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Fits, Faints and Funny Turns in Childhood

Fits, Faints and Funny Turns in Childhood

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Introduction

Medical staff are often not present to witness paroxysmal events - 'fits, faints and funny turns' - so the diagnosis relies on the description of an eyewitness observer or the child themselves if they were aware of what was happening.

Always take a detailed history from the eyewitness. A thorough history often reveals the diagnosis without needing further investigations. A video of the event would be very useful, so ask the parents to record the episodes.

Although these events usually have a stereotype attached to them, most of the time they are not seizures. This article covers the assessment and identification of fits, faints and funny turns in children.

History

Give each type of episode an identifying name. For each type it is important to ask about frequency, timing, relationship to sleep, triggers, warning beforehand, duration, colour changes, changes to conscious level, motor phenomena, recovery and symptoms following the event, and family history.

Seizures and epilepsy

A seizure is a short-term occurrence of signs and/or symptoms caused by abnormal, excessive, or synchronous neuronal activity in the brain.1

Epilepsy is a brain disease which can be defined by one or more of the following conditions: two unprovoked (or reflex) seizures occurring more than 24 hours apart; 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; and/or diagnosis of an epilepsy syndrome.

Therefore, a diagnosis of epilepsy requires that the seizures are recurrent and unprovoked (i.e. without a temporary or reversible factor lowering the seizure threshold).

Epilepsy vs acute symptomatic seizures

Acute symptomatic seizures are triggered by acute insults, including hypoglycaemia, hypoxia, electrolyte imbalances, infection, and trauma. Look for any known triggers in the history, and consider the factors leading up to the episode rather than what happened after it.

These types of seizures may also happen more than once (e.g. recurrent febrile seizures).

The role of the electroencephalogram (EEG)

Routine use of EEG is limited. False-positive rates are high and inter-ictal EEGs are usually normal in epileptic patients.

EEG can be useful to identify the kind of seizure occurring and the epilepsy syndrome.

Different Types of Seizures

Seizures can be classified into jerk/shake, stiffness, falls, and vacancy types.

  • Jerk/shake:
  • Clonic -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.
  • Stiffness: Tonic -a prolonged period of contraction of one or several muscle groups.
  • Falls:
  • Atonic -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.
  • Vacancy:
  • Absence -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 based on age of onset, seizure types, EEG findings, neurodevelopmental outcomes, response to anti-epileptic drugs, and more. It's important to understand that underlying pathology can manifest differently depending on the maturation status of the brain. Diagnosis takes time and specialist input and the following are common paediatric epilepsy syndromes.

Infantile Spasms (West Syndrome)

Age of onset:

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

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

Age of onset:

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

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 Epilepsy

Age of onset:

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

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)

Juvenile myoclonic epilepsy (JME) typically begins in those aged 12 to 18 years. Common clinical features include the following:

  • Frequently begins with a generalized tonic-clonic seizure
  • Myoclonic jerks often precede generalized tonic-clonic seizures; awareness is usually retained during these jerks
  • Absences may be present in up to one third

Electroencephalogram (EEG) often shows polyspike discharges that are followed by irregular 1-3 Hz slow waves.

Non-Epileptic Paroxysmal Episodes

Episodes Occurring in Sleep

Benign Neonatal Sleep Myoclonus

These episodes occur in healthy infants, typically a few weeks old, and they are limited to sleep. Myoclonic movements involve the body, but not the face. EEG will normal.

Parasomnias

Parasomnias include sleep paralysis, confusional arousal, sleepwalking, and night terrors. These episodes tend to recur and are confined to sleep.

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 connected to feeding are collectively called Sandifer syndrome.

Episodes Triggered by Fever

Febrile Seizures

Febrile seizures are associated with fevers but have no identifiable intracranial cause. They typically present as brief, generalized convulsive seizures. These episodes commonly occur in children aged 6 months to 6 years of age and can recur in subsequent febrile illnesses. Complex febrile seizures, which are prolonged, focal, or repeated in the same illness, have a 4-12% risk of leading to epilepsy.

Vaso-vagal Syncope

Vaso-vagal syncope typically affects older children. Triggers may include intercurrent illness, heat, low food and water intake, and prolonged standing. Symptoms often include paleness, nausea, visual greying, dizziness, and falling to the ground with some jerking and urine incontinence. Recovery is usually rapid.

Episodes Triggered by Pain, Shock, or Startle

Reflex Asystolic Syncope / Reflex Anoxic Seizure

Reflex asystolic syncope or reflex anoxic seizure is caused by sudden and unexpected pain or discomfort, such as banging the head. Features include paleness, hypotension, and unconsciousness as well as some limb stiffening or clonic jerking. These occur due to a vagally-mediated severe bradycardia or asystole and are self-limiting, requiring no treatment.

Episodes Triggered by Frustration or Stress

Children often grow up to become adults who faint at the sight of blood or needles. One common example is cyanotic breath-holding, where an extremely upset and frustrated toddler is unable to stop crying and experiences prolonged end-expiratory apnoea. As a result, they may turn blue, become limp and lose consciousness for a brief period.

Episodes Triggered by Sudden Noises or Touch

Hyperekplexia is an example of an episode triggered by a sudden noise or being touched and handled. The severe neonatal form can result in life-threatening apnoea and can be terminated by forcibly flexing the neck.

Episodes Triggered by Tiredness, Boredom or Stress

Self-gratification behavior is a comforting phenomenon observed in toddlers and children with neurological disabilities. It typically occurs in a highchair or car seat, and older children may lie prone or supine with their legs adducted and crossed as they become unresponsive. Tics are another type of episode that can be triggered by tiredness, boredom or stress, and are characterized by compulsive movements or gestures. Daydreaming, which may be confused with tics, occurs with boredom or while watching a screen and is characterized by a child not initially responding to their name being called and appearing "vacant."

Episodes Triggered by Excitement

Shuddering spells bring on shivering when excited by a toy or food, as if the child were chilled. This is generally benign. Cataplexy is an episode triggered by strong emotion, particularly laughter, which can cause a temporary loss of tone, even subtly with a brief sagging of the legs. This is associated with sleep-wake fragmentation.

Differential Diagnosis of Epilepsy in Children, Young People and Adults

Epilepsy is a neurological condition which results in seizures. It is important to have an accurate diagnosis of epilepsy in order to provide the right treatment and care. Differentiating epilepsy from other conditions can be challenging. This article will discuss the differential diagnosis of epilepsy in children, young people and adults.

For adults, differential diagnoses can include transient ischemic attack (TIA), syncope, psychogenic non-epileptic seizures, movement disorders, and sleep disorders. For children, the most common differential diagnoses are febrile seizures, migraine, sleep disorders, transient ischemic attack (TIA), syncope, and psychogenic non-epileptic seizures (conversion disorder).

There are several tools that can be used to help differentiate between epilepsy and other conditions. These include electroencephalograms (EEGs), brain imaging studies, and blood tests. An electroencephalogram is a test that records the electrical activity of the brain and can help diagnose epilepsy. Brain imaging studies such as magnetic resonance imaging (MRI) and computed tomography (CT) scans can help identify structural abnormalities in the brain. Blood tests can help rule out other conditions such as metabolic disorders.

It is important to consider the patient's medical history, family history, and lifestyle factors when making a diagnosis. The presence of a family history of epilepsy, head trauma, or a history of seizure-like activities can help to differentiate between epilepsy and other conditions. Lifestyle factors such as drug and alcohol use, sleep deprivation, and stress can also increase the risk of epilepsy.

It is important to be aware of the differential diagnoses of epilepsy in order to accurately diagnose and provide the best care for the patient. Further information on the differential diagnosis of epilepsy in children, young people and adults can be found in Appendix D of the Clinical Guidance CG137.

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