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Year 4 PAEDS > Nervous System > Flashcards

Nervous System Flashcards

1
Q

What is cerebral palsy?

A

A group of disorders in which there is developmental delay and disorder of posture and movement resulting from a non-progressive, permanent, fixed cerebral lesion in developing/immature brain

May or may not also present with other neurological symptoms eg learning difficulties / epilepsy

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2
Q

How common is cerebral palsy?

A

Most common motor impairment in children

1/500

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3
Q

What proportion of cerebral palsy are caused by damage to the immature brain antenatally, perinatally and postnatally?

A 
Antenatal = 80%
Perinatal = 10%
Postnatal = 10%
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4
Q

What are some antenatal causes of cerebral palsy?

A

1) Maternal infection = TORCH
2) Radiation exposure
3) Intraventricular haemorrhage (IVH)
4) Chorioamnionitis
5) Multiple births eg twins
6) Maternal resp or genitourinary infection

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5
Q

What is TORCH?

A 
Toxoplasmosis
Other infections eg syphilis, VZV, parvovirus B19, Listerosis and Coxsackie virus
Rubella
CMV
Herpes simplex
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6
Q

What are some perinatal causes of cerebral palsy?

A

1) Hypoxic-ischaemic encephalopathy (HIE)

2) Intrapartum trauma

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7
Q

What are some postnatal causes of cerebral palsy in the neonate?

A 
IVH
Hyperbilirubinaemia - Kernicterus
Hypoglycaemia
Cerebral infarct
Meconium aspiration
Meningitis
Encephalitis
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8
Q

What are some postnatal causes of cerebral palsy in the infant?

A

Hydrocephalus
Hypoglycaemia
CNS infection

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9
Q

What are some postnatal causes of cerebral palsy in the child?

A

Hypoxic event eg drowning
Head trauma
Lead poisoning
CNS infection

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10
Q

What is the disease progress of cerebral palsy?

A

Lesion is fixed and non-progressive but the symptoms become worse over time

= “circle on an inflating balloon” as child’s brain grows

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11
Q

What are the three classifications of cerebral palsy based on type of movement disorder?

A

1) Spastic (80%)
2) Dyskinetic
3) Ataxic

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12
Q

Describe the spastic movement of cerebral palsy

It is caused by damage to what?

A

Intermittently increased tone and pathological reflexes = stiff and tight muscles

Damage of UMN

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13
Q

What are the varying degrees of spastic movement disorder in cerebral palsy?

A

Hemiplegia
Diplegia
Quadriplegia

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14
Q

Describe hemiplegic spastic movement in cerebral palsy

A

Hemiplegia:

  • Unilateral arm and leg affected (arm>leg)
  • Arm = flexed and pronated
  • Leg = cricumducted gate, tiptoe walking, delayed walking
  • Moderate developmental delay and seizure risk
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15
Q

Describe diplegic spastic movement in cerebral palsy

A

Diplegia:

  • Mostly lower limbs affected (less arms)
  • Commando crawl = dragging legs scissored
  • Often normal intellectual development and minimal seizure risk
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16
Q

Describe quadriplegic spastic movement in cerebral palsy

A

Quadriplegia:

  • All four limbs affected
  • Increased tone
  • Swallowing difficulties
  • Significant intellectual delay
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17
Q

Describe the two types of dyskinetic movement of cerebral palsy

It is caused by damage to what?

A

1) Athetoid = writhing movement
- Often normal intellectual development

2) Dytonic = involuntary movements
- Worse on movement
- Unusual posture

Damage to basal ganglia

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18
Q

Describe the ataxic movement of cerebral palsy

It is caused by damage to what?

A

Shaky movements, poor balance and sense of positioning

Ataxic gait = wide base, unsteady trunk, jerky movements

Damage to cerebellum

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19
Q

When can the lesion occur in cerebral palsy?

A

At any point from conception - 3yrs

After 3 yrs = acquired brain injury

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20
Q

How may cerebral palsy present?

A

1) Delayed milestones
2) Abnormalities of tone eg hypotonia, spaces or dystonia
3) Abnormal motor development eg late head control, rolling, crawling
4) Feeding difficulties

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21
Q

What are the most common delayed milestones in children with cerebral palsy?

A

1) Not sitting by 8 months
2) Not walking by 18 months
3) Early hand preference before 1 year (should be ambidextrous until 18 months)

Correct for gestational age

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22
Q

What are complications of CP?

A

Physical

  • Muscle spasms and contractions
  • Feeding difficulties and nutritional problems
  • incontinence

Social

  • Problems dressing, ADLs
  • Developmental delay
  • Hearing / language impairment

MSK

  • Scoliosis
  • Hip dislocation
  • Inc risk of low bone mineral density = inc risk of osteomalacia / osteoporosis

Neuro
- Epilepsy

Other body systems

  • Recurrent respiratory infections
  • GORD
  • Constipation
  • UTI

NB many children with CP have preserved cognitive function

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23
Q

What investigations are done for CP?

A

Diagnosis made clinically

Exclude other causes eg:

  • Thyroid studies
  • Chromosomal analysis
  • CSF etc
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24
Q

What may be given to improve movement in CP?

A

Mobility aids eg orthotic devices, wheelchairs

Splinting improve ROM eg ankle joints

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25
Q

What may be given to help manage spacisity in CP?

A

Oral diazepam = useful if rapid effect needed eg pain crisis

Baclofen = sustained long term effect eg continuous discomfort

Baclofen can be given via continuous pump-administered intrathecal Baclofen

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26
Q

What may be given to help manage dystonia eg problems with posture, function or pain?

A

Trihexyphenidyl
Levodopa
Baclofen

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27
Q

When may botulinum toxin type A be given in CP?

A

Focal spasticity eg impeding fine motor function / disturbing sleep

Rapid onset spacisity

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28
Q

What professionals involved in MDT approach of CP?

A 
Paeds
OT
SALT
Nutrition
Education
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29
Q

What are febrile seizures?

A

aka febrile fit/convulsion

= Seizures occurring in children aged 6 months - 5 years, associated with fever, without an underlying cause such as a CNS infection or electrolyte imbalance

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30
Q

What two features must be present in order for a classification of a febrile convulsion?

A

1) Axillary temperature above 37.8 degrees c

2) Clinical hx / examination indicative of febrile seizures

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31
Q

What is the emergency treatment of febrile seizures?

A

If child is still convulsing or not fully altert:

  • Recovery position + ABCDE
  • Check blood glucose
  • If still seizing >5min = rectal diazepam OR single dose buccal midazolam OR IV lorazepam

Meningococcal disease suspected:
- benpen or cefotaxime

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32
Q

What is a simple febrile seizure?

A

Generalised tonic-clonic seizures
Last <15mins
Do not recur within 24hrs or within the same febrile illness

= most febrile seizures

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33
Q

What is a complex febrile seizure?

A

Must have one/more of:

1) Focal features at onset or during seizure
2) Duration >15mins
3) Recurrence within the same febrile illness

= 20% febrile convulsions

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34
Q

What is a febrile status epileptics?

A

Febrile seizure lasting more than 30 minutes

= 5% febrile convulsions

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35
Q

What are other types of seizures related to acute illness in children?

A

1) Febrile myoclonic seizures
2) Afebrile convulsions in young children with mild gastroenteritis - clusters of seizures with/without fever over several days in those with gastroenteritis = good prognosis

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36
Q

How common are febrile convulsions?

A

Common - 2-5% children

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37
Q

What are the most common causes of fever in febrile convulsions? (5)

A

Most:

1) Viral infections eg URTI
2) OM
3) Tonsillitis

Others:

4) Gastroenteritis
5) Post-immunisation

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38
Q

What are some serious illnesses which need excluding in a febrile child with a seizure?

A

1) Meningitis and septicaemia
2) UTI
3) LFTI
4) Cerebral malaria

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39
Q

What features of a seizure are important in a seizure hx?

A

1) Conscious level prior to seizure
2) Duration
3) Focal or generalised
4) Time taken to recover
5) State of child after

CHECK for signs of meningitis or sepsis

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40
Q

What investigations are performed for a febrile convulsion?

A

Investigate febrile illness rather than seizure, eg:

Bloods: FBC, ESR, glucose, U&Es, coagulation, culture

Urine microscopy/culture

LP

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41
Q

Ddx for febrile convulsion?

A 
Rigors
Syncope
Breath-holding spells
Reflex anoxic seizures
Apneoa
Postictal fever
Epilepsy
Hypoglycaemia
Encephalitis
Afebrile seizures with gastroenteritis
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42
Q

What are reflex anoxic seizures?

A

A precipitant eg minor bump causes vaguely mediated cardiac asystole lasting many seconds

Child may be pale, floppy and lose consciousness, followed my tonic and clonic movements

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43
Q

What makes a posticltal fever more likely?

A

Temp >38

Seizure lasts >10min

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44
Q

What is the management of a febrile convulsion?

A

Monitor for a few hours

Send home if child looks well, parents understand how to treat febrile illness and further seizures and can access medical services easily

Arrange review

45
Q

What advice should be given to a parent of a child with febrile convulsions?

A

Give leaflet

Explain what febrile seizures are

How to treat fever at home - remove excess clothing, give fluids, give antipyretics if child uncomfortable

Tepid sponging or cooling not recommended

Check for non-blanching rash, change of alertness, dehydration, fever >5 min - seek medical assistance

Stay with child at night

First aid if child has a fit - position child and do not put anything in their mouth

Call 999 if seizure lasts more than 5 minutes

46
Q

What is the prognosis of febrile convulsions?

A

Good

By definition do not recur beyond 5 yrs (30% do)

Intellect not affected, nor is there an inc risk of death

47
Q

What are some risk factors of recurrence of febrile convulsions? (4)

A

1) FH
2) Onset <18 months
3) Lower temperatures
4) Shorter duration of fever at onset

48
Q

What % of children with febrile seizures go on to develop epilepsy?

A

2-7%

Higher with complicated febrile convulsions

49
Q

In which age group do breath holding spells mainly occur? How common are they?

A

Babies and toddlers (<3yr)

Common - approx 2%

50
Q

What two categories can breath holding spells be split into?

A

Cyanotic

Pallid

51
Q

What is a cyanotic breath holding spell?

A

Event it always precipitated by crying because of pain or temper

Child takes a deep breath, stops breathing, becomes deeply cyanotic and the limbs extend

Prolonged attacks can lead to transient LOC and occasionally jerks of extremities

Absent of postictal phase is key

52
Q

What is the management of cyanotic breath holding spells?

A

Reassurance of parents

Parents ignoring is ideal (but cold water on forehead may be helpful)

53
Q

What is the prognosis of cyanotic breath holding spells?

A

The attacks are always benign and disappear before the child reaches school age

However, children with this hx have a higher incidence of vasovagal attacks later in life

54
Q

What is a pallid breath holding spell?

A

= Reflex atonic seizures

Classically follows a bump on the head or other minor injury which triggers vagal reflex overactivity, causing transient bradycardia and circulatory impairment

55
Q

How do pallid breath holding spells present?

A

Child may or may not start to cry, but then turns pale and collapses

There is transient apnoea and limpness, followed by rapid recovery

56
Q

How can pallid breath holding spells be differentiated from epilepsy?

A

Typical history and absence of postictal drowsiness

Normal electroencephalogram (EEG)

57
Q

How are pallid breath holding spells managed?

A

Reassurance

Disappear spontaneously prior to school age

58
Q

What is a seizure / convulsion / fit?

A

Impairment or loss of consciousness, abnormal motor activity, sensory disturbances or autonomic dysfunction

59
Q

What is epilepsy?

A

Condition of recurrent fits resulting from paroxysmal involuntary disturbances of brain function, which are unrelated to a fever or an acute cerebral insult

60
Q

How is epilepsy categorised?

A

1) Generalised from onset
2) Focal - beginning in a localised or focal area of the brain, but may become generalised resulting in a tonic-clonic fever

61
Q

Give 5 types of generalised seizure

A

1) Generalised tonic-clonic seizure - Grand mal
2) Tonic seizure
3) Absence seizures = petit mal
4) Myoclonic seizures
5) Atonic seizures

62
Q

Give 2 types of focal seizure

A

1) Focal motor seizure

2) Focal sensory seizure

63
Q

Give 4 examples of locations of focal seizures

A

1) Frontal lobe seizures
2) Temporal lobe seizures
3) Parietal lobe seizures
4) Occipital lobe seizures

64
Q

List some causes of epilepsy in children

A

1) Idiopathic = majority
2) Lesion disrupting electrical activity in brain (esp those with LD)
3) Cerebral insult eg trauma / infection

65
Q

Describe a generalised tonic clonic seizure (GTCS)

A

Tonic stage:

  • Start with sudden LOC when child falls to the ground, limbs extend, back arches and breathing stops
  • Teeth are tightly clenched and tongue may be bitten

Clonic stage:

  • Intermittent jerking movements of limbs and face
  • Onset of irregular breathing and often micturition and salivation
  • Usually lasts only a few minutes

Followed by postictal depression:
- Child may remain sleepy and disorientated for some time

66
Q

Describe an absence seizure

A

Fleeting (5-20s) impairment of consciousness, unassociated with falling or involuntary movements

Child often thought to be daydreaming, seen to stop what they are doing an look vacant before continuing as before

Can affect academic progress if occur frequently

67
Q

How can an absence seizure be confirmed?

A

EEG showing characteristic bursts of 3-per-second spike and wave activity

68
Q

Describe a myoclonic seizure

A

Shock like jerks resulting in sudden falls

69
Q

Myoclonic seizures tend to be seen in children with what background?

A

Children who have evidence of a structural neurological disorder and esp cerebral degenerative disorders

70
Q

What is west syndrome?

A

Infantile spasms

71
Q

What are infantile spasms?

A

Form of myoclonic epilepsy but considered separately as they have a particularly poor prognosis

72
Q

What is the age of onset of infantile spasms?

A

3-8 months

73
Q

Describe what happens in infantile spasms

A

Typical flexion spasms (‘jack-knife’ or ‘salaam spasms’) lasting a few seconds and occurring in clusters lasting up to half an hour

Often occur from waking from sleep

74
Q

What may be in the history of a child with infantile spasms?

A

Potentially a hx of perinatal complications eg asphyxia or meningitis

Development prior may be normal or delayed, but following onset there generally is a regression of developmental skills and prognosis is poor

75
Q

What do investigations reveal in infantile spasms?

A

EEG shows characteristic ‘hypsarrhythmic’ (chaotic) pattern

76
Q

Describe a focal seizure

A

Usually consists of twitching or jerking of one side of the face, an arm or a leg = focal motor seizures

Sometimes can manifest as a strange sensation eg tingling in one part of the body = focal sensory seizures

Consciousness is usually retained or only slightly impaired

Child may experience temporary weakness of involved part of body after an attack

77
Q

What is a Jacksonian march?

A

Sometimes occurs in focal seizures - jerking can start in one part of the body and spread

78
Q

What is a secondarily generalised seizure?

A

A focal seizure that progresses into a full blown tonic-clonic attack

79
Q

Describe a temporal lobe seizure

A 
HEADD
Hallucinations
Epigastric rising + emotional
Automatisms = lip smacking / grabbing / plucking
Deja vu + dysphasia post ictal

Postictal phase present

80
Q

How is a temporal lobe seizure diagnosed?

A

Careful description of attack

EEG showing discharges arising from the temporal lobe

81
Q

What % of children with epilepsy have LD?

A

70%

82
Q

How is epilepsy diagnosed?

A 
Clinical based on history
>1 attack
Physical examination for neurogical signs
EEG 
MRI
83
Q

When is EEG helpful in epilepsy?

A

Characteristic patterns for absence seizures and infantile spasms

Otherwise only diagnostic if EEG obtained during an attack (unlikely)

Use with caution:

  • 5% normal children have abnormal EEG
  • 40-50% children with epilepsy have normal EEGs

24hrly EEG can be helpful if daily / nightly episodes

84
Q

When is an MRI helpful in epilepsy?

A

Indicated in children with:

  • Focal neurological deficits
  • Focal EEG changes
  • Focal or intractable seizures

To exclude focal lesions eg brain tumour or evidence of encephalitis

(CT if acute brain breed suspected)

85
Q

What is the drug of first choice for GTCS?

A

Carbamazepine
Valproate
Lamotrigine

86
Q

What is the drug of first choice for absence seizures?

A

Ethosuxamide
Lamotrigine
Valproate

87
Q

What is the drug of first choice for myoclonic seizures?

A

Valproate

88
Q

What is the drug of first choice for focal seizures?

A

Carbamazepine

Lamotrigine

89
Q

What is the drug of first choice for infantile spasms?

A

Vigabatrin

90
Q

Which anti-epileptic drug has these side effects:

  • Vomiting
  • Anorexia
  • Lethargy
  • Hair loss
  • Hepatotoxicity?
A

Valproate

91
Q

Which anti-epileptic drug has these side effects:

  • Abdo discomfort
  • Skin rash
  • Liver dysfunction
  • Leucopenia?
A

Ethosuximide

92
Q

Which anti-epileptic drug has these side effects:

  • Drowsiness
  • Irritability
  • Behavioural abnormalities
  • Excessive salivation?
A

Clonazepam

93
Q

Which anti-epileptic drug has these side effects:

  • Dizziness
  • Drowsiness
  • Diplopia
  • Liver dysfunction
  • Anaemia
  • Leucopenia?
A

Carbamazepine

94
Q

Which antiepileptic is teratogenic?

A

Valproate

95
Q

What are some important rules for prescribing anti-epileptics?

A
  • Start at lowest dose of therapeutic range
  • Gradually increase dose once drug has reached a steady-state of plasma level (about 5 times the drug half life)
  • 2nd drug added if 1st drug ineffective at max therapeutic plasma level
  • 1st drug should be gradually decreased unless it was partially effective, in which case it can be continued
  • Drugs should be given at intervals no longer than one half life
  • Drugs with sedative effects given at bedtime, or coincide with seizure pattern if there is one
96
Q

What advice should be given to a child with epilepsy?

A

Restriction of physical activity is unnecessary, except child should be attended by a responsible adult while bathing and swimming

Avoid cycling in traffic and climbing high gymnastic equipment

Can only drive if fit free for 1 year

97
Q

What is the management of an acute fit?

A

Acute GTCS = put in recovery positioned

  • Terminated by giving buccal midazolam or rectal diazepam (parents instructed on how to do this)
  • Only give IV drugs in hospital where there are facilities in case child has respiratory arrested

Other types of fit do not require emergency treatment

98
Q

What is status epilepticus?

A

Prolonged convulsion lasting >30 mins, or a series of shorter convulsions with failure to regain consciousness between them

99
Q

What is the management of status epilepticus?

A

Maintain airway
Give oxygen
Check blood glucose
If PR diazepam has been given, IV lorazepam should be given
PR paraldehyde or IV phenytoin can be tried

If all these have failed, anaethetist should be called and child should undergo rapid sequence induction of anaesthesia with thiopental

Any child with prolonged seizures - monitor carefully in ICU

100
Q

What instructions should be given to a parent on how to give rectal diazepam?

A

Rectal diazepam comes in dispenser that is easily dispensed into a child’s rectum

  • Put child in knee-chest position lying on side
  • Remove cap from rectal diazepam dispenser
  • Insert nozzle gently through anus up to the hilt of its spout
  • Squeeze contents slowly (2-3mins) into anus
  • Remove applicator, lie child in recovery position and stay with them
  • May make child sleepy for several hours
101
Q

What instructions should be given to a parent on how to give buccal midazolam?

A

More acceptable to parents / public places

  • Given as a liquid into the side of the mouth between gums and cheeks
  • Absorbed quickly into bloodstream
  • Given slowly using a plastic syringe or else the child may choke
  • If possible half the dose and give one to each cheek
  • Watch carefully for signs of reduced breathing
102
Q

What is the prognosis of idiopathic generalised seizures?

A

Good - more than 70% grow out of the condition during childhood

Therapy discontinued if fit free for 2 years

103
Q

What are some provocative factors for seizures? (3)

A

Sleep deprivation
Flashing lights
Alcohol withdrawal

104
Q

What is a prodrome?

A

Uncommon - may occur hours/days before seizure

Change in mood / behaviour noted by others

105
Q

What is a partial seizure?

A

Same as focal - limited to one area of brain

“simple” = consciousness not impaired

“complex” = consciousness impaired

106
Q

What is an aura?

A

Part of seizure that precedes other manifestations eg smell, visual, deja vu. Seen in focal seizures

Esp in temporal lobe seizure

107
Q

What is an atonic seizure?

A

= akinetic

Brief, sudden hypotonia associated with falls without LOC

108
Q

Describe the postictal phase

A 
Headache
Confusion
Tiredness
Drowsiness
Amnesia
Myalgia
Todd's palsy = after focal seizure in motor cortex
Dysphagia = after focal seizure in temporal lobe

Year 4 PAEDS (23 decks)

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