Fits, Faints and Funny Turns in Childhood


Paroxysmal events in infancy and childhood

Paroxysmal events (‘fits, faints and funny turns’) are frequently not witnessed by medical staff and so the diagnosis often rests on the description from the eyewitness observer or the child themselves if awareness was retained.

Important points to keep in mind include:

  • Never rely on a second person description for a diagnosis – always take a detailed history from the eyewitness.
  • Although these events are often stereotyped, most of the time they are not seizures and epilepsy is not a diagnosis of exclusion.
  • A detailed history often confirms the diagnosis without the need for further investigations.
  • A video of the event is invaluable! Ask the parents to record the episodes.

History

Identify each type of episode observed and give it a nickname.

For each type of episode, clarify:

  • Frequency – ‘how many in a day/week/month?’
  • Timing – ‘certain time of the day or behavioural states?’
  • Relationship to sleep – ‘on falling asleep, during sleep, waking?’
  • Triggers –tired, travel, excitement, exercise?’
  • Warning beforehand – ‘clings to parent, aware of one coming on?’
  • Duration – ‘how long does it last and if clustered how long between episodes?’
  • Colour change – ‘pallor, early cyanosis?’
  • Alterations in conscious level – ‘change in responsiveness, can describe the event themselves?’
  • Motor phenomena – ‘can you act out exactly what happens, any directional movements/lateralization?’
  • Recovery and symptoms following the event – ‘how long does it take to recover, any changes from the normal child after?’
  • Family history ‘does anyone in the family have similar looking episodes?’

Is this epilepsy? ¹

A seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.

Epilepsy is a disease of the brain defined by any of the following conditions:

  1. At least two unprovoked (or reflex) seizures occurring more than 24 hours apart
  2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years
  3. Diagnosis of an epilepsy syndrome

Therefore a diagnosis of epilepsy requires the seizures to be recurrent and unprovoked (i.e. the absence of a temporary or reversible factor lowering the seizure threshold).


Epilepsy versus acute symptomatic seizures

Acute symptomatic seizures are a result of a number of acute insults, including hypoglycaemia, hypoxia, electrolyte derangements, infection, and trauma.

Focus on any known triggers in the history – think ‘before’, not ‘after’.

They may also be recurrent – the best know example being recurrent febrile seizures.


The role of EEG

Routine use of EEG is of limited value – false-positive rates can be high and inter-ictal EEGs are usually normal in epileptic patients.

Use EEG to answer the following questions:

  • What seizure types are occurring?
  • What is the epilepsy syndrome?

Types of seizures 2,3

Jerk/ShakeClonic – rhythmic contractions followed by a slower relaxation phase
Myoclonic – isolated brief, fast contractions followed by complete muscle relaxation
Spasms – slightly longer phase of sustained contraction, typically occurring in runs
StiffnessTonic – a prolonged period of contraction of one or several muscle groups
FallsAtonic – sudden loss of muscle tone. Can cause the child to slump to the ground
Tonic and large myoclonic seizures can both cause children to fall
VacancyAbsence – brief arrest of speech and activity (typically <5 sec). May show automatisms of lip-smacking, eyelid flickering. Occur many times in a day
Focal – can cause impaired awareness and unresponsiveness. Last longer (>30 sec) than absences and occur less frequently

 


Paediatric epilepsy syndromes

Epilepsy syndromes are defined almost entirely phenotypically on the basis of age of onset, seizure types, EEG findings, neurodevelopmental outcomes, response to anti-epileptic drugs, and so on.

It is useful to understand that there are a limited number of ways underlying pathology can manifest as dysfunction according to the maturation status of the brain.

Syndromic diagnoses take time and require specialist input.

Below are the more common paediatric epilepsy syndromes. Early recognition and referral is an important skill for anyone working with children.

Infantile spasms; West syndrome 4,5

Onset

  • Peak: 4 – 7 months
  • Can be as late as 12 months

Presenting symptoms

  • Infantile spasms – sudden jerks of the neck, trunk or limbs followed by a few seconds of tonic posturing
  • Shortly after waking or when falling asleep
  • Clustered – several to hundreds in runs
  • Cries at the end of the run of spasms
  • Insidious onset with subtle spasms that increase over time
  • Encephalopathy/regression – loss of visual alertness and smile

Diagnosis

  • West syndrome is a combination of infantile spasms, hypsarrhythmic pattern on EEG and regression.
  • Numerous prenatal, perinatal and postnatal aetiologies.

Further information: Infantile Spasms Awareness Video UKIST

 

Benign epilepsy with centro-temporal spikes (BECTS); Rolandic epilepsy 6,7

Onset

  • 3 – 12 years
  • Spontaneous remission by mid-adolescence

Presenting symptoms

  • From sleep
  • Focal onset – facial or perioral
  • Sensory and/or motor
  • Tingling of one side of the mouth
  • Expressive aphasia or guttural sounds
  • Post-ictal drooling
  • Can experience secondary generalization with brief tonic-clonic movements

Diagnosis

  • EEG shows centro-temporal spikes activated by sleep.

 

Childhood absence epilepsy 8

Onset

  • 4 – 8 years (onset before 3 years is rare)
  • More common in girls

 

Presenting symptoms

  • Brief arrest of speech and activity (typically <5 sec)
  • Perioral or periocular flickering movements may be seen
  • Unrousable during
  • Rapid recovery, as if nothing ever happened
  • Occurs tens or even a hundred times a day
  • Can be induced by hyperventilation

Diagnosis

  • EEG shows 3 Hz generalized spike-and-wave pattern

Video example: https://www.youtube.com/watch?v=H3iLQi6wt94

 

Juvenile myoclonic epilepsy (JME) 9

Onset

  • 12 -18 years

Presenting symptoms

  • Often present with their first generalized tonic-clonic seizure
  • GTC seizures often preceded by a number of myoclonic jerks
  • Awareness retained during myoclonic jerks: history of dropping objects while preparing breakfast is common
  • Absences occur in up to one third

Diagnosis

  • EEG typically shows polyspike discharges followed by irregular 1-3 Hz slow waves.

Non-epileptic paroxysmal episodes 10, 11

Situational clues

In sleep

Benign neonatal sleep myoclonus

  • Healthy infant a few weeks of age
  • Myoclonic movements not involving the face
  • Confined entirely to sleep
  • Normal EEG

Video examplehttps://www.youtube.com/watch?v=XUQKW9p2qWA

 

Parasomnias

  • Confined to sleep
  • Complex behaviour and experiences classified by the stage of sleep
  • Recurrent
  • Examples include:
    • Sleep paralysis
    • Confusional arousal
    • Sleep walking
    • Night terrors

 

On feeding

GORD/Sandifer syndrome

  • Occult GORD can cause apnoea
  • GORD can precipitate dystonic posturing of the head, neck and back
  • Neurological signs associated with feeding collectively are referred to as Sandifer syndrome

 

Fever

Febrile seizures

  • Seizure in association with a fever with no definable intracranial cause.
  • Brief, generalized convulsive seizure
  • 6 months to 6 years of age
  • Genetic predisposition and age-limited
  • One third have a second febrile seizure in subsequent febrile illness.
  • Complex febrile seizures are prolonged, focal or repeated in the same illness
  • If complex, 4-12% risk of developing epilepsy

 

Vaso-vagal syncope

  • Older children
  • Intercurrent illness, heat, low food and water intake, prolonged standing
  • Prodromal pallor, nausea, visual greying and dizziness
  • Can fall to the ground, may jerk and be incontinent of urine
  • Rapid recovery

 

Pain, shock, startle

Reflex asystolic syncope / reflex anoxic seizure

  • Sudden unexpected pain or discomfort (e.g. banging head) causes the child to become pale, hypotensive and lose consciousness.
  • Some limb stiffening or clonic jerking may occur
  • Vagally-mediated severe bradycardia or asystole
  • Self-limiting and require no treatment
  • Children often grow to be adults that faint to the sight of blood or needles

 

Cyanotic breath-holding

  • Typically an angry and frustrated toddler becomes stuck at the end of a period of prolonged sobbing.
  • Prolonged end-expiratory apnoea
  • Blue, limp and brief loss of consciousness

 

Hyperekplexia

  • Whole-body stiffening in response to sudden noises or being touched and handled.
  • The severe neonatal form can result in life-threatening apnoea.
  • Can be terminated by forcible flexion of the neck

 

Tired, bored, stress

Self-gratification behaviour

  • Self-comforting phenomena in toddlers and in older children with neurological disability
  • Typically occurs in a high-chair or car seat
  • Older children may lie prone or supine with adducted and crossed legs
  • May be unresponsive to attempted interruption

 

Tics

  • Compulsive movements or gestures occurring in isolation
  • May be accompanied by vocal tics in Tourette syndrome
  • Older children can somewhat voluntarily suppress the compulsion to tic

 

Daydreaming

  • With boredom or watching a screen
  • May not initially respond to their name being called and be described as ‘vacant’
  • Doesn’t interrupt conversations or normal activity

 

Excitement

Shuddering spells

  • Excitements such as a toy or food, provoke shivering, as if chilled, sometimes involving the whole body.
  • Benign

 

Cataplexy

  • Strong emotion, particularly laughter, provokes a sudden, temporary loss of tone
  • Can be subtle with a brief sagging of the legs
  • Associated with sleep-wake fragmentation

Reviewer

Dr Rob Forsyth

Consultant and Senior Lecturer in Paediatric Neurology


References

  1. Fisher, RS; Acevedo, C; et al. A practical clinical definition of epilepsy. 2014. Epilepsia, 55(4):475–482. doi: 10.1111/epi.12550
  2. Forsyth, R; Newton, R. Paediatric Neurology. Oxford specialist handbooks in paediatrics. 3rd ed. 2018. Oxford University press.
  3. Bale, JF; Bonkowsky, JL; Filloux, FM; Hedlund, GL; Larsen, PD; Morita, DC. Pediatric Neurology. 2nd ed. 2017. CRC Press.
  4. D’Alonzo, R; Rigante, D; Mencaroni, E; Esposito, S. West Syndrome: A Review and Guide for Paediatricians. Clinical Drug Investigation, 2018, Vol.38(2), pp.113-124. [LINK]
  5. Appleton, R; Camfield, P. Childhood epilepsy: management from diagnosis to remission. 1st ed. 2011. Cambridge University press.
  6. Epilepsy.org.uk. Benign Rolandic Epilepsy. [LINK]
  7. Epilepsy Diagnosis Organisation. ECTS overview. [LINK]
  8. Epilepsy Diagnosis Organisation. CAE overview. [LINK]
  9. Epilepsy Diagnosis Organisation. JME overview. [LINK]
  10. Pina-Garza, JE. Fenichel’s clinical pediatric neurology. 7th ed. 2013. Elsevier Saunders
  11. National Institute of Health and Care Excellence. Epilepsies: diagnosis and management. ‘Appendix D Differential diagnosis of epilepsy in children, young people and adults’. [LINK]

 

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