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Funny turns in small children
General Practitioner, Laindon, UK
University of Southampton, Southampton, UK
E-mail: caes{at}soton.ac.uk
| Abstract |
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Small children are often brought to the GP by their parents because they have had a funny turn. The GP must make a decision on the cause of the child's funny turn and appropriate action to take. This article presents a strategy which a GP in training can use to deal with such situations and describes the major conditions which form the differential diagnosis.
| What the GP curriculum says about funny turns in small children Diagnostic problems, such as funny turns in children form a key part of the GP curriculum statement 8: Care of children and young people. The GP curriculum requires GPs to:
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| History |
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When presented with a child who has had a funny turn, the major questions to ask yourself are:
- Was the episode a fit?
- If so, what caused it?
- If not, then is there another serious underlying cause such as heart disease?
A good history from a witness is essential. Ask when the attack happened, where the attack happened and what happened during the attack. In particular ask what the child looked like during the attack (for example colour and floppiness), whether the child lost consciousness and whether there was any jerking of the limbs and/or tongue biting. If so, ask whether the jerking was localized or restricted to one area of the body. Find out how long the attack lasted and what happened after the attack. Was the child conscious right away? Was there any disorientation or drowsiness afterwards? Explore whether there were any precipitating factors, for example viral illness or fever. Ask specifically whether the child hit his or her head and whether the child was upset or angry when the attack occurred.
The child's background history may also give clues to the diagnosis. Ask about the child's general health and any ongoing medical problems, in particular heart disease, neurological and/or developmental problems. Ask about the child's birth history including problems in pregnancy and birth trauma. Is there any family history of epilepsy?
| Examination |
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Perform a full examination. Check pulse rate and for any signs of heart disease. Do a full neurological examination. Remember to check developmental milestones and to plot the child's head circumference and weight on a centile chart.
| Non-epileptic attacks |
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These are usually self-limiting and harmless but can be very frightening for parents or carers. The differential diagnosis of non-epileptic attacks is listed in Box 1. Parental education about the likely duration and cause of attacks and reassurance that the child will come to no harm are important.
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Simple blue breath-holding attacks
Simple blue, breath-holding attacks are common and usually start over six months of age. They are provoked by frustration or upset. During an attack, a child holds his/her breath and turns blue. This is followed by generalized stiffening of the child's body and loss of consciousness. No treatment is needed and children always recover spontaneously. Most children grow out of the attacks by three years of age.
White reflex asystolic (anoxic) attacks
White reflex asystolic (or anoxic) attacks are caused by vagal asystole. They may start before the child reaches six months of age, but are most common between the ages of six months and two years. Attacks are usually triggered by minor injury or anxiety. During an attack the child becomes very pale and rapidly becomes unconscious. There may be some accompanying stiffening of the limbs and the child's eyes may roll upwards. If the child is dry, attacks may be accompanied by urinary incontinence. Children recover spontaneously and no treatment is needed.
Reflex syncope or vasovagal attacks (faints)
These are common and tend to affect older children – girls more than boys. Often the cause is not clear but known precipitants include fright (for example, when having blood taken), emotion and being unwell. During an attack there is peripheral vasodilation, bradycardia and venous pooling that result in postural hypotension.
History is characteristic. The child usually can say that he or she did not feel right before the attack. Typical prodromal symptoms include dizziness, visual disturbance, nausea, sweating, ringing in the ears, yawning and/or a sinking feeling. During the attack the child becomes extremely pale and falls to the ground unconscious. If the child is held upright, this may be accompanied by tonic-clonic jerking. The loss of consciousness is very short lived (usually seconds) and is followed by a rapid recovery. Diagnosis can usually be made on history. No treatment is needed.
Cardiac arrhythmias
These can be very difficult to detect as usually the arrhythmia is self-limiting and gone by the time medical help arrives. The NSF for Coronary Heart Disease recommends that all children with recurrent loss of consciousness or collapse on exertion are referred for paediatric cardiology assessment.
| Febrile convulsions |
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A febrile convulsion is a seizure occurring in a child aged between six months and five years of age (with a peak age of eighteen months), associated with fever arising from infection or inflammation outside the central nervous system, in a child who is otherwise neurologically normal. About 2–4% of all children have a febrile convulsion.
Febrile convulsions may be simple or complex. Simple febrile convulsions are isolated generalized, tonic-clonic seizures lasting under 15 min. Complex febrile convulsions last between 15 and 30 min or are focal, or recur during the febrile illness, or are not followed by full recovery within an hour of the convulsion. Most childhood infections can cause a febrile convulsion, with viral infections being the most common culprits. Convulsions may also occur after immunizations.
Assessment
By the time the GP arrives, the febrile convulsion is usually over, so diagnosis is based on a history of a short, generalized tonic-clonic seizure in a febrile child. The main concern when assessing children who have had a febrile convulsion is to detect and manage bacterial meningitis. Check temperature, assess level of consciousness and examine for a source of infection. If there is no obvious cause and the child is not being admitted, check a urine sample for urinary tract infection.
Complex febrile convulsions are more likely than simple febrile convulsions to be provoked by a serious condition. Suspect serious pathology and admit for further assessment if a child has:
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Management
Most children do not need admission. Admit any child who has had a febrile convulsion if:
- The child was drowsy before the seizure.
- The child is irritable, systemically unwell or toxic and/or the cause of the fever is unclear.
- Petechial rash.
- The child has any symptoms or signs of meningitis – headache, photophobia, stiff neck, bulging fontanelle, peripheral shutdown (capillary return of greater than 3 s), positive Kernig's sign (with hips fully flexed, the child resists passive knee extension), abnormal tone or posturing
- Recent/current treatment with antibiotics (may mask symptoms/signs of meningitis)
- Aged under 18 months (meningitis may present with non-specific signs)
- The cause of the fever requires hospital management in its own right
- Complex convulsion
- Early review by a doctor is not possible
- Inadequate home circumstances
- The parents or carer are anxious or unable to cope
For children not being admitted, reassure the parents or carers that febrile convulsions do not harm the child. Advise on controlling fever in the future with an antipyretic such as paracetamol or ibuprofen syrup and cool clothing. If these measures do not work, fanning the child or sponging with lukewarm water may help reduce the child's temperature. Teach parents to manage a recurrent convulsion by not forcing anything into the child's mouth and turning the child into the recovery position. Recommend that immunization schedules are completed. Advise the parents or carers to seek urgent medical help if the child deteriorates in any way, fits again or they are worried. Informatin for parents on febrile convulsions can be found in Box 2.
| Box 2. Information for parents about febrile convulsion Information for parents about febrile convulsions What are febrile convulsions? A febrile convulsion or fit happens when normal brain activity is disturbed when a child has a fever. It usually occurs without warning. During the fit your child may:
Febrile convulsions are frightening to watch, but they are not harmful to your child, don't cause brain damage, and will not cause your child to die What happens after a febrile convulsion? Your child will become tired for up to an hour after the fit. If your child remains sleepy or is difficult to rouse after sleep, seek medical attention What is the risk of recurrence after a febrile convulsion Febrile convulsions recur in subsequent febrile illnesses in around 30% of children - 9% have more than 3 seizures. Recurrence is most common in the year following the first febrile convulsion. Recurrence is more likely if:
Is there an increased risk of epilepsy after febrile convulsion? No-febrile convulsions are not epilepsy. 1% of children having a febrile convulsion go on to develop epilepsy (compared to 0.4% children who have not had a febrile convulsion). Risk is increased if any of the following features are present:
Are there long-term complications after febrile convulsions? Long-term adverse effects are rare. There is no evidence of subsequent impaired intelligence or poorer academic achievement but there is a slightly increased risk of epilepsy. Is immunization contraindicated after febrile convulsion? There is evidence to suggest immunizations do not increase risk of recurrent febrile convulsions. Immunization is not contraindicated. How can I prevent fevers which cause convulsions? Controlling fever eases symptoms. It does not prevent febrile convulsions. A high temperature can be reduced by:
What should I do if my child has another convulsion? Remember, most fits stop within a couple of minutes without treatment.
Call an ambulance if:
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Arrange early review later the same day or the following morning.
Consider referral to paediatrics or paediatric neurology if the diagnosis of febrile convulsion is in doubt or if prophylactic medication might be indicated. For example if the convulsions have been frequent, severe or complex. Also refer for review if the child is at increased risk of epilepsy, for example if the child has co-existant neurological or developmental conditions or if there is a history of epilepsy in a first degree relative. Parental request or anxiety despite reassurance is also a valid reason to refer.
Prophylactic measures should be prescribed under consultant supervision only. Rectal diazepam may prevent febrile convulsions in subsequent illness if given at the onset of a febrile episode. Rectal diazepam is safe for home use provided parents are properly educated in its use. Use of anticonvulsants on a regular basis to prevent febrile convulsions is controversial. In general benefits are outweighed by risks.
| Childhood epilepsy |
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Childhood epilepsy is a susceptibility to continuing seizures. Prevalence increases with age from around 4 in 1000 children at 7 years of age to around 5 in 1000 children by 16 years. Sixty per cent of adult epilepsy starts in childhood. Risk factors include severe learning difficulty (one in three have epilepsy), multiple disabilities, family history and febrile convulsions (1% go on to develop epilepsy).
| Always refer to a specialist paediatrician with training and expertise in epilepsy for diagnosis. All children who have had a first non-febrile seizure should be seen within two weeks.
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Classification
Seizures that begin in one area of the brain only are termed partial seizures. Partial seizures can be simple if there is no impairment of consciousness or complex if consciousness is impaired. If the whole brain is involved a seizure is termed generalized. During generalized seizures consciousness is usually, but not always impaired. Examples of generalized seizures are absence attacks and tonic-clonic seizures (in which the child loses consciousness and the limbs stiffen and jerk). Partial seizures may become generalized.
In children, epilepsy is also considered in terms of epileptic syndromes. Identifying a syndrome enables predictions about cause, severity and prognosis. Epileptic syndromes are characterized by a set pattern of seizure type(s) and other features such as physical appearance, the child's age at onset, family history, associated learning disability or developmental delay, associated neurological abnormalities and electro-encephalogram (EEG) pattern (all children with a history of two or more epileptic seizures should have an EEG). It is not possible to identify a syndrome in 30%, and symptoms and signs may take months to evolve until diagnosis can be made in others.
There are a large number of childhood epileptic syndromes. Table 1 lists some of the more common ones. Broadly they divide into benign and malignant syndromes. Children with benign syndromes are often only mildly affected, their fits are easily controlled and they may mature out of their epilepsy. Children with malignant syndromes have treatment resistant epilepsy with severe or progressive motor and/or intellectual disability.
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Benign rolandic epilepsy
Benign rolandic epilepsy is also known as childhood epilepsy with centrotemporal spikes. Clonic, partial sensorimotor attacks affect the face, tongue, pharynx, hand and arm. Attacks are most common on falling asleep (and more than half only have seizures during sleep) or soon after waking. Secondary generalization to tonic-clonic seizures may occur. EEG is characteristic. Benign rolandic epilepsy starts in children aged 2–12 years with a peak age of 7–10 years. Usually attacks have stopped by 13 years. Frequently there is a family history. Use of drug treatment depends on frequency and severity of seizures.
Juvenile myoclonic epilepsy (JME)
Juvenile myoclonic epilepsy presents with sudden, brief, bilaterally symmetrical and synchronous involuntary muscle contractions affecting the upper more than lower body. These attacks may cause the child to inexplicably throw objects or fall. Consciousness is often maintained. Attacks frequently occur soon after waking. Triggers may include light (50% of children), tiredness, or emotion. EEG is characteristic. Juvenile myolconic epilepsy starts in children or young people aged 8–24 years with a peak age of 10–16 years. About half have a family history of epilepsy. The condition progresses so that 90% of sufferers develop generalized tonic-clonic seizures within four years of diagnosis. Absence seizures also occur in 15–30% of patients with JME and may be a presenting feature. Lennox-Gastaut syndrome is a severe early onset form of myoclonic epilepsy which starts at age 2-6 years with intractable seizures and a typical EEG.
Children with JME are usually treated with sodium valproate but fits may not be well controlled with medication. JME does not remit spontaneously. Lifelong medication is needed as relapse rate is around 90% on withdrawal of anti-epileptic medication.
Absence seizures (petit mal epilepsy)
During an absence seizure, the child stops what he or she is doing and may stare into middle-space for a period of seconds (mean time 4–20 seconds). Attacks can occur 50–100 times every day and deterioration in school performance may be the presenting feature. Separating absence attacks from daydreaming can be difficult. Attacks start between the ages of 4–10 years, with peak age of onset 5–7 years. Absence attacks affect girls more often than boys and 15% have a family history. EEG is characteristic. About 15% of children with absence attacks go on to develop JME and 10% (without other adverse factors) have absence or tonic-clonic seizures in adult life. Eighty per cent become seizure-free with sodium valproate.
Localization-related epilepsies
Localization-related epilepsies present with partial (focal) seizures. They may be due to an identifiable underlying cause (such as a brain haemorrhage in a child born prematurely) when the seizures are termed symptomatic, or a cause may not be found in which case the seizures are termed cryptogenic. Clinical features, disabilities and prognosis depend on any identified cause and the location of the brain abnormality.
Infantile spasms (West's syndrome)
Infantile spasms start in the first year of life (peak age of 4 months). Runs of tonic spasms – usually flexion spasms or salaam spasms – occur every 5–10 seconds. There is a typical EEG. Infantile spasms are associated with loss of vision and social interaction. Treatment is with steroids and anti-epileptics (usually vigabatrin). Children with infantile spasms generally have a poor prognosis: 30–50% have cerebral palsy; 85% have a cognitive disability and there is a 20% death rate.
Management of childhood epilepsy
A paediatrician or a paediatric neurologist, under shared care protocols with primary care, should oversee ongoing management of any child with epilepsy. Epilepsy is a diagnosis that can cause great alarm and fear. It affects not only the child, but the whole family. The holistic care domain of the GP Curriculum for care of children and young people requires GPs in training to consider the impact of childhood epilepsy on the family and support the child and parents. Education is very important. Good leaflets are available from Epilepsy Action. Parents and patients need clear information on what to expect, what to do during an attack, avoiding risks (such as swimming or cycling alone) but not being overprotective, the importance of compliance with medication and when drug withdrawal can be considered if the child is fit free.
Drug treatment is the mainstay of epilepsy management. For children, antiepileptic treatment is less well supported by clinical trial data than for adults, and drugs are more likely to be used off-licence. There is controversy about when to treat. NICE recommends drug treatment should begin after the second seizure except in specific circumstances. The drug chosen is matched to the individual patient and type of epilepsy - this is a specialist decision. Often the most suitable drug can only be established by trial and error. Review treatment at regular intervals to ensure that children are not maintained on treatment that is ineffective or poorly tolerated, and that concordance with prescribed medication is maintained. Compliance with medication can be a particularly difficult problem with adolescents. This may be because the drugs have unacceptable side effects for them, or may be due to rebellion against their diagnosis and the limitations it puts upon them.
Other treatments used in the treatment of childhood epilepsy include surgery and special diets. Surgery, such as lesionectomy, is increasingly being used for childhood epilepsy. It is useful for intractable partial seizures, hemiepilepsy and epilepsy with focal EEG and/or radiological features. Ketogenic diet is effective for some children with refractory epilepsy.
GPs have an important role in the management of children with epilepsy, even though most of the day-to-day management of the epilepsy itself is done by the specialist secondary care team. It is important to support the child and family and ensure receipt of all available benefits. Liaise with the primary healthcare team, community, educational and specialist services to ensure prompt provision of equipment, support and services. Make referrals for new problems promptly and tell carers about local facilities, voluntary and self-help organizations. Specific problems that should be considered whenever you see a child with epilepsy are listed in Box 3.
Box 3. Particular problems to look for in children with epilepsy
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Mortality
Patients with epilepsy overall are 2-3 times more likely to die than people of the same age without epilepsy. Children with symptomatic epilepsy and those with learning or physical disabilities are particularly at risk. Some deaths are related to an underlying condition, accidents, or status epilepticus. Sudden unexplained death in epilepsy (SUDEP) is probably due to central respiratory arrest during a seizure.
| Information and support for patients and parents: |
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Epilepsy Action Tel: 0808 800 5050 Website: www.epilepsy.org.uk
West's syndrome support group Website: www.wssg.org.uk
| References |
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Van Dorp F, Simon C. Oxford GP Library: Child Health (2007) OUP ISBN: 0199215685.
National Service Framework for Coronary Heart Disease. (updated 2005). Accessed via http://www.dh.gov.uk/en/Policyandguidance/Healthandsocialcaretopics/Coronaryheartdisease/DH_4117048 [date last accessed 29.11.07].
NICE. The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care (2004) Accessed via http://www.nice.org.uk/guidance/index.jsp?action=byID&o=10954 [date last accessed 29.11.07].
NICE. Newer drugs for epilepsy in children (2004) Accessed via http://www.nice.org.uk/guidance/index.jsp?action=byID&o=11532 [date last accessed 29.11.07].
NICE. Referral guidelines for suspected cancer - quick reference guide (2005) Accessed via http://www.nice.org.uk/nicemedia/pdf/CG027quickrefguide.pdf [date last accessed 29.11.07].
Offringa M, Moyer VA. Evidence based management of seizures associated with fever (2001) 1111–4.
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