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
Side effects of ethosuximide
Night tremors | Rashes
26
Side effects of lamotrigine
Steven - Johnson syndrome | Leukopenia
27
Management of seizures in general
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
28
Status epilepticus
Seizure lasting for more than 5 mins 2+ seizures without regaining consciousness in the interim
29
Management of status epilepticus in the hospital
A-E - high flow oxygen IV lorazepam - repeated after 10 mins if seizures continue If seizure persists - IV phenobarbital or phenytoin
30
Medical management for seizures in the community
Buccal midazolam | Rectal diazepam
31
Reflex anoxic seizures
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
Causes of headaches in children
Primary: - tension headache - migraine - cluster headache Secondary: - sinusitis - SOL - meningitis or encephalitis - carbon monoxide poisoning - ENT infection - problems with vision
33
Features of tension headaches
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
Features of migraines
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
Prophylaxis for migraines and when to use it
Propranolol Pizotifen Topiramate Significantly impacting life Frequent - > 1 per week
36
Cerebral palsy
Permanent neurological problems resulting from damage to the brain around the time of birth
37
Causes of cerebral palsy
Antenatal: - maternal infections - trauma during pregnancy Perinatal: - birth asphyxia - preterm Post-natal: - meningitis - severe neonatal jaundice - head injury
38
Types of cerebral palsy
Spastic - hypertonia and reduced function Dyskinetic - hypertonia and hypotonia causing athetoid movement and oro-motor problems Ataxic - problems with coordinated movements Mixed
39
What causes spastic, dyskinetic and ataxic cerbral palsy
Spastic - damage to the UMNs Dyskinetic - damage to the basal ganglia Ataxic - damage to the cerebellum
40
Monoplegia
One limb affected
41
Hemiplegia
One side of the body affected
42
Diplegia
4 limbs are affected but mostly the legs
43
Quadriplegia
4 limbs affected more severely Often have seizures, speech disturbance and other impairments
44
Signs of cerebral palsy
- 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
Cerebral palsy neurological exam
Hemiplagia or diplegia - UMN damage Broad based gait/ataxic - cerebellar lesion High stepping gait - LMN damage causing foot drop
46
Complications associated with cerebral palsy
``` Learning disability Epilepsy Kyphoscoliosis GORD Muscle contractures Hearing and visual impairments ```
47
Management of cerebral palsy
MDT: - physiotherapy - dieticians - occupational health - speech and language therapy - may need NGT/PEG - social worker
48
Medications for cerebral palsy
Glycopyrronium bromide - excessive drooling Anti - epileptic drugs Muscle relaxants e.g. baclofen
49
Strabismus
Misalignment of the eyes causing double vision
50
Hydrocephalus pathophysiology
Accumulation of CSF within the brain and spinal cord due to eith over production or impaired clearance
51
Where is CSF created?
Choroid plexus
52
Where is CSF absorbed?
Arachnoid granulations
53
Causes of hydrocephalus
Increased production Decreased absorption: - aqueductal stenosis - arachnoid cysts - coning (arnold - chiari malformation) - cerebellum herniates through the foramen magnum
54
Presentation of hydrocephalus
Bulging of the anterior fontanelle Poor feeding and vomiting Poor tone Sleep disturbance
55
Treatment of hydrocephalus
VP shunt
56
Complications of craniosynostosis
Raised intracranial pressure: - developmental delay - cognitive impairment - vomiting - visual defects - seizures - neurological symptoms
57
Muscular dystrophy pathophysiology
Gradual weakening and wasting of muscles due to genetical conditions
58
Gower’s sign
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
Inheritamce pattern on Duchennes muscular dystrophy
X linked recessive
60
Management of muscular dystrophy
MDT: - physiotherapy - occupational therapy - medical appliance use - surgical and medical input - spinal scoliosis and HF
61
Duchennes muscular dystrophy
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
How to slow down the development of Duchennes dystrophy
Oral steroids | Creatine supplements
63
When does Beckers muscular dystrophy present
8 - 12 yo
64
Features of myotonic muscular dystrophy
Normally presents in adulthood with: - progressive muscle weakness - prolonged muscle contractions - cataracts - cardiac arrhythmia
65
Facioscapulohumeral muscular dystrophy
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
Oculopharyngeal muscular dystrophy presentation
Bilateral ptosis, restricted eye movements and swallowing difficulties
67
Emery dreifuss muscular dystrophy
Contractures affecting elbows and ankles commonly during childhood
68
Pathophysiology of spinal muscular atrophy
Autosomal recessive condition causing progressive loss of motor neurones causing progressive muscular weakness Affects LMNs in the spinal cord
69
Signs of spinal muscular atrophy
``` Fasciculations Muscle atrophy Hypotonia Reduced power Reduced or absent reflexes ```
70
Types of spinal muscular atrophy
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
Management of spinal muscular atrophy
MDT - physiotherapy - occupational therapy - respiratory support - PEG may be required