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Y4 paediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Seizures & epilepsy

A
  • seizures = paroxysmal abnormality of motor, sensory, autonomic and/or cognitive function due to transient brain dysfunction
  • epilepsy = excessive and hypersynchronous electrical activity, typically in neural networks in all or part of the cerebral cortex
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2
Q

Epilepsy causes

A

Genetic

Structural, metabolic - cerebral damage, cerebral tumour, neurodegenerative disorders, cerebral vascular occlusion,

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3
Q

Acute symptomatic seizures

A

Due to any cortical brain injury or insult, at the time of the trauma/illness

  • stroke, traumatic brain injury, intracranial infection
  • hypoglycaemia, hypocalcaemia, hypomagnesaemia, hyponatraemia/hypernatraemia
  • poisons/toxins
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4
Q

Febrile seizures

A

Epileptic seizures accompanied by a fever in the absence of intracranial infection

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5
Q

Non-epileptic seizures

A

Convulsive syncope - cardiac syncope, neurally mediated syncope, expiratory apnoea syncope

Sudden rise in intracranial pressure

Sleep disorders

Functional/medically unexplained

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6
Q

Floppy infant

A
  • can be used to describe:
    • decreased muscle tone (hypotonia)
    • decreased muscle power (weakness)
    • ligamentous laxity and increased range of joint mobility
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7
Q

Hypotonia

A
  • hypotonia - congenital hypotonia is defined as a subjective decrease of resistance to passive range of motion around the joint in a newborn
    • causes:
      • hypoxic ischaemic encephalopathy
      • intracranial haemorrhage
      • cerebral malformations
      • congenital infections
      • spinal muscular atrophy
      • infant botulism
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8
Q

Floppy infant ix

A
  • bloods: FBC, U&Es, LFTs, bone profile, magnesium, blood gas, ammonia, glucose
  • cranial USS
  • consider a septic screen
  • consider plasma amino acids and organic acids & urine organic acids → metabolic disorder
  • CK
  • consider screening to investigate for SMA
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9
Q

Floppy infant mx

A
  • depends on the cause of hypotonia
  • some babies that have severe illness will need respiratory support & feeding support
  • MDT approach is required no matter the cause
    • OT
    • PT
    • SALT
    • dietician
    • likely neurology involvement
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10
Q

Headache

A

Primary - four main groups (due to a primary malfunction of neurons and their networks)

  • migraine
  • tension-type headache
  • cluster headache
  • other primary headaches - eg. primary stabbing headache

Secondary - symptomatic of some underlying pathology eg. space-occupying lesions, raised ICP

Trigeminal & other cranial neuralgias & other headaches → root pain from herpes zoster

Only investigate if any red flag features

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11
Q

Headache red flag symptoms

A

Worse lying down/coughing/straining

Wakes up child

Associated confusion and/or morning or persistent N&V

Recent change in personality, behaviour or educational performance

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12
Q

Headache red flag signs

A

Growth failure

Visual field defects (craniopharyngioma)

Squint

Cranial nerve abnormality

Torticollis (neck spasms & twists to side)

Abnormal coordination

Gait

Papilloedema

Bradycardia

Cranial bruits

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13
Q

Generalised tonic-clonic seizures

A
  • loss of consciousness
  • tonic (muscle tensing) and clonic (muscle jerking) movements
  • typically tonic before clonic
  • may be associated tongue biting, incontinence, groaning & irregular breathing
  • after → prolonged post-ictal period where person is confused, drowsy & feels irritable/low
  • management:
    • first line: sodium valproate (except girls > 10)
    • second line: levetiracetam/lamotrigine (first line for girls > 10)
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14
Q

Focal seizures

A
  • start in the temporal lobes
  • affect hearing, speech, memory & emotions
  • various ways that focal seizures can present:
    • hallucinations
    • memory flashbacks
    • deja vu
    • doing strange things on autopilot
  • management:
    • first line: lamotrigine or levetiracetam
    • second line: carbamazepine or oxcarbozepine or zonisamide
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15
Q

Absence seizure

A
  • typically happen in children
  • patient becomes blank, stares into space & abruptly returns to normal
  • during the episode, they are unaware of their surroundings & won’t respond
  • 10-20 seconds
  • management:
    • first line: ethosuximide
    • second line: sodium valproate
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16
Q

Atonic seizures (drop attacks)

A
  • atonic seizures (drop attacks)
    • characterised by brief lapses in muscle tone
    • don’t usually last more than 3 minutes
    • typically begin in childhood
    • may be indicative of Lennox-Gastaut syndrome
    • management:
      • first line: sodium valproate
      • second line: lamotrigine
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17
Q

Myoclonic seizures

A
  • present as sudden brief muscle contractions
  • usually remain awake
  • management:
    • first line: sodium valproate or levetiracetam
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18
Q

Infantile spasms

A
  • characterised by clusters of full body spasms
  • poor prognosis (1/3 die by age 25), 1/3 are seizure free
  • management:
    • first line: prednisolone & vigabatrin
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19
Q

Febrile convulsions

A
  • seizures that occur in children whilst they have a fever
  • only occur in children between the ages of 6 months and 5 years
  • slightly increases risk of developing epilepsy in the future
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20
Q

Epilepsy ix

A
  • EEG can show typical patterns in different forms of epilepsy & support the diagnosis
    • perform after the second simple tonic-clonic seizure
  • MRI brain
    • first seizure < 2 years
    • focal seizures
    • no response to first line anti-epileptic medications
  • additional investigations
    • ECG
    • blood electrolytes
    • blood glucose
    • blood cultures, urine cultures & LP
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21
Q

Epilepsy mx

A
  • general advice
    • take showers rather than baths
    • be very cautious swimming
    • be cautious with heights
    • be cautious with traffic
    • be cautious with any heavy, hot or electrical equipment
    • avoid driving until meet criteria
  • medication
  • seizures
    • put patient in a safe position
    • recovery position, something soft under head
    • remove obstacles that could lead to injury
    • make a note of start & end time of seizure
    • call an ambulance if lasting > 5 minutes or first seizure
22
Q

Status epilepticus

A
  • seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness
  • management in hospital:
    • secure airway
    • high concentration oxygen
    • assess cardiac & respiratory function
    • check blood glucose levels
    • gain IV access
    • IV lorazepam, repeat after 10 minutes if seizure continues
    • seizures persist after final step → IV phenobarbital/phenytoin
    • medical options in the community → buccal midazolam or rectal diazepam
23
Q

Extradural haemorrhage

A
  • arterial/venous bleeding into the extradural space
  • usually follows direct head trauma, often associated with skull fracture
    • tearing of the middle meningeal artery as it passes through the foramen spinosum of the sphenoid bone
  • diagnosis confirmed by CT scan
24
Q

Extradural haemorrhage presentation

A

Lucid interval

Seizures secondary to increasing size of the haematoma

Focal neurological signs with dilatation of the ipsilateral pupil, paresis of the contralateral limbs & false localising unilateral/bilateral sixth nerve palsies

Young children - anaemia & shock

25
Q

Extradural haemorrhage mx

A

Correct hypovolaemia

Urgent evacuation of the haematoma and arrest of the bleeding

26
Q

Migraine without aura

A

Accounts for 90% of migraines

Presentation

  • bilateral headache but may be unilateral
  • characteristically pulsatile over the temporal or frontal area
  • often accompanied by unpleasant GI disturbance - N&V, abdominal pain, photophobia & phonophobia
  • aggravated by physical activity
  • relieved by sleep
27
Q

Migraine with aura

A

Accounts for 10% of migraine

Headache is preceded by an aura (visual, sensory or motor)

Presentation

  • absence of problems between episodes
  • frequent presence of premonitory episodes
  • visual disturbance aura:
    • negative phenomena → hemianopia (loss of half the visual field) or scotoma (small areas of visual loss)
    • positive phenomena → fortification spectra (seeing zigzag lines)
  • unilateral sensory/motor symptoms = hemiplegic migraines
28
Q

Migraine mx

A

Advice & safety-netting

Rescue treatments

  • analgesia - paracetamol and NSAIDs
  • antiemetics - prochlorperazine or cyclizine
  • triptans - nasal preparation of this is particularly useful in children early in a migraine attack
  • physical treatments - cold compresses, warm pads, topical forehead balms

Prophylactic treatments (headaches are frequent and intrusive)

  • sodium channel blockers
  • beta-blockers eg. propranolol - contraindicated in asthma
  • tricyclics eg. pizotifen - can cause weight gain & sleepiness, amitriptyline - can cause dangerous arrhythmias in overdose
  • acupuncture

Psychological support

  • relaxation & other self-regulating techniques
  • addressing lifestyle issues
  • ensuring adequate and regular rest, play, water & food
29
Q

Muscular dystrophies

A

Genetic conditions that cause gradual weakening & wasting of muscles

30
Q

Muscular dystrophies types

A
  • Duchennes muscular dystrophy
  • Beckers muscular dystrophy
  • myotonic dystrophy
  • facioscapulohumeral muscular dystrophy
  • oculopharyngeal muscular dystrophy
  • limb-girdle muscular dystrophy
  • Emery-Dreifuss muscular dystrophy
31
Q

Gower’s sign

A
  • children with proximal muscle weakness use a specific technique to stand up from a lying position = Gower’s sign
  • to stand up, they got onto their hands and knees, then push their hips up and backwards
  • then, they shift their weight backwards & transfer their hands to their knees
  • whilst keeping their legs mostly straight, they walk their hands up their legs to get body erect
  • muscles around the pelvis are not strong enough to get their upper body erect without help of their arms
32
Q

Muscular dystrophies mx

A
  • no curative treatment
  • aimed at allowing the person to have highest quality of life
  • involves input from occupational therapy, physiotherapy & medical appliances
  • surgical & medical management of complications eg. spinal scoliosis & HF
33
Q

DMD

A
  • caused by defective gene for dystrophin on the X-chromosome
    • protein that helps hold muscles together at the cellular level
  • X-linked recessive condition
  • present around 3-5 years with weakness in the muscles around their pelvis
    • tends to be progressive & eventually all muscles are affected
    • wheelchair bound by the time they become a teenager
    • life expectancy of around 25-35 years with good management of cardiac & respiratory complications
  • oral steroids have shown to slow progression of muscle weakness, creatine supplementation can give a slight improvement in muscle strength
34
Q

Raised IP

A

Brain & ventricles are enclosed by a rigid skull, they have a limited ability to accommodate additional volume

Additional volume will therefore lead to a rise in ICP

35
Q

Raised IP causes

A

Idiopathic intracranial hypertension

Traumatic head injuries

Infection - meningitis

Tumours

Hydrocephalus

36
Q

Raised IP clinical features

A

Headache

Vomiting

Reduced levels of consciousness

Papilloedema

Cushing’s triad - widening pulse pressure, bradycardia, irregular breathing

37
Q

Raised IP ix & monitoring

A

Neuroimaging is key to investigate the underlying cause

Invasive ICP monitoring - catheter placed into the lateral ventricles of the brain to monitor the pressure

38
Q

Raised IP mx

A

Investigate & treat underlying cause

Head elevation to 30 degrees

IV mannitol

Controlled hyperventilation - aim is to reduce pCO2

Removal of CSF - drain from intraventricular monitor, repeated LP, ventriculoperitoneal shunt

39
Q

SMA

A

Rare, autosomal recessive condition that causes a progressive loss of motor neurones → progressive muscular weakness

Affects the lower motor neurones in the spinal cord

  • fasciculations
  • reduced muscle bulk
  • reduced tone
  • reduced power
  • reduced/absent reflexes
40
Q

SMA categories

A
  • 1, 2, 3 & 4 (2 most common)
  • SMA type 1 - onset in the first few months of life, usually progressing to death within 2 years
  • SMA type 2 - onset within the first 18 months
    • most never walk, but survive into adulthood
  • SMA type 3 - onset after first year of life
    • most walk without support, but subsequently lose that ability
    • life expectancy close to normal
  • SMA type 4 - onset in the 20s
    • most retain ability to walk short distances but require a wheelchair for mobility
    • everyday tasks can lead to significant fatigue
    • respiratory muscles & life expectancy are not affected
41
Q

SMA mx

A
  • supportive & involves the MDT
  • physiotherapy can be helpful maximising strength in the muscles & retaining respiratory function
  • respiratory support with NIV → may be required to prevent hypoventilation & respiratory failure
    • children with SMA type 1 may require a tracheostomy with mechanical ventilation → can dramatically extend life by supporting failing respiratory muscles
  • PEG feeding may be required when a weak swallow makes swallowing unsafe
42
Q

Subarachnoid haemorrhage presentation

A

Severe headache with rapid onset (’thunderclap headache’)

Vomiting

Confusion

Lowered level of consciousness

Seizures

Coma

43
Q

Subarachnoid haemorrhage ix

A

CT head - identifies blood in the CSF

LP occasionally

MR angiography, CT or conventional angiography (cause is often an aneurysm or arteriovenous malformation)

44
Q

Subarachnoid haemorrhage mx

A

Neurosurgery

Interventional radiology

45
Q

Subdural haemorrhage

A
  • results from tearing of the bridging veins as they cross the subdural space
  • a characteristic lesion in nonaccidental injury caused by shaking or direct trauma & toddlers
    • retinal haemorrhages are typical of shaking injury
  • occasionally seen following a fall from considerable height
  • rarely seen in association with brain shrinkage through atrophy or over drainage of hydrocephalus
46
Q

Tension headache

A

Form of episodic primary headache

Chronic tension-type headache - defined as a tension headache occur on 15 or more days per month

47
Q

Tension headache clinical features

A

‘tight band’ around the head/pressure sensation

Bilateral

Tends to be of a lower intensity than migraine

Not associated with aura, N&V or aggravated by routine physical activity

May be related to stress

48
Q

Tension headache mx

A

Acute mx - aspirin, paracetamol or an NSAID

Prophylaxis - up to 10 sessions of acupuncture over 5-8 weeks

Low-dose amitriptyline

49
Q

Homonymous hemianopia

A

Incongruous defects: lesion of optic tract

Congruous defects: lesion of optic radiation/occipital cortex

Macula sparing: lesion of occipital cortex

50
Q

Homonymous quadrantanopias

A

Superior - lesions of the inferior optic radiations in the temporal lobe

Inferior - lesion of the superior optic radiations in the parietal lobe

PITS

51
Q

Bitemporal hemianopia

A

Lesion of optic chiasm

Upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour

Lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma

Y4 paediatrics (8 decks)

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