Neurology Flashcards

1
Q

Why is child neurology considered dynamic?

A
  • Brain continues to grow and function evolves

- Static lesions produce evolving features

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2
Q

Give examples of types of pathology that can affect children?

A
  • Congenital anomalies
  • Neurogenetic diseases and syndromes
  • Neurometabolic diseases and syndromes
  • Acquired: infection, ischaemia, trauma, tumour
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3
Q

What is included in the neurological consultation in childhood?

A
  • History taking interactive
  • Avoid medical jargon
  • Time course of symptoms
  • Distinguishing static from slowly progressive symptoms can be challenging
  • Perinatal, developmental, family history
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4
Q

What is assessed in a developmental history?

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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5
Q

What is looked at in a paediatric neurological examination?

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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6
Q

How common are neurological problems?

A
  • 10% of primary care consultations for children
  • 25% of hospitalised children
  • Migraine 7.7% of children 10-17
  • Traumatic Brain Injury: 180-300/ 100,000 children
  • Tourette syndrome: 1% of all children with high frequency in ADHD and OCD
  • Epilepsy: 0.7% of all children, 1/3 will have intractable epilepsy
  • Brain tumours: second most common cancer in children
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7
Q

What is the epidemiology of headache disorders?

A
  • 40% of children by age 7

- 75% of children by age 15

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8
Q

What are the 4 classifications of headache?

A
  • Isolated acute
  • Recurrent acute
  • Chronic progressive
  • Chronic non-progressive
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9
Q

What do you want to know about the typical episode of chronic headache?

A
  • Any warning?
  • Location?
  • Severity?
  • Duration?
  • Frequency?
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10
Q

What should you look at when examining a child due to headache?

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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11
Q

What features would suggest childhood migraine?

A
  • Associated abdominal pain, nausea, vomiting
  • Focal symptoms/ signs before, during, after attack: Visual disturbance, paresthesia, 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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12
Q

What is the typical presentation of migraine?

A
  • Hemicranial pain
  • Throbbing/ pulsatile
  • Abdo pain, nausea, vomiting
  • Relieved by rest
  • Photophobia/ phonophobia
  • Visual, sensory, motor aura
  • Positive family history
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13
Q

What is the typical presentation of tension headache?

A
  • Diffuse, symmetrical
  • Band-like distribution
  • Present most of the time (but there may be symptom free periods)
  • “Constant ache”
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14
Q

What type of headache would suggest raised intracranial pressure?

A
  • Aggravated by activities that raise ICP eg. Coughing, straining at stool, bending
  • Woken from sleep with headache
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15
Q

What type of headache would suggest analgesic overuse headache?

A
  • Headache is back before allowed to use another dose
  • Paracetamol/ NSAIDs
  • Particular problem with compound analgesics eg. Cocodamol
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16
Q

What are the indications for neuroimaging in headache?

A
  • Features of cerebellar dysfunction
  • Features of raised intracranial pressure
  • New focal neurological deficit eg. new squint
  • Seizures, esp focal
  • Personality change
  • Unexplained deterioration of school work
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17
Q

How is acute migraine treated in children?

A

Effective pain relief and triptans

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18
Q

What is the preventative treatment for migraines in children?

A
  • Pizotifen
  • Propranolol
  • Amitryptiline
  • Topiramate
  • Valproate
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19
Q

How are tension type headaches treated 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: Amitryptiline
  • Discourage analgesics in chronic TTH
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20
Q

Seizure/fit

A

Any sudden attack from whatever cause

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21
Q

Syncope

A

Faint ( a neuro-cardiogenic mechanism)

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22
Q

Convulsion

A

Seizure where there is prominent motor activity

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23
Q

What is a an epileptic seizure due to?

A

An electrical phenomenon coming form the brain

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24
Q

What does presentation of an epileptic seizure depend on?

A
  • Seizure location
  • Degree of anatomical spread over cortex
  • Duration
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25
Q

What is epilepsy?

A
  • A tendency to recurrent, unprovoked (spontaneous) epileptic seizures
  • A question that must be answered clinically with resource to EEG only supportive evidence
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26
Q

What non-epileptic seizures and mimics can occur in children?

A
  • Acute symptomatic seizures: due to acute insults eg. Hypoxia-ischaemia, hypoglycemia, infection, trauma
  • Reflex anoxic seizure: common in toddlers
  • Syncope
  • Parasomnias eg. night terrors
  • Behavioural stereotypies
  • Psychogenic seizures (NEAD: Non-epileptic attack disorder)
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27
Q

What is the commonest cause of acute symptomatic seizure in childhood

A

Febrile convulsion

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28
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

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29
Q

What are the different seizure types?

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

What are epileptic fits chemically triggered by?

A
  • Decreased inhibition (gama-amino-butyric acid, GABA)
  • Excessive excitation (glutamate and aspartate)
  • Excessive influx of Na and Ca ions
31
Q

What is the mechanism of epileptic fits?

A
  • Chemical stimulation produces an electrical current
  • Summation of a multitude of electrical potentials results in depolarisation of many neurones which can lead to seizures, can be recorded from surface electrodes (Electroencephalogram)
32
Q

What is a focal seizure?

A

A seizure which is restricted to one hemisphere of one part of the one hemisphere

33
Q

What is a generalised seizure?

A

Abnormally activity in both hemispheres

34
Q

Why can diagnosis of childhood epilepsies be challenging?

A
  • Non-epileptic paroxysmal disorders are more common in children
  • Difficulty in explaining (Children are not young adults)
  • Difficulty in interpretation (witness)
  • Difficulty in interpretation and synthesising information(physician)
35
Q

What type of seizures tend to occur in childhood?

A

Idiopathic and generalised

36
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 etiology?
  • What are the social and educational effects on the child?
37
Q

What is the effectiveness of interictal EEGs?

A
  • An interictal EEG has limited value in deciding when the individual has epilepsy
  • Sensitivity of first routine interictal EEG: 30- 60%
38
Q

What are the disadvantages of using EEG for diagnosis of fits?

A

Problematic false positives

  • Paroxysmal activity seen in 30%
  • Frankly epileptiform activity in 5% of normal children
39
Q

What are EEGs useful in?

A

Identifying seizure types, syndrome and aetiology

40
Q

How are seizures/fits diagnosed?

A

-History
-Video recording of event
-ECG in convulsive seizures
-Interictal/ ictal EEG
MRI Brain: to determine etiology eg. Brain malformations/ brain damage
-Genetics: idiopathic epilepsies are mostly familial; also single gene disorders eg. Tuberous sclerosis
-Metabolic tests: esp if associated with developmental delay/ regression

41
Q

When should anti-epileptic drugs be considered for use 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
42
Q

What should be considered when choosing anti-epileptics drugs?

A
  • Age
  • Gender
  • Type of seizures
  • Epilepsy
43
Q

How are anti-epileptic drugs used in children?

A

Start with one AED: slow upward titration until side-effects manifest or drug is considered to be inefficient.

44
Q

What are the possible side effects of anti-epileptic seizures?

A
  • CNS related
  • Drowsiness
  • Effect on learning
  • Cognition and behavioural effects
45
Q

What is the first line treatment for generalised epilepsies?

A

Sodium valproate

46
Q

What is the first line of treatment for focal epilepsies?

A

Carbamazepine

47
Q

Other than AED what other therapies can be used in childhood epilepsy?

A
  • Steroids
  • Immunoglobulins
  • Ketogenic diet (resistant epilepsy)
  • Vagal nerve stimulation
  • Surgery
48
Q

What do neuromuscular disorders affect?

A

Disorders of the peripheral nervous system from the anterior horn cells down

49
Q

When should you 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
50
Q

What signs suggest a floppy baby?

A
  • Infant exhibits weakness and flaccidity of all musculature
  • Infant hangs like rag doll when lifted under abdomen
  • Infant is unable to sit up or hold up head. Head drops back when infant is lifted by its hands
51
Q

How do muscular dystrophies present?

A
  • Shoulders and arm held back when walking
  • Sway back
  • Weak buttock muscles
  • Knees bend to take weight
  • Thick lower leg muscles
  • Thick heel cord (may walk on toes)
  • Belly sticks out
  • Thin weak thighs
  • Poor balance
  • Foot drop and tip toe contractures
52
Q

Give examples of neuromuscular conditions which affect the muscles.

A
  • Muscular dystrophies
  • Myopathies (congenital and inflammatory)
  • Myotonic syndromes
53
Q

Give an example of a neuromuscular conditions which affects the neuromuscular junction.

A

Myasthenia syndromes

54
Q

Give examples of neuromuscular conditions which affect the nerves.

A

Hereditary or acquired neuropathies

55
Q

Give an examples of a neuromuscular condition which affects the anterior horn cell.

A

Spinal muscular atrophy

56
Q

What are the classical signs of Duchenne muscular dystrophy?

A

Symmetrical proximal weakness

  • Waddling gait
  • Calf hypertrophy
  • Gower’s sign positive

Delayed gross motor skills

57
Q

What investigation is done for Duchenne muscular dystrophy?

A

Creatinine kinase levels

-Elevated >1000 in DMD

58
Q

What are the complications of Duchenne muscular dystrophy?

A
  • Cardiomyopathy

- Respiratory involvement in teens

59
Q

What is the incidence of Duchenne muscular dystrophy?

A

1 in 3,500 male infants

60
Q

What is the genetic abnormality in Duchene muscular dystrophy?

A

Xp21 dystrophin gene

61
Q

What is an epileptic seizure?

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
62
Q

Neuropathy: Site of weakness

A

Distal weakness

63
Q

Neuropathy: Reflexes

A

Lost early

64
Q

Neuropathy: Fasciculation’s

A

May be present

65
Q

Neuropathy: Contractures

A

Not a feature

66
Q

Neuropathy: Myocardial dysfunction

A

Not a typical feature

67
Q

Neuropathy:Sensory

A

May have concomitant sensory symptoms and signs

68
Q

Myopathy: Site of weakness

A

Usually proximal

69
Q

Myopathy: Sensory

A

Pure motor

70
Q

Myopathy: Reflexes

A

Preserved till late

71
Q

Myopathy: Fasciculation’s

A

Not typical

72
Q

Myopathy: Contractures

A

Present

73
Q

Myopathy: myocardial Dysfunction

A

May have accompanying cardiac dysfunction with the dystrophies