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4th Year > Paeds Neuro > Flashcards

Paeds Neuro Flashcards

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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
What are the 4 medical/surgical treatment options for spasticity?
Botox injections Selective dorsal rhizotomy Intrathecal baclofen Deep brain stimulation of basal ganglia
26
What is epilepsy?
2 or more unprovoked epileptic seizures
27
What is a febrile seizure?
Seizure due to a fever but not intracranial infections
28
Who gets febrile seizures?
6m - 6yrs | 10% have family history
29
When do febrile seizures occur?
When pyrexial | Generally during viral illness
30
What type of seizures are febrile seizures?
Usually brief tonic-clonic 40% have more than one
31
Do febrile seizures increase the risk of epilepsy?
1 = no increased risk | Focal, prolonged, or repeated = 4-12% risk
32
What is the management of febrile seizures?
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
What happens in frontal epilepsy?
Motor symptoms Clonic movements travelling proximally = Jacksonian march Tonic seizures eg. raise both arms for a few seconds
34
What happens in temporal epilepsy?
``` Aura - taste/smell Lip smacking Clothes plucking Automatism Deja vu ``` Impaired consciousness
35
What happens in parietal epilepsy?
Contralateral dysaethesias = altered sensations | Distorted body image
36
What happens in occipital epilepsy?
Stereotyped vial hallucinations
37
What investigations are useful in epilepsy?
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
What are the features of a generalised seizure? (4)
Loss of consciousness >3sec No warning Symmetrical seizure Bilateral synchronous discharge on EEG
39
What are the features of absence seizures? (4)
Loss of consciousness Abrupt start and end No motor Sx May be trigged by hyperventilation
40
What is a myoclonic seizure?
Jerking of limbs/neck/trunk
41
What is a tonic seizure?
Generalized increase in tone
42
What is a tonic-clonic seizure?
Tonic = stiff, fall, cyanosed then Clonic = jerking, irregular breathing, biting tongue, urinary incontinence Lasts minutes, followed by deep sleep for few hrs
43
What is an atonic seizure?
Initial myoclonic jerk then loss of muscle tone -> fall
44
What is the management of epilepsy?
Antiepileptics until 2yr seizure-free Need them forever if Juvenile absence/myoclonic epilepsy Valproate/Lamotrigine for generalised Carbamazepine for focal
45
What advice is it important to give patients?
Avoid swimming No driving until 1yr seizure-free Need contraception for girls Epilepsy Action = support group
46
What is status epilepticus?
Seizure lasting 30mins or longer, or repeated seizures without recovery
47
What are the side effects of valproate?
Weight gain Hair loss with curly regrowth Idiosyncratic liver failure
48
What are the side effects of lamotrigine?
Rash Insomnia Ataxia
49
What are the side effects of carbamazepine?
Rash Ataxia Hyponatraemia
50
What is West's Syndrome?
3-12m Appears similar to colic in 20-30sec bursts
51
What does an EEG show for West's Syndrome?
Hypsarrhythmia
52
What is the management of West's Syndrome?
Vigabatrin and steroids 70% response but lots of S/Es Prognosis poor - many develop LD
53
What is Juvenile Absence Epilepsy?
10-20yrs Absence and tonic-clonic seizures, generally with photosensitivity Good response to treatment but will need lifelong
54
What is Juvenile Myoclonic Epilepsy?
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
What is Childhood Absence Epilepsy?
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
Can may induce Childhood Absence Epilepsy?
Hyperventilation/blowing for 2-3mins | Useful for conducting EEG
57
What is Benign Rolandic Epilepsy?
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
What is Parayiotopoulos Syndrome?
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
What are breath-holding spells?
Upset toddler cries, holds breath on expiration, turns blue, briefly loses consciousness Recovers rapidly
60
What is the treatment for breath-holding spells?
Behaviour modification with distraction | Drug therapy = unhelpful

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