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4th Year > Paeds Neuro > Flashcards

Paeds Neuro Flashcards

1
Q

Define cerebral palsy

A

Permanent disorder of movement and/or posture, and of motor function due to a non-progressive abnormality in developing brain

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2
Q

What if brain is damaged after 2yrs old?

A

Acquired Brain Injury

NOT cerebral palsy

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3
Q

What causes 80% of cerebral palsy?

A

Antenatal factors eg. cerebrovascular haemorrhage/ischaemia, maldevelopment, congenital infection

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4
Q

What makes up the other 20% of cerebral palsy?

A

10% hypoxihc-ischaemic injury intrapartum

10% postnatal

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5
Q

Why are preterms more at risk of CP?

A

Vulnerable to periventricular leukomalacia, severe intraventricular haemorrhage, and venous infarcts

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6
Q

What are the post-natal causes of CP?

A

I+4H

Infection
Head injury
Hypoglycaemia
Hydrocephalus
Hyperbilirubinaemia
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7
Q

What are the types of CP?

A 
Spastic = 90%
Dyskinetic = 6%
Ataxic = 4%
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8
Q

What are the early features of CP?

A 
Delayed milestones
Difficulties feeding eg. slow, gagging, vomiting
Asymmetric hand function <12m
Abnormal gait
Primitive reflexes may persist
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9
Q

Gross Motor Classification System - 5 levels

A 
1 = walks without limitation
2 = walks with limitation
3 = walks with hand-held mobility device
4 = self-mobility with limitation eg. powered mobility
5 = manual wheelchair
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10
Q

What are the features of spastic CP?

A

Tone increased
Brisk tendon reflexes
Clasp-knife rigidity

Presents early

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11
Q

What is unilateral spastic CP?

A

Presents 4-12m with fisting of hand, pronated forearm and flexed wrist
Tiptoe walking on affected side

Affects arm more than leg
Spares the face

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12
Q

What is bilateral spastic CP?

A

Affected all limbs
Trunk tendency to opisthotonus (extensor posturing)
Poor head control

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13
Q

Why is bilateral spastic CP bad?

A

Severe

Associated with seizures, microcephaly and mod-severe intellectual disability

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14
Q

What is bilateral diplegia?

A

Legs affected more than arm
Hand function may appear normal

Associated with Periventricular Leukomalacia on MRI

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15
Q

What the features of dyskinetic CP?

A

Involuntary, uncontrolled, stereotyped movements
Primitive reflexes remain

Signs = chorea, athetosis and dystonia

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16
Q

What is chorea?

A

Chorea = irregular, sudden and brief non-repetitive movements

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17
Q

What is athetosis?

A

Athetosis = slow, writhing, distal movements eg. fanning fingers

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18
Q

What is dystonia?

A

Dystonia = contraction of agonist and antagonist muscles at the same time, causes twisting appearance

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19
Q

When do chorea, athetosis and dystonia arise in dyskinetic CP?

A

10-12m

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20
Q

What causes chorea, athetosis and dystonia?

A

Basal ganglia dysfunction - shown on MRI

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21
Q

What causes basal ganglia dysfunction leading to CP?

A

Hypoxic-ischaemic encephalopathy

Previously kernicterus secondary to hyperbilirubinaemia

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22
Q

What causes ataxic CP?

A

Generally genetically determined

May be due to ABI, features are often ipsilateral to lesion and symmetrical

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23
Q

What are the early features of ataxic CP?

A

Trunk and limb hypotonia
Poor balance
Delayed motor development

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24
Q

What are the later features of ataxic CP?

A

Uncoordinated movements
Intention tremor
Ataxic gait

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25
Q

What are the 4 medical/surgical treatment options for spasticity?

A

Botox injections
Selective dorsal rhizotomy
Intrathecal baclofen
Deep brain stimulation of basal ganglia

26
Q

What is epilepsy?

A

2 or more unprovoked epileptic seizures

27
Q

What is a febrile seizure?

A

Seizure due to a fever but not intracranial infections

28
Q

Who gets febrile seizures?

A

6m - 6yrs

10% have family history

29
Q

When do febrile seizures occur?

A

When pyrexial

Generally during viral illness

30
Q

What type of seizures are febrile seizures?

A

Usually brief tonic-clonic

40% have more than one

31
Q

Do febrile seizures increase the risk of epilepsy?

A

1 = no increased risk

Focal, prolonged, or repeated = 4-12% risk

32
Q

What is the management of febrile seizures?

A

Rule out meningitis - give Abx if can’t get LP
Buccal midazolam if >5mins
Paracetamol won’t prevent but will make child more comfortable

Antipyretics and anti epileptics do not prevent

33
Q

What happens in frontal epilepsy?

A

Motor symptoms

Clonic movements travelling proximally = Jacksonian march
Tonic seizures eg. raise both arms for a few seconds

34
Q

What happens in temporal epilepsy?

A 
Aura - taste/smell
Lip smacking
Clothes plucking
Automatism
Deja vu

Impaired consciousness

35
Q

What happens in parietal epilepsy?

A

Contralateral dysaethesias = altered sensations

Distorted body image

36
Q

What happens in occipital epilepsy?

A

Stereotyped vial hallucinations

37
Q

What investigations are useful in epilepsy?

A

ECG - exclude long-QT syndrome
EEG - ictal/interictal/ambulatory-24hr
MRI-FLAIR - looking for mesial temporal sclerosis which can be cured surgically
PET/SPECT - if considering surgery

38
Q

What are the features of a generalised seizure? (4)

A

Loss of consciousness >3sec
No warning
Symmetrical seizure
Bilateral synchronous discharge on EEG

39
Q

What are the features of absence seizures? (4)

A

Loss of consciousness
Abrupt start and end
No motor Sx
May be trigged by hyperventilation

40
Q

What is a myoclonic seizure?

A

Jerking of limbs/neck/trunk

41
Q

What is a tonic seizure?

A

Generalized increase in tone

42
Q

What is a tonic-clonic seizure?

A

Tonic = stiff, fall, cyanosed
then Clonic = jerking, irregular breathing, biting tongue, urinary incontinence

Lasts minutes, followed by deep sleep for few hrs

43
Q

What is an atonic seizure?

A

Initial myoclonic jerk then loss of muscle tone -> fall

44
Q

What is the management of epilepsy?

A

Antiepileptics until 2yr seizure-free
Need them forever if Juvenile absence/myoclonic epilepsy

Valproate/Lamotrigine for generalised
Carbamazepine for focal

45
Q

What advice is it important to give patients?

A

Avoid swimming
No driving until 1yr seizure-free
Need contraception for girls

Epilepsy Action = support group

46
Q

What is status epilepticus?

A

Seizure lasting 30mins or longer, or repeated seizures without recovery

47
Q

What are the side effects of valproate?

A

Weight gain
Hair loss with curly regrowth
Idiosyncratic liver failure

48
Q

What are the side effects of lamotrigine?

A

Rash
Insomnia
Ataxia

49
Q

What are the side effects of carbamazepine?

A

Rash
Ataxia
Hyponatraemia

50
Q

What is West’s Syndrome?

A

3-12m

Appears similar to colic in 20-30sec bursts

51
Q

What does an EEG show for West’s Syndrome?

A

Hypsarrhythmia

52
Q

What is the management of West’s Syndrome?

A

Vigabatrin and steroids
70% response but lots of S/Es
Prognosis poor - many develop LD

53
Q

What is Juvenile Absence Epilepsy?

A

10-20yrs
Absence and tonic-clonic seizures, generally with photosensitivity
Good response to treatment but will need lifelong

54
Q

What is Juvenile Myoclonic Epilepsy?

A

10-20yrs
Myoclonic, tonic-clonic and absence seizures may occur, generally in the morning
Typical Hx = throwing breakfast due to myoclonus
Good response to treatment but will need lifelong

55
Q

What is Childhood Absence Epilepsy?

A

4-12yrs, 2% childhood epilepsy, 2/3 female
Momentary unresponsive state <30sec
No recollection, only knows that they’ve missed something
No LD
80% remission

56
Q

Can may induce Childhood Absence Epilepsy?

A

Hyperventilation/blowing for 2-3mins

Useful for conducting EEG

57
Q

What is Benign Rolandic Epilepsy?

A

4-10yrs, 15% childhood epilepsy
Have tonic-clonic seizures in sleep
EEG shows focal sharp waves from rolandic area
Completely benign, remits in teens, no Tx

58
Q

What is Parayiotopoulos Syndrome?

A

1-5yrs, 5% childhood epilepsy
Vomiting and unresponsive staring in sleep with head and eye deviation
May progress to convulsions
EEG shows posterior sharp waves and occipital discharge even with eyes closed
Remits in childhood, no Tx, may have some specific LD

59
Q

What are breath-holding spells?

A

Upset toddler cries, holds breath on expiration, turns blue, briefly loses consciousness
Recovers rapidly

60
Q

What is the treatment for breath-holding spells?

A

Behaviour modification with distraction

Drug therapy = unhelpful

4th Year (15 decks)

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