Case 15 Fits, faints and funny turns

A 15-year-old girl called Nina is referred to outpatients with a 2-year history of abnormal episodes. The GP originally diagnosed them as being vasovagal but she is seeking advice because they have changed recently. The consultant general paediatrician documents the history from Nina and her mother. Nina says some are predictable and occur when she stands up too quickly and this can make her feel hot, sweaty and dizzy. The next thing she remembers is waking up on the floor with staff or friends expressing concern. She recovers after about 10 minutes. She had a minor head injury during one episode. Her mother describes her as ‘ghostly white’ and states that she has had occasional, brief (10 seconds), symmetrical shaking of all 4 limbs. However, in the past 6 months Nina has had at least 12 new episodes that her family have never witnessed. She has no warning, but school staff and friends say that she stiffens all over, loses awareness and ‘drops to the floor like a rag doll’. Sometimes she has jerking movements of her legs. There is no colour change. She has bitten the tip of her tongue twice but there has been no other injury and no incontinence. She is back to normal within minutes.

Nina is making good progress in Year 10 and has a stable group of friends. She has missed some school and has been banned from sport because of these episodes. Apart from menorrhagia and irregular periods there is no other history of note. Examination is normal including fundoscopy and lying and standing blood pressures.

Does she have epilepsy?

The consultant agrees that the first episodes were vasovagal but is concerned that the new episodes are seizures. An ECG, EEG and MRI scan are normal. He asks the family to keep a diary, and at review 6 weeks later there have been another 8 events, all either at school or when out with her friends. He makes a diagnosis of epilepsy, prescribes sodium valproate 1g bd and makes an appointment for 6 months.

Was this reasonable management?

At the next appointment Nina’s family report severe drowsiness at the start of treatment with 3 further weeks off school. The frequency and pattern of the episodes is unchanged. The consultant concludes that these are myoclonic seizures and adds clonazepam. Nina reports that her periods have become even more irregular and that she has not had a bleed for 4 months. She denies being sexually active. The consultant explains that sodium valproate can cause menstrual irregularity due to the polycystic ovary syndrome.

What would you have done?

Six months later Nina returns to clinic with her parents and her 6-week-old baby boy. He has significant congenital abnormalities – Tetralogy of Fallot, cleft palate and hypospadias – picked up on a late antenatal scan at 29 weeks gestation when she realized she was pregnant. Nina stopped both drugs immediately and has had no episodes since.

The family file a lawsuit claiming that the consultant was not an expert in epilepsy and should not have treated their daughter. In particular, they complain that Nina should have been warned about the potential teratogenic effects of sodium valproate despite her denial (in front of her mother) of being sexually active.

Expert opinion

A diagnosis of epilepsy is a life-altering event for any patient and family. It is made primarily from the history. Fits, faints, jerks and blackouts are common in children but almost half of those referred to a tertiary paediatric neurologist with a suggested diagnosis of epilepsy do not have the condition. It is crucial to get it right but it is often difficult. An EEG can support the clinical diagnosis, determine the seizure type and help with treatment. A MRI scan may identify structural abnormalities that cause certain, usually focal, epilepsies. Neither diagnose epilepsy. Nina’s first episodes were clearly vasovagal but the second episodes were almost certainly pseudo-seizures, not an uncommon diagnosis in teenage girls. Particular pointers are that, despite their frequency, they had never been witnessed by the family, the lack of injuries and biting the tip of the tongue – patients are aware that people often ‘bite their tongue’ during a fit, but do not realise that it is never the tip.

There are comprehensive NICE guidelines for the management of epilepsy in children and young people with one of the key recommendations being that all children with a recent-onset suspected seizure should be seen urgently by a specialist. The latter was defined as ‘a paediatrician with training and expertise in the epilepsies’. The British Paediatric Neurology Association has developed Paediatric Epilepsy Training (PET) courses and it would now be considered bad practice for a child to be managed by a doctor in secondary care who has not completed at least a PET 1 course. Making the diagnosis is rarely urgent and it is more important to obtain witnessed accounts and video evidence and to refer to a tertiary neurologist if there is continued doubt. Having made the wrong diagnosis, the doctor compounded his errors by incurring severe side effects with a huge initial dose of valproate which lead to significant sedation, delaying a review for 6 months and then using polypharmacy instead of following the principles of monotherapy. There was no involvement of an Epilepsy Specialist Nurse and the doctor failed to counsel Nina who is of child-bearing age. Despite her public denial, the risk of her being sexually active was significant and there should have been a confidential discussion to ensure that she understood the risks of sodium valproate and the need to plan a pregnancy. All staff dealing with children with chronic diseases must have disease-specific and generic skills to manage and communicate with adolescents. This doctor failed to recognize their limitations and that they were practising outside their field of expertise.

Legal comment

This could be a complicated legal case. Nina may be able to make a claim for what could be termed an unwanted pregnancy (in the sense that she may have sought a termination, if the pregnancy had been diagnosed sufficiently early and the foetus’ abnormalities had been explained to her).

However, the claim of Nina, the mother of the baby, is academic. The fact is that the paediatrican should have referred Nina to a paediatric neurologist or to a paediatrician with a special interest in epilepsy; his failure to do this amounts to a breach of duty. If he had done this, then Nina would not have been diagnosed as suffering from epilepsy and the sodium valproate would not have been prescribed. It is likely that an instructed expert will conclude that her baby’s abnormalities were caused by the sodium valproate.

On this analysis, the baby will have a valid claim against the hospital in his own right; he was damaged in utero by the negligence of the doctor. Experts will need to examine the baby to judge the extent of his disabilities and how they will affect him in his future life. The claim could be worth several hundred thousand pounds, especially if the instructed experts conclude that the baby suffers from developmental delay as a result of the sodium valproate.