Paediatric Neurology Flashcards

1
Q

When does maximum head growth occur?

A

approx. 2 y / o

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2
Q

What is the young brain very susceptible to?

A

insult

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3
Q

What is symbolic play?

A

Pretend play

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4
Q

What is the second most common cancer in children?

A

Brain tumours

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5
Q

Types of headaches in childhood

A
Isolated acute
Recurrent acute
- migraines
Chronic progressive
Chronic non progressive (TTH)
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6
Q

What is a red flag sign of a headache?

A

If the headache is localised to the back of the head

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7
Q

What is looked at in a headache exam?

A
Growth parameters, OFC, BP 
Sinuses, teeth, visual acuity 
- sinusitis, carious teeth, headaches from visual problems 
Fundoscopy
- papilloedema
Visual fields (craniopharyngioma)
Cranial bruit
Focal neurological signs
Cognitive and emotional status
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8
Q

What would the identification of a bruit be indicative of in children?

A

AV fistula

Aneurysm

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9
Q

Examples of primary headaches in children

A

Migraine

Tension headache

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10
Q

Presentation of childhood migraine

A
Pain 
- hemicranial 
- throbbing
- pulsatile
Nausea
Vomiting
Focal symptoms / signs
- visual disturbance
- paraesthesia
- weakness
Pallor
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11
Q

When can focal symptoms / signs of migraine occur in the attack?

A

During
Before
After

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12
Q

What are migraines aggravated by?

A

Bright light

Nosie

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13
Q

What are migraines related to?

A

Fatigue

Stress

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14
Q

What are migraines helped by?

A

Sleep
Rest
Dark
Quiet room

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15
Q

What is often found in the history of migraine?

A

FH

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16
Q

Treatment of childhood migraine

A
Acute attack 
- effective pain relief 
- Triptans
Preventative (at least 1 week)
- Pizotifen 
- Propanolol 
- Amitriptyline
- Topiramate
- Valproate
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17
Q

What is a key feature of tension headaches?

A

Featureless and never severe

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18
Q

How often do tension headaches last?

A

They are there all of the time

But there may be symptom free periods

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19
Q

Features of pain in tension headaches

A

Diffuse, symmetrical pain
A band
Constant ache

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20
Q

Treatment of tension headahce

A
Aim at reassurance; no sinister cause
Attention to underlying chronic, physical, psychological or emotional problems 
Acute attacks - simple analgesia 
Prevention 
- amitriptyline
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21
Q

What is discouraged in chronic TTH?

A

Analgesics

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22
Q

Clues to raised ICP

A
Aggravated / increased by 
- coughing
- straining at stool 
- bending
Woken from sleep with headache
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23
Q

Features of analgesic overuse headache

A

headache is back before allowed to use another dose

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24
Q

What analgesics give analgesic overuse headache?

A

Paracetamol
NSAIDs
particular problem with compound analgesics e.g. cocodamol

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25
Indications for neuroimaging
``` Features of cerebellar dysfunction Features of raised ICP New focal neurological deficit e.g. new squint Seizures esp focal Personality change Unexplained deterioration of school work ```
26
What describes paroxysmal disorders?
Fits, faints and funny turns
27
Seizure vs convulsion
Seizure / fit = any sudden attack where there is prominent motor activity Convulsion = Seizure where there is prominent motor activity
28
Pathology of an epileptic seizure
An abnormal excessive hyper synchronous discharge from a group of (cortical) neurones Chemically triggered by imbalances in your neurotransmitters - decreased inhibition (GABA) - excessive excitation - excessive influx of Na and Ca ions Chemical stimulation produces an electrical current Depolarising current can be measured on EEG Paroxysmal change in motor, sensory or cognitive function
29
Definition of epilepsy
A tendency to recurrent, unprovoked / spontaneous epileptic seizures
30
How many seizures do you need to have to be diagnosed with epilepsy?
2 attacks
31
What is a partial / focal seizure?
Abnormal activity restricted to one hemisphere or part of the hemisphere
32
What is a general seizure?
Abnormal activity coming from both halves of the brain
33
When is an interictal EEG used?
When they are NOT having a seizure
34
EEG is used in identifying...
Seizure types Seizure syndromes Aetiology
35
Investigations for epilepsy
``` History Recording of event ECG in convulsive seizures Interictal / ictal EEG MRI brain Genetics Metabolic tests ```
36
What does an MRI of the brain tell you about epilepsy?
The cause e.g. damage / aetiology
37
Treatment of epilepsy in children
``` Anti-epileptic Drugs (AED) - Sodium valproate (General) - Carbamazepine (focal) Steroids Immunoglobulins Ketogenic diet (mostly for resistant therapies) VNS (palliative procedure for treatment of epilepsy) Surgery ```
38
S/Es of AED
``` CNS related Drowsiness effect of learning cognition behavioural ```
39
Examples of non epileptic seizures
``` Acute symptomatic seizures due to acute insults Reflex anoxic seizures Syncope Sleep Parasomnias e.g. night terrors Behavioural stereotypes Psychogenic seizures (NEAD) ```
40
Causes of acute symptomatic seizures
``` Hypoxia-ischaemia Hypoglycaemia Infection Trauma Pyrexia ```
41
Who is reflex anoxic seizure common in?
Toddlers
42
What triggers reflex anoxic seizures?
Pain Upset Fright
43
What does NEAD stand for?
Non epileptic attack disorder
44
What is a febrile convulsion?
An event occurring in infancy / childhood, usually between 6 months and 5 years of age, associated with fever but without evidence of intracranial infection or defined cause for the seizure
45
What is the commonest cause of acute symptomatic seizure in childhood?
Febrile convulsion
46
Types of seizures
``` Jerk / shake - clonic = rhythmic - myoclonic = one attack - spasms Stiff - tonic seizure usually Falls - atonic - hypertonia / tonic Vacant attack / absence / partial seizure ```
47
Features of a myoclonic seizure
One attack Fully aware but cannot control the jerks Can be very subtle
48
What happens in an atonic seizure?
Sudden drop of tone
49
What is a risk of atonic seizures?
Injury
50
What happens in a hypertonic seizure?
Sudden increase in tone | Posture maintained
51
Presentation of a vacant attack / absence seizure
Acute onset Eye flickering Does not last long
52
What are vacant attacks / absence seizures triggered by?
Hyperventilation
53
What happens in a tonic clonic seizure?
Go blue | Go stiff
54
What do neuromuscular disorders affect?
The peripheral nervous sysem
55
Features of when to suspect a NM disorder
``` Baby 'floppy' from birth Slips from hands Paucity of limb movements Alert but less motor activity Delayed motor milestones Able to walk but frequent falls ```
56
Presentation of NM disorders
Belly sticks out Thin, weak thighs except from the front Poor balance - falls often - awkward clumsy walking Weak muscles in front of the leg cause "foot drop" and tip toe contractures Tight heel cord (contracture) child may walk toes Thick lower leg muscles (mostly fat and not strong) Myopathic facies Sway back Shoulders and arms held back awkwardly when walking Weak butt muscles (hip straightners) Myotonia - difficulty in relaxing the muscles (eyes, fists) Knees may bend back to take the weight Very high foot arch - pess cavis Hammar toes
57
Features of myopathic facies
Ptosis Cant close mouth due to shape Lack of muscle control around mouth Poor head position control
58
NM muscle conditions
Muscle dystrophies Myopathies congenital / inflammatory Myotonic syndromes
59
NM NMJ conditions
Myasthenic syndromes
60
NM nerve conditions
Hereditary or acquired neuropathies
61
NM anterior horn cell conditions
Spinal muscular atrophy
62
Genes of Duchenne Muscular Dystrophy
Xp21 | Dystrophin gene
63
Who gets Duchenne Muscular Dystrophy?
Female carriers | Males have the disease
64
Presentation of Duchenne Muscular Dystrophy
``` Delayed gross motor skills Symmetrical proximal weakness - waddling gait (pelvic waddle) - calf hypertrophy (replaced by fat) - Gower's sign positive Elevated Creatinine kinase - > 1000 Cardiomyopathy Respiratory involvement in teens ```
65
What is creatinine kinase a measure of?
Muscular breakdown
66
What value of creatinine kinase is normal?
200
67
Treatment of Duchenne Muscular Dystrophy
Steroids | No cure
68
Prognosis of Duchenne Muscular Dystrophy is what and depends on what?
With treatment can live to early 30s | Depends on gene type
69
Characteristics of febrile seizures
``` Temp > 38.4C Age 6 months - 5 years Peak 18 - 22 months Usually after 1 - 24 hours of recognised fever Seizure type is generalised or focal ```
70
Two broad types of seizures
Typical | Atypical
71
Features of a typical seizure
Generalised convulsion for < 15 mins | Single convulsion in one illness
72
Features of an atypical seizure
Focal Lasts for > 15 mins Abnormal neurological signs Developmental delay
73
What condition protects against febrile seizures?
Gastroenteritis (apart from shigella)
74
Does febrile seizures run in the family?
Yes
75
Investigations of a seizure
``` PLASMA GLUCOSE! Electrolytes CSF examination FBC Neuroimaging EEG ```
76
Treatment of febrile convulsion
Antipyretics
77
Do antipyretics reduce the risk of recurrence in febrile convulsions?
No
78
Complications of febrile seizures
Todds paresis Recurrent of FS Epilepsy
79
What is the chance of a febrile seizure happening again?
1 in 3
80
Atypical febrile seizures increased he risk for developing epilepsy in the future by how much?
8-9%
81
How long is a prolonged seizure?
5 mins or more
82
Definition of an epileptic seizure
An electrical phenomenon in which an abnormally excessive synchronous discharge from a group of neurones (usually from cerebral cortex) occurs
83
Definition of epilepsy
Tendency to recurrent, unprovoked, spontaneous epileptic seizures
84
Definition of a symptomatic seizure
A seizure from a secondary cause e.g. brain tumour, hypoglycaemic etc
85
Types of seizures
``` Focal/partial - simple (consciousness retained) - complex (consciousness impaired) - secondary generalised Generalised - absence - myoclonic jerks - tonic / clonic / GTC - atonic / drop attacks ```
86
Differential diagnosis of a seizure
``` Reflex anoxic seizure Vasovagal syncope Breath holding attack Sleep related events (parasomnias) GORD Shuddering attack, temulousess/startles (hyperekplexia) Benign positional vertigo Pseudo seizures ```
87
1st line treatment for generalised epilepsy
Valproate
88
1st line treatment for focal epilepsy
Carbamazepine
89
Other treatments for seizures
``` Steriods IgS Ketogenic diet Vagus nerve stimulation Surgery ```
90
Who gets reflex anoxic seizures?
Toddlers
91
Pathology of reflex anoxic seizures
Triggers cause vasovagal overactivity
92
Treatment for reflex anoxic seizures
None
93
When do frontal lobe seizures particularly happen?
At night
94
Features of GORD that look like a seizure
Babies arch their back and clench hands to prevent symptoms
95
Features of infantile spasms
Repetitive
96
Recovery of reflex anoxic seizures vs epileptic seizures
Reflex anoxic - rapid recovery | Epileptic - prolonged recovery
97
What are the seizure patterns in west syndrome?
Infantile spasms
98
When does west syndrome present?
First 4 - 8 months
99
Which gender is west syndrome more common in?
Males
100
What is west syndrome often associated with?
A serious underlying condition and carry a poor prognosis
101
Presentation of west syndrome
Characteristic 'salaam' attacks; flexion of head, trunk and arms followed by the extension of the arms Lasts 1 - 2 seconds but may be repeated up to 50 times Progressive mental handicap
102
Investigation of west syndrome
EEG - hypssarrhytmia in 2/3rds of infants | CT demonstrates against diffuse or local brain disease in 70% (e.g. tuberous sclerosis)
103
Prognosis of west syndrome
Poor
104
Treatment of west syndrome
Vigabatrin 1st line | ACTH
105
Most common cause of headache in children
Migraine