Neuro Flashcards

1
Q

what are the types of headaches?

A
  1. Primary →
    a. migraine,
    b. tension-type,
    c. cluster,
    d. other eg stabbing
  2. Secondary → symptomatic of underlying pathology (↑ ICP, SOL) meningitis, alcohol withdrawal, medication overuse, Acute sinusitis.
  3. Trigeminal and other cranial neuralgias → eg. nerve root pain from herpes zoster
  • Episodic → <15 days per month
  • Chronic → 15+ days per month
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2
Q

how do tension headaches present?

A

This is a symmetrical headache of gradual onset, often
described as tightness, a band or pressure. There are
usually no other symptoms.

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3
Q

how do MIGRAINE WITHOUT AURA present?

A
  • 90% of migraine
  • Episodes lasting 1-72 hours, commonly bilateral but can be unilateral
  • Pulsatile over temporal or frontal area
  • Can be accompanied by GI disturbance eg. nausea, vomiting, abdo pain, photo/phono-phobia
  • Aggravated by physical activity
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4
Q

how do MIGRAINE WITH AURA present?

A
  • 10% of migraine
  • Preceded by aura (visual, sensory, motor), but can occur without a headache:

migraine aura often begins with positive phenomena such as shimmering lights, zigzags in the vision, or tingling and

then followed in minutes by negative symptoms such as scotoma, numbness, or loss of sensation

  • Characteristically: absence of problems between episodes, frequent presence of premonitory symptoms
  • Aura symptoms are fully reversible
  • Commonly visual

• Episodes last a few hours

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5
Q

what are the risk factors and triggers for migraine?

A

• Often triggered by disturbance to inherent biorhythms – late nights, stress, winding down (cheese, chocolate, caffeine)

  • Genetic predisposition
  • Oral Contraceptive Pill and menstruation
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6
Q

what are the postivie and negative phenomena in migraine?

A

o Negative phenomena
▪ Hemianopia
▪ Scotoma (small areas of visual loss)
numbness, or loss of sensation

o Positive phenomena
▪ Fortification spectra
shimmering lights, zigzags in the vision, or tingling

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7
Q

what are the Uncommon forms of migraine?

A

caused by;

Benign paroxysmal vertigo of childhood
Abdominal migraine
Cyclical vomiting

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8
Q

In raised ICP, which nerve is most commonly affected?

what signs are seen and why?

A

VIth (abducens) cranial nerve has a long
intracranial course and is often affected when
there is raised pressure, resulting in a squint with
diplopia and inability to abduct the eye beyond
the midline. It is a false localising sign.

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9
Q

how do cluster headaches present?

A
  • Unilateral (around eye/side of face)
  • Severe, sharp/burning/throbbing pain
  • ± watery eye, nasal congestion/runny nose, swollen eyelid, facial swelling, constricted pupil/droopy eyelid
  • Ranges from 1 every other day to 8 per day
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10
Q

which red flag features should alert on headaches involving raised ICP and SOL ?

A
  1. Worse on lying down and morning vomiting
  2. Can cause night-time waking
  3. ± change in mood, personality, educational performance

others:
• torticollis (tilting of the head)
• growth failure, e.g. if craniopharyngioma or hypothalamic lesion
• papilloedema – a late feature
• cranial bruits – may be heard in arteriovenous malformations but these lesions are rare
• early or late puberty.
papilloedema - late sign

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11
Q

How do we ivx and mx headaches?

A
  1. Detailed explanation and advice
    • Ivx / Imaging not needed unless red flag features present

o Medication cannot cure but can help symptoms

Options:
o Analgesia - Paracetamol and NSAIDS (all ages)
o Anti-emetics:
▪ 6+ → cyclizine
▪ 12+ → prochlorperazine and metoclopramide, consider codeine phosphate

2nd line
A o Serotonin 5HT1 agonists → if 12+ → consider sumatriptan, nasal preparation

• Prophylaxis
o If headaches are frequent, obtrusive
1 ▪ Beta blockers → propranolol → contraindicated in asthma

o Refer to Neurology consultant for guidance:
2 ▪ Na channel blockers → sodium valproate, topiramate
3▪ Pizotifen → 5HT agonist

o Psychiatry support
▪ Decrease stressors → anxiety, bullying, relaxation techniques

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12
Q

caveat of Na channel blockers eg topiramate in headache rx?

A

• Note → fetal malformation link and decreased effectiveness of OCP – fertile young females should be on 2 birth control methods

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13
Q

caveat of 5HT agonist in headache rx?

A

causes increased weight and sleepiness

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14
Q

How would you manage a migraine?

A

1o Assess the severity and frequency of attacks, and the impact on the patient’s life:

2o Headache diary - 8 weeks to identify triggers
▪ Identify triggers (menstruation)
▪ Record frequency, duration, severity

Acute Management (in 12-17 year olds)
o Step 1: Paracetamol or ibuprofen

o Step 2: Nasal sumatriptan

o Step 3: Nasal triptan + NSAID/paracetamol

§ Consider adding anti-emetic e.g. metoclopramide or prochlorperazine

Follow-up within 1 month -> return sooner if symptoms get worse

§ NOTE: ORAL triptans are not licensed in people < 18 years

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15
Q

Define a seizure.

What are the types of seizures?

A

Seizure:
An ACUTE & TRANSIENT (<5 mins) neurological events caused by abnormal electrical discharges within the brain.

  1. Epileptic
    - a disease characterised by an persistent predisposition to generate epileptic seizures
    - due to excessive electrical activity
    - most commonly idiopathic (70-80%)
2. Non-epileptic
Triggers iniclude stress
No EEG changes in brain
Still having jerking/shaking
Not made up or being done on purpose
Not epilepsy
usually outgrown
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16
Q

what is the difference between a seizure and convulsions?

A

A convulsion is a seizure (epileptic or non-epileptic)
with motor components, particularly stiff (tonic), a
massive jerk (myoclonic), jerking (clonic), trembling
(vibratory), thrashing about (hypermotor);

as opposed to a non-convulsive seizure with motor arrest, e.g. an unresponsive stare (as in generalized epileptic absence
seizures and some focal seizures), or drop
attack (as in an epileptic atonic seizure).

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17
Q

define epilepsy?

A

Epilepsy is a brain disorder that predisposes the
patient to have RECCURENT UNPROVOKED seizures.

Definition/diagnosis: At least two unprovoked (or reflex) seizures occurring more than 24 hours apart

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18
Q

define febrile seizure?

what are the NICE criteria?

incidence?

A

Seizure AND fever, in the absence of intracranial infection.

Child has to be between 6 month – 6 years

incidence: 3%

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19
Q

characterise febrile seizures?

prognosis?

A

Occurs in a viral infection when the temperature is rapidly rising.

  • Usually a brief, generalised tonic clonic seizure
  • Some genetic predisposition, 30% will have further seizures

Prognosis:
- About 1 in 3 children will have another febrile seizure.

  • Simple febrile seizures do not cause brain damage and do not ↑ risk of epilepsy
  • Complex febrile seizures → focal, prolonged, repeated in same illness, will lead to an ↑ risk of 4-12% of subsequent epilepsy
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20
Q

how do we ivx febrile seizures?

A

IX:
• Not routinely needed

Clinical diagnosis
• Lumbar puncture - if meningitis or encephalitis suspicion
• Urine MCS - if UTI suspected
• Check blood glucose

• EEG not recommended as it does not guide treatment or predict recurrence

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21
Q

How do we mx an acute febrile seizures?

A

DURING THE SEIZURE:

Basic safety protocol; timing, rercording, recovery position etc

If the seizure lasts > 5 mins: (call ambulance)

Simple seizure: nothing needed, can give antipyretics.

  1. -> Buccal midazolam or Rectal diazepam

1b -> Call an ambulance, if 10 mins after the first dose:
§ Seizure has NOT stopped
§ Child has ongoing twitching
§ Another seizure has started before the child has regained consciousness

1c. Can repeat once after 10 mins if the seizure hasn’t stopped

  1. Identify and treat cause
    • admit if meningitis
    • if no foci of infection found - admit n observe
  2. Immediate hospital assessment by a paediatrician if: conditions met eg lasted >15mins, or <1.5y/o

Can give Ibuprofen/paracetamol BUT they dont help.

Current febrile illness + hx of febrile seizure:
Give prophylactic diazepam (after specialist referral)
—————–
History of 2+ COMPLEX febrile seizures, diazepam uneffective:
- prophylactic anti-epileptic (after specialist referral)
—————–
Status epilepticus:
- Refer to paeds neurologist
- Benzodiazipine
- Start full protocol

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22
Q

when is a paeds review needed after febrile seizure?

A

§ First febrile seizure or if second seizure in a child who has not been assessed before

§ < 18 months old
§ Diagnostic uncertainty about the cause of the seizure
§ Seizure lasted > 15 mins (complex seizure)
§ Focal features during the seizure

§ Seizure recurred in the same febrile illness (or within 24 hours)
§ Incomplete recovery after 1 hour
§ No serious clinical findings but is currently taking antibiotics
§ Parents are anxious and feel that they cannot cope
§ Suspected cause of the fever (e.g. pneumonia)

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23
Q

what are the causes of non-epileptic seizures?

A

• Convulsive syncope
– Cardiac syncope e.g. prolonged Q-T syndrome
– Neurally mediated syncope:
(reflex anoxic seizures), (vasovagal syncope)

– Expiratory apnoea syncope (“blue breath holding
spells”)

– Hypovolaemic syncope e.g. with haemorrhage, dehydration or anaphylaxis

• Sudden rise in intracranial pressure e.g.
hydrocephalic attack, haemorrhage

• Sleep disorders e.g. benign neonatal sleep myoclonus, hypnic jerks

• Functional/medically unexplained e.g.
dissociative states

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24
Q

what are the differentials for seizures in kids?

paroxysmal disorders (‘funny turns’} in kids

A

‘Blue breathholding’ spells

Reflex anoxic seizures (asystolic syncope)

Syncope (transient loss of consciousness) - eg in a hot room or standing for long periods or from fear. ± clonic movements

Benign paroxysmal vertigo

Long QT syndrome / arrhythmia

Benign myoclonus

Pseudo seizure

Panic attacks

Tics

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25
Q

what happens in breath holding attacks?

management?

A

• Toddlers when upset, child cries, holds their breath, goes blue, may briefly lose consciousness and go limp, rapidly recovers fully

  • Attacks resolve spontaneously, drug therapy unhelpful
  • Manage with behaviour modification and distraction
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26
Q

what are reflex anoxic seizures?

A
  • Infants and toddlers
  • Triggered by - pain, head trauma, cold food (ice cream), fright, fever
  • Child becomes pale and falls to floor ± general tonic clonic
  • Episodes due to cardiac asystole due to vagal inhibition
  • Seizure is brief, and child recovers rapidly
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27
Q

what are the causes, presentation and ivx for Vasovagal syncope?

A

Causes
• Emotional → fear, pain, shock, sudden sounds or sights
• Orthostatic/ Postural hypotension → prolonged standing, crowds, hot

Clinically:
• Brief LOC, spontaneous recovery, no signs of seizure activity, link to trigger

IX:
• BP measurement standing AND sitting (Postural)

± ECG if indicated
• Need to rule out any cardiac cause

• FBC to rule out bleeding / anaemia

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28
Q

how do we manage vasovagal syncope?

A

Patient education – teaching how to spot warning symptoms, avoid triggers and learn how to abort attacks

• Physical techniques such as physical counter-pressure manoeuvres and tilt training

• Increase volume – increase dietary salt and electrolyte rich sports drinks
o May give fludrocortisone

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29
Q

what are risk factors for epilepsy?

A
• Risk factors
o Genetic disposition
o Perinatal asphyxia
o Metabolic disorders
o Trauma
o Structural CNS abnormalities
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30
Q

How do Absence seizures and Tonic clonic seizures present?

A

• Absence seizures
o Brief impairment of consciousness, 5-10 seconds
o Behavioural arrest or staring – interrupting normal activity

• Tonic clonic
o Patient falls unconscious ± preceding aura
o Violent muscle contractions and shaking
o Stiffness, jerking
o Ask about symmetry of movements
o Eyes may roll back, tongue biting, incontinence
o Post-ictal phenomena
“tonic” = stiff

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31
Q

Define status epilepticus?

prognosis?

A

Old definition: a seizure needed to last longer than 20 minutes to be considered status epilepticus

New definition:
1. A single seizure that last more than 5 minutes.
OR
2. Two or more seizures within a 5-30minute period with no recovery in-between.

prognosis: 15-20% mortality within 30 days.

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32
Q

how do we manage status epilepticus?

A
  1. ABCDE, secure airway, check blood glucose,

If seizure lasts more than 5 minutes:

Step 2. Then get IV access → IV lorazepam

If no IV access:
-> Buccal midazolam OR Rectal diazepam (repeat after 5 mins if seizure has not stopped)

Step 3. Second dose of Benzo / IV lorazepam (15mins)

  • call senior help ; anaesthetics
  • prep phenytoin
  • note; max 2 doses benzodiazepines regardless iv/buccal/rectal

Step 4. (25mins) Phenytoin 20 mg/kg by intravenous infusion over 20 mins

->Consider rectal paraldehyde 0.8 ml/kg whilst awaiting anaesthetist if seizure aint stopped

if on regular phenytoin:
4b -> Phenobarbital 20 mg/kg intravenously over 5 mins
->Consider rectal paraldehyde 0.8 ml/kg

Step 5: Rapid sequence induction of anaesthesia using thiopental sodium (basically put them to sleep fast) - this is what the anaesthetists would do. its a muscle relaxant

Protect from injury → cushion head, remove harmful objects, make sure environment is safe, do not restrain

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33
Q

which drugs are suited for the different types of seizures?

A

• Tonic-clonic → Valproate
2nd line Lamotrigine

  • Absence → ethosuximide or valproate
  • Myoclonic → valproate
  • Infantile spasms → vigabatrin
  • Partial → carbamazepine or lamotrigine
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34
Q

what are the complications of Anti-epileptic treatment?

A

• Lamotrigine → Steven Johnson Syndrome (toxic epidermal necrosis, life threatening skin condition)

  • Phenytoin → gingival hypertrophy
  • Carbamazepine → neutropenia, rickets
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35
Q

How do we ivx seizures?

A

Depends on what your dfx are for the cause
May need to do:

Septic screen;
Blood cultures, Urine MCS, LP
(want to localise infectious source)

Bloods; 
FBC - WCC, CRP, ESR (infection)
U+Electrolytes - (hypokalaemia/calcaemia)
Blood glucose
Genetic screening

ECG - electrolyte imbalance
EEG - can do a sleep EEG or use photo stimulation to pick up abnormalities better

CT - if suspecting SOL (much quicker logistically than MRI - can do MRI later for more detialed picture)

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36
Q

What are the types of seizures?

A
GENERALISED
A. Tonic-clonic (stiffness+jerking)
B. Myoclonic (shock like)
C. Atonic (sudden loss of tone - drop attacks)
D. Absence - non motor

FOCAL/PARTIAL
A. Secondary generalised (start focal but spreads to involve both sides)
B. Motor/Sensory (causing either one-sided motor or sensory disturbance - feeling or visual etc)
C. Simple/Complex (s- retained consciousness, c- LOC)

-> note partial seizures can never not be worrying -> must be investigated - head imaging

UNKNOWN

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37
Q

How do Myoclonic seizures present?

what are the subtypes?

A
o Brief arrhythmic muscular jerking movements
o Last a few seconds
- sudden jerking or twitching
- symmetric
- can happen 100s of times a day

note they can have the jerks without a seizure as per say, check the EEG- in such cases, no treatment needed, reassure that it is not epilepsy

o Juvenile myoclonic epilepsy
▪ Usually involving neck, shoulders, upper arms
- most occur after WAKING UP
- There is often a mixture of absence, myoclonic and tonic–clonic seizures, so;
- poor concentration / daydreaming etc
▪ Begin around puberty/TEENS
- reassure as usually outgrown

o Progressive myoclonic epilepsy
▪ Rare syndromes
▪ Combination of myoclonic and tonic-clonic
▪ Patient deteriorates over time

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38
Q

which seizures in kids tends to be photosensitive (flashing or flickering lights)?

A

Juvenile myoclonic epilepsy

events cluster in the morning

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39
Q

what are the causes of epilepsy?

A

VITAMIN

Vascular - Stroke
Infection - meningitis, Neurocysticercosis
Truama - CNS injury
Autoimmune - SLE

Metabolic disorders - MELAS, hypoglycaemia or hyponatraemia, calcium changes (in young infants make sure to exclude inborn errors of metabolism)

Idiopathic - most common cause
Neoplasm - Brain tumours/SOLs

Note:
It is only epilepsy if the underlying cause is treated but seizures remain.

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40
Q

What would EEG show in seizures & epilepsy?

A

Myoclonic seizures eg Juvenile myoclonic epilepsy:

  • Polyspikes
  • polyspikes and waves
  • > atonic presents similarly

Tonic-clonic seizures
- varies in tonic, clonic and postictal phases

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41
Q

what does childhood absence epilepsy involve?

A

numerous absence seizures each day

kids may appear to be daydreaming and may be diagnosed with an attention deficit

42
Q

how may focal seizures present?

A

Lateralising signs:

  • To the contralateral side
  • Todds paralysis - postictal weakness on side opposite to seizure

Localising signs:
Temporal - automatisms, aura, speech arrest
Parietal - somatosensory auras
Frontal - nocturnal, behavioural
Occipital - flashing lights, geometric shapes

43
Q

What are INFANTILE SPASMS?

Aetiology and presentation?

what is west syndrome

A

seizure disorder that presents frorm 3-8 months of age

It is serious! Aggressive treatment needed! 1 in 2000 live births.

typical movement : sudden flexion (bending forward) in a tonic (stiffening) fashion of the body and legs +

extensor spasms - extension of arms + ‘shrug of shoulders’

  • can also have head bobbing

each episode lasts just 1 or 2 seconds after which there is a pause for between five and ten seconds which is then followed by a further spasm.

spasms can happen singly, but
more commonly ‘clusters’. There may be 5 or up to 20 or more spasms in a ‘cluster’.

may be associated/ triggered by sleep, feeding hence;

can be diagnosed late as are often misdiagnosed as colic / reflux or myoclonus due to pattern of movement.

Child develops:

  • Difficulty feeding
  • Slowed/ reversed development
  • Sleep pattern changes
44
Q

How do we ivx Infantile spasms?

A

History

Parents - tell them to record the episodes and bring to doctor

EEG shows a very disorganised pattern, called hypsarrhythmia

  • may only show in sleep so 2 EEGs needed
  • always abnormal in west syndrome

MRI

Bloods, Urine - looking for underlying cause

45
Q

what is West’s syndrome ?

A

(infantile spasms, developmental plateau, and hypsarrhythmia).

46
Q

How do we treat infantile spasms?

A

1st line - Prednisolone
- (for 2 weeks - longer if doesnt stop)

Or Tetracosactide

  • synthetic ACTH
  • can do this + prednisolone
    • risks: immunosuppression, HTN, high glucose

Alternatively;
Vigabatrin - 1st line anti epileptic
- risks: peripheral vision loss

Treat underlying cuase if any
Monitor developmental progress

47
Q

what is the goal of rx in infantile spasms?

complications ?

A

To treat/ control seizures before progression to West syndrome

or before cognitive impairment
or impaired development

want to see a clear EEG + no seizures
good outcomes associated with good EEGs

complications:
severe epilepsy
risk of sudden death - SUDEP

48
Q

wat is the presentation and ivx of EXTRADURAL HAEMORRHAGE ?

A
  • Usually following direct trauma
  • Can be associated with skull fracture – tear of middle meningeal artery (75%)

forms between the inner surface of the skull and outer layer of the dura

Clinically:
• Nausea, vomiting, headache, confusion
• LOC immediately after a head injury followed by Lucid interval followed by progressive LOC (hours) and seizures

• +/- focal neuro signs
     o Dilatation of ipsilateral pupil
     o Paresis of contralateral limb
     o Anaemia
     o Shock

Raised ICP -> brainstem herniation + death

49
Q

typical signs in EXTRADURAL HAEMORRHAGE include?

A

Tenderness of the skull (in the context of injury)
Confusion

Reduced Glasgow Coma Score
Cranial nerve deficits (e.g. oculomotor nerve palsy causing fixed dilation of the ipsilateral pupil)

Motor or sensory deficits of the upper and/or lower limbs (e.g. hemiparesis, paraesthesia)

Hyperreflexia and spasticity

Upgoing plantar reflex (Babinski’s sign)

Cushing’s triad: a physiological response to raised intracranial pressure including bradycardia, hypertension and deep/irregular breathing.

50
Q

how do you ivx supected EXTRADURAL HAEMORRHAGE?

A

• Dx confirmed by CT scan:

  • Primarily: bi-convex “lemon-shaped” mass
  • Secondary: midline shift and brainstem herniation

Bloods:
Glucose - rule out hypoglycaemia
Coagulation studies - warfarin blood thinning
G&S - ahead of surgery

51
Q

how do we mx EXTRADURAL HAEMORRHAGE?

A

Neurosurgery or interventional radiology referral.

Definitive: Craniotomy
- which type depends on sixe of lesion, midline and BS invovlement

Prophylactic antibiotics - if skull fracture - reduce infection.

Anticonvulsant medication - eg levetiracetam

Agents to reduce ICP - Mannitol
correct coagulopation deficits and other abnormalities

52
Q

which brain haemorrhage is characteristically associated with NAI / shaking of a baby ?

A

SUBDURAL HAEMORRHAGE

between Dura + arachnoid matter
• Results from Rupture of bridging cranial veins

Gradual deterioration eg over 2 weeks

• +/- retinal haemorrhages

also trauma associated

53
Q

what is the CT appearance of SUBDURAL HAEMORRHAGE?

A

concave / crescent shaped

54
Q

which brain haemorrhage is characteristically associated with thunderclap headache?

aetiology?

A

Subarachnoid haemorrhage

rupture of berry aneurysm
rare in kids

acute onset head pain, neck stiffness, fever +/- seizures or coma

IX: CT- defect around circle of willis

avoid LP in acute setting due to risk of increased ICP.

55
Q

what is the presentation and risk factors of INTRAVENTRICULAR HAEMORRHAGE?

A

RFs:
premature babies
ECMO in preterm infants,
Congenital CMV infection

  • Present with sleepiness and lethargy
  • Can also have apnoea, reduced/absent Moro reflex, Poor tone, Tense fontanelle, Coma
56
Q

whatt are the grades of INTRAVENTRICULAR HAEMORRHAGE?

A
  • Grade I – bleeding just in germinal matrix
  • Grade II – intraventricular bleeding (but no enlargement)
  • Grade III – ventricles enlarged by accumulated blood
  • Grade IV – bleeding extends into brain tissue around ventricles

3/4 can cause hydrocephalus
50% of infants with post-haemorrhagic ventricular dilatation have cerebral palsy

57
Q

how do we mx INTRAVENTRICULAR HAEMORRHAGE?

A
  • Fluid replacement
  • Anticonvulsant
  • Acetazolamide (reduce CSF)
  • Surgery – VPS
58
Q

what is the prevalence of CNS tumours in children?

A

Astrocytoma 40%
- Benign to highly malignant, can be posterior fossa too

Medulloblastoma 20%
- Associated spinal mets, arise from midline posterior fossa

Ependymoma 8%
- Mostly posterior fossa

Brainstem glioma 6%
- Malignant tumours with poor prognosis

Craniopharyngioma 4%
- Squamous remnant of Rathke pouch (primitive oral cavity) not truly malignant but locally invasive

Atypical teratoid/rhabdoid tumour (ATRT)
- Rare type of aggressive tumour commonly occurring in young children

59
Q

list the presentation of cns tumour based on locatoin?

A
  • Headaches (worse in morning, coughing), vomiting (on waking), gait problems, co-ordination problems, clumsy, irritability, failure to thrive, visual changes, behaviour or personality change.
  • ↑ ICP → cushings triad etc
  • Separation of sutures/tense fontanelle, developmental delay or increased head circumference
  • Depending on site of tumour
o Supratentorial (CORTEX) → seizures, hemiplegia, personality change, focal neuro signs
o Midline → visual field loss (bitemporal hemianopia), pituitary failure (↓ growth, diabetes insipidius, weight gain)
o Cerebellar → truncal ataxia, co-ordination difficulties, abnormal eye movements (new onset squint), scannign speech

o Brainstem → CN defects, pyramidal tract signs, cerebellar signs
o Spinal tumours → back pain, peripheral weakness, bladder or bowel dysfunction

60
Q

how are CNS tumours usually ivx?

A
  • MRI Scan
  • Biopsy, hearing tests, pituitary function tests, CSF analysis (raised beta-HCG and raised α-fetoprotein)

some tumours cause various pitiiutary hormone deficiencies so can screen for them

61
Q

how are CNS tumours usually mx?

A
  • Surgery is usually first line
  • Aim to TX hydrocephalus, provide tissue DX, attempt maximum resection
  • ± radiotherapy or chemotherapy depending on age and tumour type

depends on cause
craniopharyngioma can cause endocrine insufficiency eg hypothyroid - so will have to replace those

The whole MDT could get involved at some stage

o Support - Headstrong charity

62
Q

what are the cells of the brain?

A

Glial cells
Neuronal cells / neurons
Meninges

63
Q

which brain tumours arise from glial cells?

which brain tumours arise from neuronal cells?

A

Glial:
Astrocytoma (astrocytes) - most common
Ependymoma
Oligodendroglioma (rarer in kids)

Neuronal:
Medulloblastoma
PNET

64
Q

what are the most common paediatric brain tumours:

  1. Benign
  2. Malignant
A
Most common benign:
Pilocytic astrocytoma (Juvenile)

Most common malignant:
Medulloblastoma

both more common in males
benign one doesnt get radiation.

65
Q

what are prognostic factors in brain tumours?

A

Age
- the younger @ diagnosis the worse the outcomes - sometimes

Tumour histology

Metasteses

Histology

Response to treatment

66
Q

what are some complications of surgical resection in tumours?

A

Hydrocephalus

  • attention deficitis
  • issues with processing speed

Cerebellar mutism aka posterior fossa syndrome

  • period of mutism
  • can have ataxia, irritability, hypotonia
  • can persist for 1 year
  • worse if mutliple surgeries

Chemotherapy

  • Cisplatin: ototoxicity - ear
  • Vincristine: fine motor deficit

Radiotherapy - gradual decline in

  • IQ, academic ability
  • attention
67
Q

which brain tumour often presents with obstructive hydrocephalus (as well as nausea,vomiting and increased ICP)? why?

A
  1. Ependymoma
    - becasue it arises from the 4th ventricle -> can block outflow of CSF
  2. Craniopharyngioma
    - blocks 3rd ventricle
    - compresses optic chiasm too
68
Q

where does neuroblastoma most commonly arise?

A

Abdomen with abdominal pain

69
Q

which tumour presents with paraneoplastic symptoms eg

secretory diiarrheoa
increased sweating
HTN
Dancing eyes and feet (opsoclonus, myoclonus)

A

neuroblastoma

70
Q

in what other ways can neuroblastoma present?

A

if spinal mets -> paraplegia

if thoracic mets -> horners syndrome
Lump in chest

Bone -> bone pain
Cytopaenia -> bone marrow infiltrate
Orbital proptosis and ecchymosis -> Raccoon eyes
“bruising around eye”

Bluish subcutaneous nodules

71
Q

what might ivx show in nueroblastoma?

A

CT/MRI - calcifications

Tumour markers:
Urine Homovanellic acid (HNA)
Vanillymandelic acid (VNA)

N-myc -> poor prognosis if present

72
Q

List risk factors for neuroblastoma?

A

Hirschprung
Fetal alcohol syndrome
Phaechromo

73
Q

mx of neuroblastoma?

A

Chemotherapy
Radiotherapy
Stem cell transplant
Retinoic acid

74
Q

what are the types of hydrocephalus?

A

Communicating hydrocephalus, aka nonobstructive hydrocephalus, is caused by impaired CSF REABSORPTION
There is NO obstruction.
- includes normal pressure hydrocephalus

Non-communicating:
caused by obstruction in ventricles or nearby foramen

75
Q

what are the causes of hydrocephalus?

A

Communicating:
subarachnoid/intraventricular hemorrhage,
meningitis,
congenital absence of arachnoid villi

Non-communicating:
▪Genetic
▪ Congenital malformations* can lead to narrowing/atresia  of the outflow foramen. (e.g. congenital aqueduct stenosis)
▪Obstructing tumour or cyst 
*eg Chiari malformation 

tumours and masses

76
Q

what is the presentation and signs + sx of hydrocephalus?

A
  • Acute: vomiting, irritable, impaired consciousness
  • Chronic: FTT, dev delays

poor feeding, and frequent vomiting.

• Signs/Symptoms:
o ↑ head circumference (rapidly increasing)
o Tense /bulging anterior + posterior fontanelle
o Sunset sign - eyes turn downwards
o Increased tone
o Papillodema
o Ataxic gait
o 6th nerve palsy – medial deviation
77
Q

how do we ivx hydrocephalus and what are some of the findings?

A

• Cranial US (1st line in infants) – monitor head circumference

• CT head
- enlargement of the ventricles (however this is not present in every case of hydrocephalus).

  • loss of the sulcal gyral pattern and CSF exudation from the ventricles (appearing as a subtle hypodensity tracking around the ventricular margins).

Generalised ventricular dilatation suggests a communicating hydrocephalus

78
Q

how do we mx hydrocephalus

A

• Neurosurgery referral

In the ACUTE setting:
1st line -> Insert EVD (external ventricular drain)

Adjunct -> Furosemide - Inhibit CSF (not for long term)

Definitive rx (depends on cause):
o VPS - Ventriculo-peritoneal  shunts or ventriculo-atrial (VA) shunts
-> long-term solution to reduce CSF pressures 

o Tumour removal
o choroid plexus resection etc

• Long term – yearly check-ups for shunt, look out for learning or developmental difficulties

79
Q

what is a complication of VPS therapy in hydrochephalus?

A

infection can happen by coag-neg staphylococcus - CONS eeg s. saprophyticus

if so vancomycin covers this

80
Q

what is the aetiology of Neurofibromatosis?

A

Neurofibromatosis causes tumours to grow along nerves. The tumours are usually benign but may cause a range of symptoms. Neurofibromatosis type 1 is MORE common than NF2.

Type 1:
• Auto Dom, high penetrance
• Wide spectrum – mild to severe 
• Mutation of NF1 gene on chromosome 17
  - tumour suppressor gene

Type 2:
• Mutation of NF2 gene on chromosome 22

81
Q

what is the presentation of Neurofibromatosis NF1?

A

NF1

2+ needed for diagnosis:
• More than 6 café au lait spots, >5mm in size pre-puberty, >15mm in size post-puberty

• >1 neurofibroma – firm nodular overgrowth of nerve

  • > note: as they get older, they increase in number!
  • once they appear they dont go
  • Axillary freckles
  • Optic glioma +/- visual impairment
  • One Lisch nodule – hamartoma of iris (on slit-lamp exam)
  • Bony lesions from spheroid dysplasia +/- eye protrusion
  • First degree relative with NF1
82
Q

what is the presentation of Neurofibromatosis NF2?

A

NF2:
presents in adolescence,
bilateral acoustic neuroma aka vestibular schwanomma +/- bilateral sensorineural deafness,
cerebellopontine angle syndrome with facial nerve paresis and cerebellar ataxia
Cataracts
Meningioma - psammoma bodies on histology
Ependymoma

83
Q

Patient has polyendocrinopathy - eg lots of endocrine conditions/ presentations and hash cafe au lait spots. diagnosis?

A

Mccune albright

84
Q

how do we treat Neurofibromatosis ?

A

It is recommended that children diagnosed with NF1 at an early age have an examination each year, which allows any potential growths or changes related to the disorder to be monitored

If tumour involving optic tract - consider chemotherapy

Monitor masses for malignancy - 1/3 patients malignancies:

Consider surgical resection of tumours - can cause nerve damage / movement disorders

85
Q

what is tuberous sclerosis and its aetiology?

A

Serious condition

Dominantly inherited (1/3rd)

  • TSC 1 ts gene mutation -> Chr 9
  • TSC 2 ts gene mutation -> Chr16
  • Need 2 hits, so condition has variable expressivity of symptoms
  • 2/3rds new mutations

Rare

Benign tumours develop in different parts of the body

86
Q

what is the presentation of Tuberous sclerosis?

A

Depigmented ash leaf shaped patches which fluoresce under UV light – Wood’s light

  • Roughened patches of skin (shagreen patches) usually over lumbar spine
  • Adenoma sebaceum (angiofibromata) in butterfly distribution over bridge of nose/cheeks

• Often develop West syndrome:
Infantile spasms + altered EEG + mental changes

  • Seizures - due to tumours in brain calcifying at eg 4months+
  • Developmental delay, intellectual impairment, learning difficulties +/- autistic features, ADHD
  • Epilepsy – often focal

Also → subungual fibromata, dense white areas on retina, rhabdomyomata of heart, polycystic kidneys

You MAY get Harmatoma’s,

  • Angiofibroma’s (skin),
  • Cardiac rhabdomyomas (tumours of cardiac muscle) -> may see on ANTENATAL US. Can cause mitral valve prolapse
  • Renal angiomyolipoma’s - tumours of kidney
87
Q

What are the findings on ivx of Tuberous sclerosis?

A

• CT scan –

detect calcified subependymal nodules and tubers from 2+ years

• MRI more sensitive and clearly identified other tubers and lesions

88
Q

how do we manage tuberous sclerosis?

A

depends where the tumours develop and what sx come up:

Infantile spasms - Prednisolone, Vigabtrin +- ACTH

Mx for tumours eg perhaps surgery for Astrocytoma

89
Q

what is STURGE-WEBER SYNDROME?

A

Sporadic disorder →

Port wine stain / hemangioma in the Ophthalmic division of trigeminal nerve associated with similar lesion intracranially (Lepto-meningeal angioma / capillary malformation)

Presents with epilepsy, learning disability +/- hemiplegia

later down the line - vision loss

On CT/ head scan:
- ‘tram-track’ calcifications

90
Q

what is the aetiology of retinoblastoma? presentation?

A

Aetiology
o Retinoblastoma susceptibility gene is on Chr 13, pattern of inheritance is
dominant, with incomplete penetrance Clinically

Presentation:
• White pupillary
reflex is noted to replace red one or comes in with a squint

91
Q

mx of retinoblastoma?

A

Ivx:
• MRI and examination under anaesthetic
• Tumours are frequently multifocal

Management
• Chemo used, especially if bilateral, to shrink tumour followed by laser treatment to retina

  • Radiotherapy may be used in advance disease, often reserved for treatment of recurrence
  • Most patients cured, some may be visually impaired
  • Eunucleation of eye may be necessary in more advanced disease
92
Q

what eye disease are premature infants at risk of?

mx?

A

RETINOPATHY OF PREMATURITY

  • Vascular proliferation → retinal detachment → fibrosis → blindness
  • Risk is increased by uncontrolled use of high concentrations of oxygen

can cause Severe bilateral visual impairment in 1%

Mx: Laser eye treatment

93
Q

what is strabismus and what are the causes?

A

Whereas normally both eyes fixate (look at) the object of interest, in strabismus one eye fixates and the other (non-fixating eye) is deviated.

  1. Non-paralytic (refractive error in one or more eye)
  2. Paralytic
    (squinting eye could be caused by motor nerve paralysis or SOL)
94
Q

how may strabismus present?

A

Aesthetically - can see defect

Common cause of diplopia (double vision) and visual confusion (seeing different objects in the same place) in adults.

Important cause for amblyopia (decreased vision in an anatomically normal eye)

Abnormal eye movements

long/short sighted

95
Q

what is the ongoing management of Epilepsy / seizures? what info do we need to know?

A

Information:
What type of seizures
Age of patient, Gender (eg no valproate for teen girls)
Weight
Epilepsy syndromes (eg benign rolandic epilepsy) / non-syndrome:

You choose meds based on all this information

Drugs:
We dont use benzodiazepines long term because they are Addictive and are Sedatives!

Instead we use:
1st line drugs:
Carbemezapine
Sodium Valproate - not in females in puberty
Lamotrigine 

Vigabatrin - 1st in infantile spams
Ethosuximide - useful in absence seizures

  • We ideally want mono-therapy - as all the meds have serious side effects - parents must monitor actively for these.
  • prescribed according to weight - hepatotoxicity risk with polytherapy!!

If infrequent seizures, we may not give any drugs.

-> Advice to give those with new diagnosis

96
Q

What is the basic protocol for management of any seizure?

A

In Community:

A. Danger - make sure kid is safe and not going to hurt themselves.
B. Set a TIMER for seizure
C. Can video RECORD if first episode
D. Call an AMBULANCE >5mins
E. Put in recovery position after seizure
- so dont aspirate vomit
- dont try and stop the seizure or hold them

Hospital:

  1. Call for help
  2. If seizing for more than 5 mins -> drug protocol: 1st - IV Lorazepam etc etc

note: 1st 2 doses are benzodiazepines -> whether buccal/rectal or IV! so you dont give IV 2x if you have already given bucaal 1x.

97
Q

what are the NICE criteria for SIMPLE febrile seizures?

A

The seizure usually lasts 2–3 minutes, and rarely lasts more than 10 minutes.

The seizure is a generalized tonic-clonic type

There may be foaming at the mouth, difficulty breathing, pallor, or cyanosis.

A brief post-ictal period of drowsiness, irritability, or confusion, with complete recovery within 1 hour.

The child may have had a previous febrile seizure.

98
Q

what are the featues of a COMPLEX febrile seizure?

A

A partial onset or focal features (movement limited to one side of the body or one limb).

The seizure lasts more than 15 minutes.

There is seizure recurrence within 24 hours or within the same febrile illness.

There is incomplete recovery within 1 hour, and there may be prolonged post-ictal drowsiness or transient hemiparesis (Todd’s palsy).

complex ones associated iwth increased future epilepsy risk

99
Q

what are the ivx for epilepsy - and how do we diagnose it?

A

Clinical diagnosis really - from hx of events before during and after

Supportive ivx:
EEG - can do a sleep EEG or use photo stimulation to pick up abnormalities better

All your same ivx above eg:
FBC + Infection screen if suspecting - including LP
Glucose etc

100
Q

what is benign rolandic epilepsy ?

A

Childhood Epilepsy with Centrotemporal Spikes aka Benign Rolandic Epilepsy

Seizures that come on when the child is awake involve twitching, numbness, or tingling of one side of the child’s face or tongue without impaired awareness (called a focal aware seizure).

Seizures that come on during sleep often evolve to convulsive activity affecting both sides of the body.

Because the seizures may be infrequent and usually occur at night, many children do not need daily antiseizure medication.

In almost every case, seizures stop on their own by adolescence or 4 years affter onset.
Can gie meds if daytime/frequent seizures.

EEG: spikes in the centro-temporal region of the brain

101
Q

what is spina bifida and what are the most common forms?

A

congenital anomaly; neural tube defect

Spina bifida is when a baby’s spine and spinal cord does not develop properly in the womb, causing a gap in the spine

There are several different types of spina bifida, including:

myelomeningocele – the most severe type of spina bifida; the baby’s spinal canal remains open along several vertebrae in the back, allowing the spinal cord and protective membranes around it to push out and form a sac in the baby’s back. can leak csf. infection risk

meningocele – another serious type where the meninges) push out through the spine; the spinal cord usually develops normally so surgery can often be used to remove the membranes without damaging the nerves

spina bifida occulta – the most common and mildest type of spina bifida; 1 or more vertebrae does not form properly, but the gap in the spine is very small; spina bifida occulta does not usually cause any problems and most people are unaware they have it

102
Q

according to NICE what is 1st line treatment / rx in first presentation of seizure which has been confirmed with EEG activity etc that its epilepsy?

A
  1. Carbamazepine or lamotrigine as first-line
    - to children, young people and adults with newly diagnosed focal seizures
  2. Offer levetiracetam or oxcarbazepine
    - to women and girls of childbearing potential if carbamazepine or lamotrigine are unsuitable or not tolerated for newly diagnosed focal seizures.

DO NOT give sodium valproate to women/girls of cb age