Paediatric Neurology Flashcards

1
Q

Prodrome for syncope

A

Hot, clammy or sweaty
Dizzy
Vision going blurry
Headache

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2
Q

Primary syncope causes

A

Dehydration
Missed meals
Extended standing
Stimulus - surprise, pain or sight of blood

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3
Q

Secondary causes of syncope

A
Hypoglycaemia 
Dehydration 
Anaemia 
Infection 
Anaphylaxis
Arrhythmias
Valvular heart disease 
Hypertrophic obstructive cardiomyopathy
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4
Q

Investigations for syncope

A

ECG or 24hr ECG if paroxysmal arrythmia
Echocardiogram
Bloods - FBC, electrolytes and blood glucose

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5
Q

Epilepsy pathophysiology

A

Tendency to have seizures in which there is abnormal electrical activity

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6
Q

Generalised tonic clonic seizures

A

Loss of consiousness
Tonic - muscle tensing
Clonic - muscle jerking

Associated symptoms: 
- tongue biting 
- incontinence 
- irregular breathing 
-
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7
Q

Post ictal period

A

Period after the seizure where the patient is confused, drowsy and irritable which lasts a while

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8
Q

Management of a generalised tonic clonic seizure

A

First line - sodium valproate

Second line - lamotrigine or carbamazepine

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9
Q

Focal seizure features

A
  • start in temporal lobes
  • affect hearing, speech, memory and emotions

Associated features:

  • hallucinations
  • memory flashbacks
  • Deja vu
  • doing strange things on auto pilot
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10
Q

Management of focal seizures

A

First line: lamotrigine ot carbamazepine

2nd line: sodium valproate or levetiracetam

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11
Q

Absence seizure features

A

Patient becomes blank, stares into space and wont respond then abruptly returns to normal

Lasts 10 to 20 seconds

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12
Q

Managment of absence seizures

A

First line: sodium valproate or ethosuximide

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13
Q

Atonic seizure pathophysiology

A

Drop attacks - brief lapses in muscle tone

Lasts for less than 3 minutes

May indicate lennox gastaut syndrome

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14
Q

Atonic seizure management

A

1st line - sodium valproate

2nd line - lamotrigine

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15
Q

Myoclonic seizure pathophysiology

A

Sudden brief muscle contractions like a sudden jump when awake

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16
Q

Management of myoclonic seizures

A

1st line: sodium valproate

2nd line: lamotrigine, levetiracetam or topiramate

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17
Q

West syndrome pathophysiology

A

Infantile spasms starting at 6 months

Clusters of full body spasms

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18
Q

Treatment of infantile spasms

A

Prednisolone and vigabatrin

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19
Q

Features of febrile convulsions

A
  • Occur in children aged 6 months to 6 years
  • Seizures due to fever
  • Paracetamol will not decrease the number of seizures
  • < 15 mins - simple
  • > 15 mins, multiple or focal - complex
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20
Q

Investigations for epilepsy

A

EEG - after second simple tonic clonic seizure

MRI - rule our tumours, encephalopathy

Bloods - electrolytes

Blood glucose - hypoglycaemia

Blood, urine cultures and LP - if meningitis, encephalitis or sepsis is suspected

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21
Q

General advice for seizures

A
Take showers rather than baths 
Cautious about swimming 
Caution with heights 
Caution with traffic 
Caution with heavy, hot or electrical equipment
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22
Q

Side effects of sodium valproate

A

Teratogenic - contraception advice
Liver damage and hepatitis
Hair loss
Tremors

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23
Q

Side effects of carbamazepine

A

Agranulocytosis
Aplastic anaemia
P450 inducer

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24
Q

Side effects of phenytoin

A

Folate and vitamin D deficiency - megaloblastic anaemia and osteomalacia

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25
Q

Side effects of ethosuximide

A

Night tremors

Rashes

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26
Q

Side effects of lamotrigine

A

Steven - Johnson syndrome

Leukopenia

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27
Q

Management of seizures in general

A

Recovery position or safe position
Put something soft under their head
Remove potential hazards
Make a note of the time and end of seizure
Call an ambulance if it lasts > 5 minutes

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28
Q

Status epilepticus

A

Seizure lasting for more than 5 mins

2+ seizures without regaining consciousness in the interim

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29
Q

Management of status epilepticus in the hospital

A

A-E - high flow oxygen

IV lorazepam - repeated after 10 mins if seizures continue

If seizure persists - IV phenobarbital or phenytoin

30
Q

Medical management for seizures in the community

A

Buccal midazolam

Rectal diazepam

31
Q

Reflex anoxic seizures

A

Occurs when child is startled as vagus nerve sends signals to heart to transiently stop beating

Child goes pale, loses consciousness and may have muscle twitching

Regains consciousness in 30 seconds

32
Q

Causes of headaches in children

A

Primary:

  • tension headache
  • migraine
  • cluster headache

Secondary:

  • sinusitis
  • SOL
  • meningitis or encephalitis
  • carbon monoxide poisoning
  • ENT infection
  • problems with vision
33
Q

Features of tension headaches

A

Site - band like pattern around the head, symmetrical

Onset - gradual

Character - ache

Relieving factors - analgesia, hydration

Aggravating factors - dehydration, stress, skipping meals and infection

Timing - approx 30 mins

Associated features - pale, tired

34
Q

Features of migraines

A

Site - unilateral, often forehead or eyes

Onset - can be gradual or sudden

Character - sharp, throbbing

Relieving factors - dark room, rest, analgesia (triptans)

Aggrevating factors - stress, solvents, dehydration

Timing - takes longer to resolve

Associated features - photophobia, aura, nausea, abdo pain

35
Q

Prophylaxis for migraines and when to use it

A

Propranolol
Pizotifen
Topiramate

Significantly impacting life
Frequent - > 1 per week

36
Q

Cerebral palsy

A

Permanent neurological problems resulting from damage to the brain around the time of birth

37
Q

Causes of cerebral palsy

A

Antenatal:

  • maternal infections
  • trauma during pregnancy

Perinatal:

  • birth asphyxia
  • preterm

Post-natal:

  • meningitis
  • severe neonatal jaundice
  • head injury
38
Q

Types of cerebral palsy

A

Spastic - hypertonia and reduced function

Dyskinetic - hypertonia and hypotonia causing athetoid movement and oro-motor problems

Ataxic - problems with coordinated movements

Mixed

39
Q

What causes spastic, dyskinetic and ataxic cerbral palsy

A

Spastic - damage to the UMNs

Dyskinetic - damage to the basal ganglia

Ataxic - damage to the cerebellum

40
Q

Monoplegia

A

One limb affected

41
Q

Hemiplegia

A

One side of the body affected

42
Q

Diplegia

A

4 limbs are affected but mostly the legs

43
Q

Quadriplegia

A

4 limbs affected more severely

Often have seizures, speech disturbance and other impairments

44
Q

Signs of cerebral palsy

A
  • failure to meet developmental milestones
  • prefence of 1 hand before the age of 18 months
  • hypertonia or hypotonia
  • problems with speech, coordination and walking
  • feeding or swallowing problems
  • learning difficulties
45
Q

Cerebral palsy neurological exam

A

Hemiplagia or diplegia - UMN damage
Broad based gait/ataxic - cerebellar lesion
High stepping gait - LMN damage causing foot drop

46
Q

Complications associated with cerebral palsy

A
Learning disability 
Epilepsy 
Kyphoscoliosis 
GORD
Muscle contractures 
Hearing and visual impairments
47
Q

Management of cerebral palsy

A

MDT:

  • physiotherapy
  • dieticians
  • occupational health
  • speech and language therapy - may need NGT/PEG
  • social worker
48
Q

Medications for cerebral palsy

A

Glycopyrronium bromide - excessive drooling
Anti - epileptic drugs
Muscle relaxants e.g. baclofen

49
Q

Strabismus

A

Misalignment of the eyes causing double vision

50
Q

Hydrocephalus pathophysiology

A

Accumulation of CSF within the brain and spinal cord due to eith over production or impaired clearance

51
Q

Where is CSF created?

A

Choroid plexus

52
Q

Where is CSF absorbed?

A

Arachnoid granulations

53
Q

Causes of hydrocephalus

A

Increased production

Decreased absorption:

  • aqueductal stenosis
  • arachnoid cysts
  • coning (arnold - chiari malformation) - cerebellum herniates through the foramen magnum
54
Q

Presentation of hydrocephalus

A

Bulging of the anterior fontanelle
Poor feeding and vomiting
Poor tone
Sleep disturbance

55
Q

Treatment of hydrocephalus

A

VP shunt

56
Q

Complications of craniosynostosis

A

Raised intracranial pressure:

  • developmental delay
  • cognitive impairment
  • vomiting
  • visual defects
  • seizures
  • neurological symptoms
57
Q

Muscular dystrophy pathophysiology

A

Gradual weakening and wasting of muscles due to genetical conditions

58
Q

Gower’s sign

A

Children with proximal muscle weakness stand up by first getting on their hands and knees, pushing their hips up and backwards then shifting their weight back and putting hands on knees

Indicative of Duchennes muscular dystrophy

59
Q

Inheritamce pattern on Duchennes muscular dystrophy

A

X linked recessive

60
Q

Management of muscular dystrophy

A

MDT:

  • physiotherapy
  • occupational therapy
  • medical appliance use
  • surgical and medical input - spinal scoliosis and HF
61
Q

Duchennes muscular dystrophy

A

Defective gene for dystrophin

Present at 3 - 5 yo with proximal muscle weakness of their pelvis - Gower’s sign

Wheelchair bound by teenage years

62
Q

How to slow down the development of Duchennes dystrophy

A

Oral steroids

Creatine supplements

63
Q

When does Beckers muscular dystrophy present

A

8 - 12 yo

64
Q

Features of myotonic muscular dystrophy

A

Normally presents in adulthood with:

  • progressive muscle weakness
  • prolonged muscle contractions
  • cataracts
  • cardiac arrhythmia
65
Q

Facioscapulohumeral muscular dystrophy

A

Affects facial muscles and then progresses to the shoulders and arms

  • sleep with eyes open and weakness in pursing lips and blowing cheeks out
66
Q

Oculopharyngeal muscular dystrophy presentation

A

Bilateral ptosis, restricted eye movements and swallowing difficulties

67
Q

Emery dreifuss muscular dystrophy

A

Contractures affecting elbows and ankles commonly during childhood

68
Q

Pathophysiology of spinal muscular atrophy

A

Autosomal recessive condition causing progressive loss of motor neurones causing progressive muscular weakness

Affects LMNs in the spinal cord

69
Q

Signs of spinal muscular atrophy

A
Fasciculations  
Muscle atrophy 
Hypotonia 
Reduced power 
Reduced or absent reflexes
70
Q

Types of spinal muscular atrophy

A

SMA 1 - onset within few months of birth, normally die within 2 years

SMA 2 - onset within first 18 months, cant walk but survive to adulthood

SMA 3 - onset after 12 months old, walk with support but eventually lose ability

SMA 4 - onset in 20 yos - can walk short distances

71
Q

Management of spinal muscular atrophy

A

MDT

  • physiotherapy
  • occupational therapy
  • respiratory support
  • PEG may be required