Paediatric Neurology Flashcards

1
Q

types of pathology causing neurological issues in children

A
• Congenital
• Neurogenetic diseases and syndromes
• Neurometabolic diseases and syndromes
• Acquired
o Infection
o Ischaemia
o Trauma
o Tumour
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2
Q

describe the neurologicl consultation in children

A
  • History taking: interactive
  • Hear what was said, not what you thought was said
  • Avoid quasi-medical language
  • Time course of symptoms crucial
  • Distinguishing static from slowly progressive symptoms can be challenging
  • Perinatal, developmental, family history
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3
Q

describe a developmental hx

A
  • Motor milestones: gross and fine motor skills
  • Speech and language development
  • Early cognitive development
  • Play esp. symbolic play and social behaviour
  • Self-help skills
  • Vision and hearing assessment
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4
Q

describe the neurological examination in children

A
  • Opportunistic approach and observation skills
  • Appearance
  • Gait
  • Head size
  • Skin findings
  • Real world examination (depends on age)
  • Synthesis of history and clinical findings into a differential diagnosis and investigation plan
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5
Q

what % of hospitalised children have a neurological conditions

A

25%

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6
Q

what percent of childre aged 10-17 have migraines?

A

7.7.%

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7
Q

what are the first and second most common cancers in children?

A

leukaemia

brain tumours

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8
Q

by age 7 and 15 what % of children have had a headache

A

40%

75%

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9
Q

most parents who seek help for a child with a headache are looking for what?

A

reassurance that it is not due to a serious cause usually brain tumour

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10
Q

4 ways to describe the onset of a headache

A
  • Isolated acute
  • Recurrent acute
  • Chronic progressive
  • Chronic non-progressive
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11
Q

draw out the different types of headache in a child

A

see notes

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12
Q

recurrent or chronic headache in children hx

A
Is there more than 1 type of headache?
Typical episode:
• Any warning
• Location
• Severity
• Duration
• Frequency
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13
Q

headache examination in children

A
  • Growth parameters, OFC, BP
  • Sinuses, teeth, visual acuity
  • Fundoscopy
  • Visual fields (craniopharyngioma)
  • Cranial bruit
  • Focal neurological signs
  • Cognitive and emotional status
  • The diagnosis of headache aetiology is clinical
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14
Q

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

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15
Q

migraine vs tension headache

A
Migraine Tension Headache
Hemicranial pain Diffuse, symmetrical
Throbbing/pulsatile Band-like distribution
Abdo pain, N+V Present most of the time but there may be
symptom free periods
Relieved by rest Constant ache
Photophobia/phonophobia
Visual, sensory, motor aura
Positive family history
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16
Q

features of raised intracranial pressure

A

Aggravated by activities that raise ICP e.g. coughing. Woken from sleep with headache

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17
Q

features of analgesic overuse headache

A
  • Headache is back before allowed to use another dose
  • Paracetamol/ NSAIDs
  • Particular problem with compound analgesics e.g. Cocodamol
18
Q

indications for neuroimaging in children with headaches

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
19
Q

management of migraine in children

A
  • Acute attack: effective pain relief, triptans

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

20
Q

management of TTH 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
21
Q

define seizure/fit

A

any sudden attack from whatever cause

22
Q

define syncope

A

faint - a neuro-cardiogenic mechanism

23
Q

define convulsion

A

seizure where there is prominent motor activity

24
Q

define epileptic seizure

A

an abnormal excessive hypersyncronous discharge from a group of usually cortical neurones

25
Q

what do clinical features of an epileptic seiure depend on?

A
  • Paroxysmal change in motor, sensory or cognitive function

* Depends on seizure’s location, degree of anatomical spread over cortex, duration

26
Q

define epilepsy

A

a tendency to recurrent unprovoked epileptic seizures

27
Q

examples of non-epileptic seizures in children

A

• Acute symptomatic seizures: due to acute insults e.g. Hypoxia-ischaemia, hypoglycemia,
infection, trauma
• Reflex anoxic seizure: common in toddlers
• Syncope
• Parasomnias e.g. night terrors
• Behavioural stereotypies
• Psychogenic seizures (NEAD)

28
Q

define 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

29
Q

types of seizure

A
  • Distinguishing seizure types can be challenging
  • Jerk/ shake: clonic, myoclonic, spasms
  • Stiff: usually a tonic seizure
  • Fall: Atonic/ tonic/ myoclonic
  • Vacant attack: absence, complex partial seizure
30
Q

mechanism of an epileptic seizure

A

• Chemically triggered by:
o Decreased inhibition (gamma-amino-butyric acid, GABA)
o Excessive excitation (glutamate and aspartate)
o 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)

31
Q

childhood vs adult onset epilepsies

A
  • Majority are idiopathic in origin (both Focal & Generalised)
  • Majority of epilepsies are generalised
  • Seizures can be subtle (absences, myoclonus, drop attacks)
32
Q

why can a diagnosis of epilepsy be difficult?

A

o Non-epileptic paroxysmal disorders are more common in children
o Difficulty in explaining (Children are not young adults)
o Difficulty in interpretation (witness)
o Difficulty in interpretation and synthesising information(physician

33
Q

stepwise approach to a diagnosis of 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?
34
Q

role of the EEG in 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
35
Q

diagnosis of epilepsy in children

A

• History
• Video recording of event
• ECG in convulsive seizures
• 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

36
Q

management of epilepsy in 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
• 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: Levetiracetam, Lamotrigine,
Perampanel
• Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for resistant
epilepsies)

37
Q

when to suspect a neuromuscular disorder

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
38
Q

types of neuromuscular disorders

A
  • Muscle: muscular dystrophies, myopathies- congenital and inflammatory, myotonic syndromes
  • Neuromuscular junction: myasthenic syndromes
  • Nerve: Hereditary or acquired neuropathies
  • Anterior Horn Cell: Spinal muscular atrophy
39
Q

DMD: gene

A

Xp21, dystrophin gene

40
Q

DMD: signs

A
• Delayed gross motor skills
• Symmetrical proximal weakness
o Waddling gait, calf hypertrophy
o Gower’s sign positive
• Elevated Creatinine Kinase levels
o >1000 in DMD
• Cardiomyopathy
• Respiratory involvement in teens