Guide ABC of Epilepsy (ABC Series)

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Seconds Figure 2. Secondarily generalised seizures are partial seizures, in which the epileptic discharge spreads to both cerebral hemispheres, so that a tonic-clonic seizure convulsion ensues. The patient may experience and recollect an aura, but this is not always the case if the seizure spreads rapidly. Generalised seizures The commonest of these are the tonic-clonic seizure and absence seizures, but there are also rarer seizure types that we will discuss.

Generalised tonic-clonic seizures GTCS; often termed convul- sions usually occur without warning, although sometimes they can be preceded by increasing frequency of another generalised seizure type, such as myoclonic jerks or absences. Initially the person is tonic stiff and may cry out the 'epileptic cry'.

The person will fall and may bite the side of the tongue as the jaw clenches. The person may also become cyanosed at this point. Clonic movements then begin, usually predominantly in the upper limbs.

These are coordinated regular jerks that eventually slow and stop, at which point incontinence can occur. Most convulsions last less than 2 minutes. There is then a post-ictal period characterised by sleepiness and confusion lasting up to 20 minutes, but it may take longer for people to get over the full effects of the seizure including lethargy, muscle aches, headache and a severely bitten tongue. Typical absence seizures almost always begin in childhood or adolescence. There is motor arrest and staring. On occasions there can be fluttering of the eyelids, swallowing, and flopping of the head.

The attacks usually last a few seconds, can occur many times a day and may be unrecognized, leading to a delay in diagnosis. There is immediate recovery and no post-ictal phase. This seizure type is associated with a characteristic EEG of three-per-second generalised spike-and-wave discharges Figure 2. Typical absences need to be differentiated from complex partial seizures, which can sometimes also present as blank spells. There are also atypical absences, which are usually part of more severe epilepsy syndromes associated with learning difficulties, such as the Lennox-Gastaut syndrome see below.

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Also the onset and cessation of the seizure is less clear and there are often additional features such as changes in body tone. Myoclonic seizures are sudden jerks that can involve a part of or the whole body. They commonly occur in the morning within a couple of hours of waking in idiopathic generalised epilepsies see below. Although they usually occur in more benign epilepsy syn- dromes, they can rarely be associated with devastating epilepsies with cognitive and neurological decline - the progressive myoclonic epilepsies.

Not all myoclonus is epileptic and can even be physio- logical, for example sleep starts hypnic jerks. Other generalised seizures consist of mainly atonic and tonic seizures. These are often termed 'drop attacks' and consist of sudden loss of body tone atonic or sudden increase in body tone tonic , resulting in a fall. Recovery is generally rapid, notwithstanding any head injury. These seizures usually occur in more severe epilepsies with associated learning difficulties such as the Lennox-Gastaut syndrome.

Classification of epilepsy syndromes An epilepsy syndrome consists of a combination of clinical, seizure and EEG characteristics that make up a distinct entity. Diagnosis of an epilepsy syndrome has implications for prognosis and manage- ment.

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However, diagnosis of a particular epilepsy syndrome does not necessarily imply a single aetiology: many epilepsy syndromes are known to have multiple aetiologies. Some epilepsy syndromes are characterised by generalised seizures, others by focal seizures, and a few by both focal and generalised seizures. The first two of these can be considered generalised and focal epilepsy syndromes, respectively. Some epilepsy syndromes are always or nearly always idiopathic; others are always or nearly always symptomatic or probably symptomatic, but a number exist in idiopathic, probably symptomatic and symptomatic forms e.

West syndrome. The term epileptic encephalopathy is used to denote epilepsies in which ongoing epileptic activity either overt as clinical seizures or covert with ongoing epileptiform EEG discharges without obvious clinical seizures gives rise to progressive but potentially reversible neurological dysfunction which can be manifested as learning and behavioural problems or occasionally as motor problems.

Only a very small number of epilepsies follow this pattern. This is because the seizures arise in the context of an external provoking factor fever. They are epileptic seizures occurring in association with fever but without evidence of infection of the CNS, excluding those who have had previous non-febrile seizures or in whom there is a known cause of seizures.

Epilepsy and seizure prediction

They usually occur between the ages of six months and six years. A family history is common. Most febrile seizures are GTCS, but other seizure types can be precipitated by fever, including tonic, clonic and myoclonic seizures. It is usual to classify febrile seizure as simple or complex. The latter implies that the seizure had focal features, was prolonged usually defined as lasting 15 minutes or longer or was repeated more than once during the same febrile illness.

In most children febrile seizures are self-limiting and benign. However, febrile status is the commonest cause of convulsive status epilepticus in children. One-third of children will experience one or more further febrile seizures. The risk of epilepsy is increased in children who have had a febrile seizure. Risk factors for developing epilepsy after febrile seizures are family history of epilepsy, complex febrile seizures, and pre-existing neurodevelopmental problems. Many different types of epilepsy can follow febrile seizures.

There is particular interest in the risk of temporal lobe epilepsy following prolonged febrile seizures. Idiopathic generalised epilepsies These are common forms of epilepsy occurring in childhood, ado- lescence and in adult life, and feature combinations of three seizure types: typical absences, myoclonic seizures and GTCS. Despite reports of linkage to a variety of chromosome regions and of specific mutations in individuals and families, their genetic basis is still unclear.

Childhood absence epilepsy is common in paediatric practice and usually starts between the ages of four and nine years.

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It is more common in girls than boys. It is characterised by typical absence seizures, usually lasting less than 20 seconds and occurring many times each day. The EEG is characterised by generalised 3 -Hz spike-and-wave discharges.

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Response to appropriate medication is usually excellent, and most children become seizure free. The epilepsy commonly resolves, usually before age 12 years. However, a minority continue to have absences, and often develop GTCS. Juvenile absence epilepsy is similar to childhood absence epilepsy, but is rarer and usually starts in later childhood or adolescence.

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The typical absence seizures occur less frequently, but GTCS are common and occasional myoclonic jerks may also occur. Describing and Classifying the Condition Response to appropriate treatment is generally good, but the con- dition is likely to persist throughout adolescence and into adult life. Juvenile myoclonic epilepsy is also common and probably under diagnosed. It usually begins between the ages of 10 and 18 years and has a genetic basis.

Although the seizures can be well controlled with appropriate medication, the condition is usually lifelong. Benign myoclonic epilepsy of infancy is rare, usually starting between six months and three years of age. The predominant, often sole, seizure type is myoclonic seizures occurring singly or in clusters. They may arise spontaneously or be provoked by noise or tactile stimuli.

Remission commonly occurs between six months and five years after onset. A minority of children develop other seizure types and have educational problems. Patients with Doose syndrome have myoclonic-astatic seizures; this starts in early childhood and is characterised by drop attacks caused by myoclonic seizures, atonic seizures seizures characterised by a loss of muscle tone or a combination of these seizure types myoatonic seizures , and piratically induced eyelid myoclonia with absences.

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