood?

A

Benign Rolandic epilepsy

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9
Q

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

A

Sodium valproate

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10
Q

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

A

Carbamazepine

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11
Q

First line tested for generalised tonic-clonic seizures?

A

Sodium valproate

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12
Q

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

A

Girls of childbearing potential

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13
Q

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

A

Ethosuximide

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14
Q

What is second line treatment for tonic or atonic seizures ?

A

Lamotrigine

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15
Q

What anti epileptic can impair effectiveness of oral contraceptives?

A

Topiramate

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16
Q

Define atonic seizure

A

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

17
Q

What epilepsy syndromes have myoclonus seizures?

A

Juvenile myoclonic epilepsy
Lennox-Gastaut syndrome
Progressive myoclonic epilepsy

18
Q

What MRI findings are highly suggestive of acute disseminated encephalomyelitis ?

A

Multi focal diffuse white matter changes

19
Q

What is inheritance of friedreich’s ataxia ?

A

Autosomal- recessive

20
Q

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

A

Hypertrophic cardiomyopathy

21
Q

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

A

Friedreich’s ataxia

22
Q

What is characteristic of Landau-kleffner syndrome ?

A

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

23
Q

How do benign Rolandic epilepsy cases present?

A

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

24
Q

Big head, hands and feet is what syndrome?

A

Sotos syndrome

25
Q

Describe infantile spasm

A

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

26
Q

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

A

Rett syndrome

27
Q

Focal seizures occurring during sleep suggests what?

A

Frontal epilepsy

28
Q

What is first line treatment for West Syndrome?

A

Vigabatrin

29
Q

What anti-epileptic may cause peripheral visual field loss?

A

Vigabatrin

30
Q

What is threshold for concern regarding weight centiles?

A

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

31
Q

What is first line treatment for anorexia nervosa?

A

Focused family therapy

32
Q

How do proton pump inhibitors work?

A

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

33
Q

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

A

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

34
Q

Describe sturge-Weber syndrome

A

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

35
Q

What is commonest cause of congenital adrenal hypoplasia

A

21-hydroxy enzyme def

36
Q

What type of cells in Hodgkin’s lymphoma

A

Reed Sternberg cells

37
Q

Describe Lennox-Gestraut syndrome

A

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