Fits, Faints and Funny Turns in Childhood

Introduction

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.

Never rely on a second person description for a diagnosis, always take a detailed history from the eyewitness. 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.

Although these events are often stereotyped, most of the time they are not seizures. This article will cover the assessment and identification of fits, faints and funny turns in children. 

History

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

For each type of episode, it is important to ask about:

• 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?’

Seizures and epilepsy

A seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.¹

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 vs acute symptomatic seizures

Acute symptomatic seizures are a result 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 (e.g recurrent febrile seizures).

The role of the electroencephalogram (EEG)

Routine use of EEG is of limited value. False-positive rates are 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?

Table 1. An overview of different types of seizures.^2,3

Paediatric epilepsy syndromes

Epilepsy syndromes are defined entirely phenotypically based on 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. Listed below are the more common paediatric epilepsy syndromes. 

Infantile spasms (West syndrome) ^4,5

Typical age of onset:

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

Typical clinical features include:

• 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

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

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

Typical age of onset:

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

Typical clinical features include:

• 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

EEG shows centro-temporal spikes activated by sleep.

Childhood absence epilepsy⁸

Typical age of onset:

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

Typical clinical features include:

• 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

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

Juvenile myoclonic epilepsy (JME) ⁹

Typical age of onset:

• 12 -18 years

Typical clinical features include:

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

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

Non-epileptic paroxysmal episodes

Episodes occurring in sleep

Benign neonatal sleep myoclonus

These typically occur in healthy infants a few weeks old. They are confined entirely to sleep and involve myoclonic movements not involving the face. The EEG will be normal.

Parasomnias

Examples of parasomnias include sleep paralysis, confusional arousal, sleepwalking and night terrors. They are confined to sleep and are usually recurrent. 

Episodes occurring on feeding

GORD/Sandifer syndrome

Gastro-oesophageal reflux disease can cause apnoea and dystonic posturing of the head, neck and back. Neurological signs associated with feeding collectively are referred to as Sandifer syndrome.

Episodes triggered by fever

Febrile seizures

Febrile seizures refer to seizures in association with a fever with no definable intracranial cause. They typically present as a brief, generalized convulsive seizure.

Febrile seizures usually occur in children aged 6 months to 6 years of age. 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, there is a 4-12% risk of developing epilepsy.

Vaso-vagal syncope

Vaso-vagal syncope typically occurs in older children. Triggers may include intercurrent illness, heat, low food and water intake, prolonged standing.

Typical features include prodromal pallor, nausea, visual greying and dizziness. Children can fall to the ground, may jerk and be incontinent of urine. There is a rapid recovery following the episode.

Episodes triggered by 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.

These episodes occur due to vagally-mediated severe bradycardia or asystole. They are self-limiting and require no treatment.

Children often grow to be adults that faint at 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. There is prolonged end-expiratory apnoea. Children may turn blue, limp and experience a brief loss of consciousness.

Hyperekplexia

Hyperekplexia is whole-body stiffening in response to sudden noises or being touched and handled.

The severe neonatal form can result in life-threatening apnoea. Episodes can be terminated by forcible flexion of the neck

Episodes triggered by tiredness, boredom or stress

Self-gratification behaviour

Self-comforting phenomena in toddlers and in older children with neurological disability. Typically occurs in a highchair or car seat.

Older children may lie prone or supine with adducted and crossed legs. They may be unresponsive to attempted interruption.

Tics

Tics are 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

Daydreaming occurs with boredom or watching a screen. Children may not initially respond to their name being called and be described as ‘vacant’. Daydreaming doesn’t interrupt conversations or normal activity

Episodes triggered by excitement

Shuddering spells

Excitements such as a toy or food, provoke shivering, as if chilled, sometimes involving the whole body. These are benign.

Cataplexy

Strong emotion, particularly laughter, provokes a sudden, temporary loss of tone. This 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

Editor 

Dr Chris Jefferies

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. 3^rd ed. 2018. Oxford University press.
3. Bale, JF; Bonkowsky, JL; Filloux, FM; Hedlund, GL; Larsen, PD; Morita, DC. Pediatric Neurology. 2^nd 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. Available from: [LINK]
5. Appleton, R; Camfield, P. Childhood epilepsy: management from diagnosis to remission. 1^st ed. 2011. Cambridge University press.
6. Epilepsy.org.uk. Benign Rolandic Epilepsy. Available from: [LINK]
7. Epilepsy Diagnosis Organisation. ECTS overview. Available from: [LINK]
8. Epilepsy Diagnosis Organisation. CAE overview. Available from: [LINK]
9. Epilepsy Diagnosis Organisation. JME overview. Available from: [LINK]
10. Pina-Garza, JE. Fenichel’s clinical pediatric neurology. 7^th 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’. Available from: [LINK]