Faints and funny turns, or non-epileptic paroxysmal events (NEPEs) as they are currently called, are very common in children.
They can be due to physiological or exaggerated physiological responses, parasomnias or movement disorders. NEPEs comprise a wide range of entities and can be challenging for the clinician to diagnose.
Epilepsy is the tendency to have recurrent unprovoked epileptic seizures. Misdiagnosis in epilepsy is not uncommon (up to 30%), owing to the lack of any specific biochemical marker or test, and is still a clinical diagnosis, supported by other relevant investigations such as EEG.
It may be impossible to differentiate between epileptic and non-epileptic phenomena at first assessment. The GP will rarely observe the episode in question at the time of evaluation, but clinical experience and a good history, with a clear description of the event, is often the key to accurate diagnosis.
The following is a brief description of the common NEPEs seen in children and an approach to their diagnosis in general practice.
Breath-holding
Breath-holding is a common phenomenon in young children. The onset is usually between six and 12 months, gradually escalating in frequency over the second year of life.
The sequence of events is classical. The child has been frightened or frustrated, or has had a minor injury, and begins to cry vigorously. Then the breathing stops and after several seconds, the child turns blue and loses consciousness. The child is often limp at this time.
The period of unconsciousness is brief, usually lasting less than one minute. The spell terminates with a sudden gasp and prompt return to consciousness.
A strong family history is obtained in as many as one in three children. There is a general misconception that the breath is held purposefully.
Anaemia has been associated with breath-holding spells and correction with iron supplementation can be considered. Drug treatment is seldom necessary.
The prognosis is good and there is no relationship to epilepsy. Addressing parents' stress and concern is an important aspect of care.
Reflex anoxic seizures
Reflex anoxic seizures (also called pallid breath-holding or pallid infantile syncope) are reflex asystolic syncopes. They are common in young children, but can occur at any age.
Any unexpected bump to the head, fright or seeing blood triggers a neurally mediated vagal discharge, leading to severe bradycardia, asystole, syncope and anoxic seizure.
The child falls unconscious, white as a sheet, and looks 'dead', followed by stiffening and jerking of limbs.
Education and reassurance are the mainstay of management. Cardiac pacing, the only definitive treatment, is required for severe cases.
Syncope
Syncope is defined as the temporary loss of consciousness resulting from a reversible disturbance of cerebral function. It is more common among adolescents than other ages. An estimated 20% of all children will experience at least one episode of fainting before the end of adolescence.
The differential diagnosis of syncope is wide, but most cases are benign, neurally mediated syncope.
More serious causes include underlying cardiac rhythm problems, structural defects and long QT syndromes.
Most of these children faint in response to one or more limited provocations - the sight of blood, a crowded and hot environment, upright posture or prolonged standing. The faint is usually preceded by brief dizziness, light-headedness, nausea, changes in vision or hearing, or a feeling of warmth.
Eye rolling, urinary incontinence, injury and vocalisations are all possible during the syncopal event and do not make a diagnosis of epilepsy. Many patients will have convulsive movements or isolated myoclonic jerks at the end of an episode.
The GP should take a detailed and focused history. Full neurological and cardiac examination will be required in suspected syncope.
The setting and stimulus are the most important identifiable factors. All children should have a baseline ECG to document QTc interval and have lying and standing BP recorded.
If there are any red flag signs (see box) or persistent symptoms, the child should be referred to a paediatrician. General advice about increasing fluid and salt intake, avoiding skipping meals and eliminating caffeine intake is often helpful.
| Childhood syncope |
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Red flag signs for cardiac syncope in children
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BNSM
Benign neonatal sleep myoclonus (BNSM) is a well-recognised sleep phenomenon, noted within the first three months of life.
The myoclonus occurs during non-rapid eye movement (NREM) sleep in otherwise normal neonates. It mainly affects the distal parts of the upper extremities.
Jerks can be synchronous or asynchronous, unilateral or bilateral, mild or violent, and can occur in clusters. They typically do not involve the face, stop on arousal and resolve by six months of age.
Night terrors
Night terrors (pavor nocturnus) are associated with transitions from NREM to REM sleep. They are more common in children aged three to 12 years, affecting both sexes. They usually happen in the first 90 minutes of sleep and generally do not recur.
Night terrors are characterised by sudden partial arousal. Children may sit upright, walk, run and talk incoherently, with extreme agitation. They appear to be wide-eyed, breathing fast, and often sweating and confused.
Night terrors are generally short-lasting, with complete amnesia about the event. They usually disappear with time, although they may persist into adolescence.
Gratification disorder
Gratification disorder, also called infantile masturbation, benign idiopathic infantile dyskinesia or paroxysmal dystonia, presents in boys and girls. It is often mistaken for epilepsy, abdominal pain, paroxysmal dystonia or dyskinesia. Onset is generally between the ages of three months and three years.
It usually occurs during periods of boredom, loneliness, excitement or anxiety. The child may be sitting in a car seat or high chair, lying on the floor or watching television.
These are stereotyped episodes of variable duration and the child can be flushed and sweaty. Most episodes in children lack the manual stimulation of genitalia, but have behaviours such as dystonic posturing, rocking, grunting, facial flushing with diaphoresis and posturing of the lower extremities, allowing pressure on the perineum.
The child will usually stop with distraction, which in turn leads to them becoming annoyed. There is no alteration of consciousness.
Counselling the family and reinforcing the message that this is a benign condition with spontaneous resolution is necessary to assist with a favourable outcome.
Dealing with funny turns
The history is the most crucial aspect of diagnosis in most cases. The GP should carefully evaluate all aspects of the episode in question, including events before, during and after.
The following points should be considered in the history when assessing these children.
- What is the age of onset?
- Is it one type, or different types?
- Detailed description, including sequence, periodicity, frequency, duration and evolution.
- Any possible precipitants or prodromal symptoms?
- Any relation to eating, place, activity, sleep, play, temperament?
- Is the child distractible?
- Any other signs, symptoms or autonomic changes?
- Is there a family history?
- Any developmental problems?
Investigations
The key investigation depends on the history and examination. Most of these children need no further tests. EEG should not be performed to rule out epilepsy and is only helpful if the episodes are strongly suspected to be epileptic, to support the diagnosis.
Home video recordings can be extremely helpful. Parents who have a smartphone can record an episode to show their GP at the next consultation. I often ask parents and adolescents to keep a diary of these events, documenting all of the details before, during and after.
Very rarely, patients may need a prolonged ambulatory EEG or videotelemetry.
Management
Most of these disorders are benign and do not require specific intervention. However, the GP may have to exclude serious cardiac problems and some patients may need more comprehensive tests. Reassurance is generally what is needed, because the family can become very anxious.
Conclusion
Faints and funny turns are very common in children, with a wide range of differential diagnoses, which can be both epileptic and non-epileptic.
History-taking and examination can dramatically increase diagnostic accuracy. Home video is helpful.
EEG should not be used as a tool to diagnose these non-epileptic spells. Most are age-related benign conditions which do not require specific treatment.
- Dr Beri is a consultant paediatric neurologist, Spire Bushey Hospital and Imperial College Healthcare NHS Trust, London
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Resources
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- DiMario FJ. The nervous system. In: Rudolph AM, Kamei RK, Overby KJ (eds). Rudolph's Fundamentals of Pediatrics (third edition). New York, Mcgraw Hill, 2002.
- Kotagal P, Costa M, Wyllie E et al. Paroxysmal nonepileptic events in children and adolescents. Pediatrics 2002; 110(4): e46.
- DiMario FJ. Prospective study of children with cyanotic and pallid breath-holding spells. Pediatrics 2001; 107: 2165-269.
- Wills L, Garcia J. Parasomnias: epidemiology and management. CNS Drugs 2002; 16: 803-10.
- Narchi H. Infantile masturbation mimicking paroxysmal disorders. J Pediatr Neurol 2003; 1: 43-5.
This is an updated version of an article that was originally published in January 2014.
