Paediatric neurology Flashcards

1
Q

What is the epidemiology of headache disorders?

A

In about 40% children by age 7, 75% of children by age 15

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2
Q

What are the different types of headache patterns and which ones are investigated?

A
  • Isolated acute (investigated)
  • Recurrent acute
  • Chronic progressive (investigated)
  • Chronic non-progressive
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3
Q

What should be asked in the history of a child with a headache disorder?

A
  • Is there more than 1 type of headache?
  • Typical episode:
    □ Any warning?
    □ Location?
    ® Headaches located at the back of the head are more concerning
    □ Severity?
    □ Duration?
    □ Frequency?
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4
Q

What examination should be done on a child with headache disorder?

A
  • Growth parameters, OFC, BP
    □ The head growing too fast may mean excessive CSF and hydrocephalus
    □ If the child isn’t growing well then it could be a indication of a brain tumour
  • Sinuses, teeth, visual acuity
  • Fundoscopy
  • Visual fields (craniopharyngioma)
  • Cranial bruit
    □ Put a stethoscope on a child’s temporal area and listen for bruits
  • Focal neurological signs
  • Cognitive and emotional status
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5
Q

What is a primary headache?

A

Tension headache and migraine

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6
Q

What are the pointers to childhood migraine?

A
  • Associated abdominal pain, nausea, vomiting
  • Focal symptoms/ signs before, during, after attack: Visual disturbance, paraesthesia, weakness
  • ‘Pallor’
  • Aggravated by bright light/ noise
  • Relation to fatigue/ stress
  • Helped by sleep/ rest/ dark, quiet room
  • Family history often positive
  • Hemicranial pain
  • Throbbing/ pulsatile
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7
Q

What are the pointers to tension headache in children?

A
  • Diffuse symmetrical
  • Band like distribution
  • Present most of the time but there may be symptom free periods
  • “constant ache”
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8
Q

True or false: It is rare to have mixed migraine and tension type headache

A

False: they are common

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9
Q

What are the pointers to raised intercranial pressure in children?

A
  • Aggravated by activities that raise ICP e.g. Coughing, straining at stool, bending
  • Woken from sleep with headache
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10
Q

What are the pointers to analgesic overuse headache in children?

A
  • Headache is back before allowed to use another dose
  • Paracetamol/ NSAIDs
  • Particular problem with compound analgesics e.g. Cocodamol
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11
Q

What are the indications for neuroimmaging?

A
  • Features of cerebellar dysfunction
  • Features of raised intracranial pressure
  • New focal neurological deficit e.g. new squint
  • Seizures, esp. focal
  • Personality change
  • Unexplained deterioration of school work
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12
Q

What is the management of migraine in children?

A

□ Acute attack: effective pain relief, triptans

□ Preventative (at least 1/week): Pizotifen, Propranolol, Amitriptyline, Topiramate, Valproate

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13
Q

What is the management of tension type headache in children?

A
□ Aim at reassurance: no sinister cause
□ Multidisciplinary management
□ Attention to underlying chronic physical, psychological or emotional problems
□ Acute attacks: simple analgesia
□ Prevention: Amitriptyline
□ Discourage analgesics in chronic TTH
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14
Q

What is a seizure/ fit?

A

Any sudden attack from whatever cause

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15
Q

What is syncope?

A

Faint (a neuro-cardiogenic mechanism)

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16
Q

What is a convulsion?

A

Seizure where there is prominent motor activity

17
Q

What is an epileptic siezure?

A
  • An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons
  • It may have clinical manifestations
  • Paroxysmal change in motor, sensory or cognitive function
  • Depends on seizure’s location, degree of anatomical spread over cortex, duration
18
Q

What is epilepsy?

A
  • A tendency to recurrent, unprovoked (spontaneous) epileptic seizures
  • A question that must be answered clinically, with recourse to EEG only for supportive evidence
  • A seizure is not necessarily epileptic
  • Consequences of misdiagnosis of epilepsy can be serious
19
Q

What are the mechanisms of an epileptic fit?

A

□ Chemically triggered by:
® Decreased inhibition (gama-amino-butyric acid, GABA)
® Excessive excitation (glutamate and aspartate)
® Excessive influx of Na and Ca ions
□ Chemical stimulation produces an electrical current
□ Summation of a multitude of electrical potentials results in depolarization of many neurons which can lead to seizures, can be recorded from surface electrodes (Electroencephalogram)

20
Q

What is the stepwise approach to diagnosing epilepsy?

A

□ Is the paroxysmal event epileptic in nature?
□ Is it epilepsy?
□ What seizure types are occurring?
□ What is the epilepsy syndrome?
□ What is the aetiology?
□ What are the social and educational effects on the child?

21
Q

What is the role of EEG in diagnosing epilepsy?

A

® An interictal EEG has limited value in deciding when the individual has epilepsy
® Sensitivity of first routine interictal EEG: 30- 60%
® Problematic false positive rates: paroxysmal activity seen in 30%, frankly epileptiform activity in 5% of normal children
® Useful in identifying seizure types, seizure syndrome and aetiology

22
Q

How is epilepsy dianosed in children?

A

® History
® Video recording of event
® ECG in convulsive seizures
◊ Can get epilepsy with arrhythmias
® Interictal/ ictal EEG
® MRI Brain: to determine aetiology e.g. Brain malformations/ brain damage
® Genetics: idiopathic epilepsies are mostly familial; also single gene disorders e.g. Tuberous sclerosis
® Metabolic tests: esp. if associated with developmental delay/ regression

23
Q

What should be considered when thinking about giving anti-epileptc drugs to children?

A
  • Anti-epileptic drugs (AED) should only be considered if diagnosis is clear even if this means delaying treatment
  • Role of AED is to control seizures, not cure the epilepsy
  • Start with one AED: slow upward titration until side-effects manifest or drug is considered to be inefficient
  • Age, gender, type of seizures and epilepsy should be considered in selecting AEDs
  • S/Es: CNS related can be detrimental; Drowsiness, effect on learning, cognition and behavioural
24
Q

What are the antiepiletic drugs (and other therapies) that are used in children?

A

□ Sodium Valproate: first line for generalised epilepsies (not in girls- recent MHRA advice)
□ Carbamazepine: first line for focal epilepsies
□ Several new AEDs with more tolerability and fewer side effects: Levatiracetam, Lamotrigine, Perampanel
□ Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for resistant epilepsies), surgical procedures

25
Q

What is a vagus nerve stimulator?

A
  • Used in the treatment of childhood epilepsy
    □ Sends tiny electrical shocks to the vagus nerve
    □ 3 pieces of equipment used in VNS
    ® Programmable generator
    ® Lead with two coils at the end
    ® Hand held magnet
26
Q

What are the different types of non-epileptic seiures and other mimics in children?

A
  • Acute symptomatic seizures: due to acute insults e.g. Hypoxia-ischaemia, hypoglycaemia, infection, trauma
  • Reflex anoxic seizure: common in toddlers
    □ Triggers: pain, fright
  • Syncope
  • Parasomnias e.g. night terrors
  • Behavioural stereotypies
    □ Pre-school aged children
    □ Repetitive behaviours e.g. rocking in a corner or head banging
  • Psychogenic seizures (NEAD)
27
Q

What is a febrile convulsion?

A
  • An event occurring in infancy/ childhood, usually between 3 months and 5 years of age
  • Associated with fever but without evidence of intracranial infection or defined cause for the seizure
  • Commonest cause of ‘acute symptomatic seizure’ in childhood
28
Q

What are the different siezure types?

A
  • Jerk/ shake: clonic, myoclonic, spasms
  • Stiff: usually a tonic seizure
  • Fall: Atonic/ tonic/ myoclonic
  • Vacant attack: absence, complex partial seizure
29
Q

What is microcephaly?

A
  • Definition:
    □ OFC <2 SD: mild
    □ OFC <3 SD: moderate/ severe
  • Microcephaly usually indicates small brain: ‘microencephaly’
  • Prenatal or postnatal onset: is it crossing centiles downwards?
  • Timing of onset may be a clue
  • Multiple causes: antenatal, postnatal, genetic and environmental
30
Q

What is macrocephaly?

A
  • Definition: OFC > 2SD
  • Is it crossing centiles upwards?
  • Sutures?
  • Fontanelles?
  • Familial?
  • Hydrocephalus?
  • Large brain?
  • Development normal?
  • Other physical abnormalities – facial features, hepatosplenomegaly, bony deformities etc
31
Q

What should you think about when there are head shape problems in a child?

A
  • Often in first year of life
  • Timing of onset?
  • Is it getting better or worse?
  • Plagiocephaly ‘flat-head’
  • Brachycephaly ‘short head or flat at back’
  • Scaphocephaly ‘boat shaped skull’
  • Craniosynostosis
32
Q

When should you suspect neuromuscular disorders in a child?

A
  • 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
  • Not being able to hold its head up by 3 months
  • Girdle sign- demonstrates pelvic girdle weakness
33
Q

What is myopathic facies?

A

Muscle problem- the child has droopy eyelids and cannot close mouth, poor head control

34
Q

What is myotonia?

A

difficulty in relaxing muscles (common in congenital myotonia and myotonia dystrophies)

35
Q

What is Pes cavis?

A

high foot arch and hammer toes- Charcot-Marie-tooth disorder

36
Q

What is Duchene muscular dystophy?

A
  • Xp21, dystrophin gene, 1/3500 male infants
  • Delayed gross motor skills
  • Symmetrical proximal weakness
    □ Waddling gait, calf hypertrophy
    □ Gower’s sign positive
  • Elevated Creatinine Kinase levels
    □ >1000 in DMD
  • Cardiomyopathy
  • Respiratory involvement in teens
37
Q

What is the managemnt of Duchene muscular dystrophy?

A

Steroids can prolong life (they can now live until late 20s to early 30s)