Idiopathic Epileptic Seizures and Syndromes in Infancy

CP Panayiotopoulos

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Panayiotopoulos CP. The Epilepsies: Seizures, Syndromes and Management. Oxfordshire (UK): Bladon Medical Publishing; 2005.

The Epilepsies: Seizures, Syndromes and Management.

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Chapter 6Idiopathic Epileptic Seizures and Syndromes in Infancy

The idiopathic epileptic seizures (that do not require a diagnosis of epilepsy) and idiopathic epileptic syndromes, which start mainly in the infantile (or very early childhood) period are listed below:¹^,²

Conditions with Epileptic Seizures That Do Not Require a Diagnosis of Epilepsy

Febrile seizures
Benign familial infantile seizures^*
Benign infantile seizures (non-familial)^*

Idiopathic Epileptic Syndromes

Generalised epilepsy with febrile seizures plus (GEFS+) (autosomal dominant epilepsy with febrile seizures plus)^**
Benign myoclonic epilepsy in infancy

Febrile Seizures

Patient note
Convulsions occur to children if acute fever be present… These complaints occur most readily to children, who are very young up to their seventh year; older children and adults are not equally liable to be seized with convulsions…
Hippocrates in part 24 of Prognostics 400 bc.

Clinical note
Febrile seizures^5–20 (a term preferred to its synonym febrile convulsions) are due to an age-related and predominantly genetic benign susceptibility to epileptic fits precipitated by fever of at least 38°C without evidence of intracranial infection or other cause. Children who have suffered a previous non-febrile seizure are excluded.

Demographic Data

Febrile seizures are the most common seizure disorder in childhood. The age at onset is from 6 months to 5 years with a peak at 18–22 months.⁸^,¹²^,¹³ Onset outside this age range (6 months to 5 years) is not acceptable for the pure forms of febrile seizures (see febrile seizures plus on page 128)

Boys (60%) are slightly more affected than girls.

The prevalence is approximately 3%, but this is higher in certain ethnic groups such as Japan (7%) and Guam (14%). The annual incidence is 460 per 100 000 children in the age group 0–4 years.²¹

Clinical Manifestations

Patient note
One minute he was a little boy with a cold and slight fever, lying on the sofa feeling miserable and the next his body was madly convulsing … It was very scary.
From an Internet description by a father

The body temperature that is accepted for the term ‘febrile’ is at least 38°C (others prefer 38.5°C) rectal temperature. Though a rapid rise in temperature is assumed to be a trigger, the temperature level may be more important.²⁸ One-fifth (21%) of febrile seizures occur within 1 h of the onset of fever, while 57% occur between 1 and 24 h, and 22% after more than 24 h from the onset of fever.¹⁷

Types of Febrile Seizures

Generalised tonic clonic seizures (GTCS) are by far the commonest seizure type (80%). Tonic (13%), atonic (3%), and unilateral or focal onset tonic clonic seizures (4%) may occur in the remaining 20%. On rare occasions, seizures consist of staring accompanied by stiffness or floppiness, rhythmic jerking movements without prior stiffening, focal stiffness or jerking only. Myoclonic jerks or absences are not part of febrile seizures. Repetitive seizures in the same febrile illness occur in 16%.

Because of different prognostic implications, febrile seizures are categorised into simple and complex febrile seizures.

Simple Febrile Seizures

Simple febrile seizures (70% of all) are generalised tonic clonic convulsions with a duration of less than 15 min and without recurrence within the next 24 h (or within the same febrile illness).

According to the 1999 practice parameter of the American Pediatric Academy¹²^,¹³ simple febrile convulsions are strictly defined as occurring in neurologically healthy children between 6 months and 5 years of age whose seizure is brief (less than 15 min), generalised and occurs only once during a period of 24 h during a fever. Children whose seizures are attributable to a central nervous system (CNS) infection and those who have had a previous non-febrile seizure or CNS abnormality are excluded.

ILAE and Other Formal Definitions for Febrile Seizures

The 1989 ILAE Classification categorised febrile seizures amongst the ‘situation-related seizures’ (which is synonymous to ‘conditions with epileptic seizures that do not require a diagnosis of epilepsy’ of the ILAE Task Force)² and defined them as follows.

Febrile convulsions are an age-related disorder almost always characterised by generalised seizures occurring during an acute febrile illness. Most febrile convulsions are brief and uncomplicated, but some may be more prolonged and followed by a transient or permanent neurological sequel, such as the hemiconvulsion–hemiplegia–epilepsy syndrome. Febrile convulsions tend to recur in approximately one-third of affected patients. Controversy about the risks of developing epilepsy later has largely been resolved by some recent large studies. The overall risk is probably no more than 4%. The indications for prolonged drug prophylaxis against recurrence of febrile convulsions are now more clearly defined and most individuals do not require prophylaxis. Essentially, this condition is a relatively benign disorder of early childhood.¹ In the 1980 Consensus Development Conference on Febrile Seizures, National Institutes of Health, febrile seizures were defined as follows.²²^,²³

A febrile seizure (an abnormal, sudden, excessive electrical discharge of neurones (grey matter) which propagates down the neuronal processes (white matter) to effect an end organ in a clinically measurable fashion) is an event in infancy or childhood, usually occurring between 3 months and 5 years of age, associated with fever, but without evidence of intracranial infection or defined cause. Seizures with fever in children who have suffered a previous non-febrile seizure are excluded. Febrile seizures are to be distinguished from epilepsy, which is characterised by recurrent non-febrile seizures.²²

The working group of the Research Unit of the British Paediatric Association⁸ considered that it was proper to use the term ‘epileptic’ instead of ‘event’ (used by the Americans)²²^,²³ and to distinguish ‘convulsions with fever’ from ‘febrile convulsions’. Accordingly, for the purposes of clinical management the term febrile convulsions should be limited to an epileptic seizure occurring in a child aged from 6 months to 5 years, precipitated by fever arising from infection outside the nervous system in a child who is otherwise neurologically normal. The working group considered that it was proper to use the term ‘epileptic’ insofar as the neurophysiological substrate of a febrile convulsion is a paroxysmal neuronal discharge, as in an epileptic seizure. Among children who have convulsions with fever are those with pyogenic or viral meningitis, herpes simplex encephalitis, other acute encephalitides, cerebral palsy with intercurrent infection and metabolic or neurodegenerative disease with a seizure precipitated by fever. Children who have a prolonged seizure or who have not completely recovered within 1 h should be suspected of having one of these conditions and investigated accordingly.⁸

Febrile status epilepticus of longer than 30 min is either one long-lasting seizure or a series of shorter seizures, without regaining consciousness inter-ictally.⁶^,^24–27

Complex Febrile Seizures

Complex febrile seizures (also known as ‘complicated’ or ‘atypical’) are defined by seizures that may be prolonged or repetitive, or seizures that have focal features. One-third of all febrile seizures may have one, two or all three of these complicating factors. ⁵^,⁶^,²⁹^,³⁰

Prolonged febrile seizures are those which last for more than 15 min (8%).³¹
Repetitive febrile seizures occur in clusters of two or more within 24 h (11²⁹–16%⁵).
Focal febrile seizures are those with a focal onset or those occurring in children with perinatal psychomotor deficits (3.5–7%). These also include those with the exceptional Todd’s postictal paresis (0.4%).

Complex focal seizures of repetitive or prolonged character are probably easy to diagnose.³² However, focal elements and particularly lateral eye deviation, staring episodes and motor asymmetries in the context of a bilateral convulsion may not be easy to recognise and categorise.³²

Duration of Febrile Seizures

In 92% of cases seizures are brief, lasting 3–6 min or less than 15 min. In the other 8% the convulsions are longer than 15 min and two-thirds of these long seizures progress to febrile status epilepticus. Prolonged febrile seizures may constitute the initial stage of the hemiconvulsion–hemiplegia syndrome¹ (Chapter 8), but this is extremely rare (0.06%)⁵^,⁶ and found only among children with a recognisable specific illness (usually infectious) of the CNS.¹⁵

Post-Ictal Stage

Post-ictal symptoms other than drowsiness are rare and should raise the suspicion of another diagnosis. Todd’s paralysis is exceptional (0.4%).⁵^,⁶

Risk Factors for Febrile Seizures

The risk factors for a first febrile seizure have been studied in comparison with age-matched febrile and non-febrile control children.³³^,³⁴

The risk of a first febrile seizure is approximately 30% if a child has two or more of the following independent risk factors:

a first- or second-degree relative with febrile seizures
delayed neonatal discharge of greater than 28 days of age
parental reports of slow development
day care attendance.

The risk for younger siblings of affected children is approximately 10–20% and this risk is greater if a parent is also affected. Males are more susceptible than females.

One-half of children will have recurrences: 32% have one, 15% have two and 7% have three or more recurrent seizures after a first febrile seizure. Half of those with a second febrile seizure will suffer at least one additional recurrence.

The following predisposing factors make recurrences more likely.³⁵

The first febrile seizure occurs in the first year of life.
The first febrile seizure occurs during a short and low-grade febrile illness.
There is a family history of febrile seizures in first-degree relatives.
There are persistent neurological abnormalities.
The first febrile seizure is complex.

Aetiology

Genetic predisposition is important, as febrile seizures are often familial.³⁶^,³⁷ Children with siblings or parents with febrile seizures have a four- to fivefold higher risk than the general population. The reported concordance rates range up to as high as 70% in monozygotic twins and 20% in dizygotic twins. The mode of inheritance is unknown though this is likely polygenic. Autosomal recessive inheritance is unlikely, as there is an excess of parents affected and the risk to siblings is less than 25%.⁶ Large autosomal dominant kindreds of febrile seizures made it possible to identify at least four different genetic loci mapped on chromosomes 8q13–21,³⁸^,³⁹ 19p13.3 and 19q13.1,⁴⁰^,⁴¹ 5q14–q15⁴² and 6q22–q24,⁴³ thereby assuring genetic heterogeneity.³⁷ No definitive gene or locus for febrile seizure has yet been established. However, genetic defects have been identified in a new genetically important syndrome of GEFS+ characterised by heterogeneous phenotypes of focal and generalised epileptic seizures³^,³⁷^,⁴⁴ as detailed on page 128. Evidence suggests the involvement of various sodium channels, GABA_A receptors and additional auxiliary proteins in the pathogenesis of febrile seizures plus and even in simple febrile seizures.³⁶

Pathophysiology

The pathophysiology of febrile seizures is unknown. They constitute a specific response to fever irrespective of cause. Circulating toxins and immune reaction products and viral or bacterial invasion of the CNS have been implicated, together with a relative lack of myelination in the immature brain and increased oxygen consumption during the febrile episode. Immaturity of thermoregulatory mechanisms and a limited capacity to increase cellular energy metabolism at elevated temperatures have been suggested as contributory factors.

The central histaminergic neuronal system may be involved in inhibition of the seizures associated with febrile illnesses in childhood. Children in whom the cerebrospinal fluid histamine does not rise during febrile illnesses may be susceptible to febrile seizures.⁴⁵

Viral diseases are commoner, but this may reflect their higher prevalence in children. A specific association between acute human herpesvirus 6 infection (roseola infantum) and febrile seizures has been postulated.⁴⁶ The causes of fever vary and include upper respiratory tract infection or pharyngitis (38%), otitis media (23%), pneumonia (15%), gastroenteritis (7%), roseola infantum (5%) and non-infectious illness (12%).

Important note
Seizures occurring soon after immunisation with diphtheria/pertussis/tetanus and measles vaccines are due to fever and not to an adverse effect of the vaccine.

Diagnostic Procedures

Febrile seizures do not need any investigation if the diagnosis is secure. EEG and brain imaging are unhelpful.

However, it is important to distinguish ‘febrile convulsions’ from ‘convulsions with fever’ such as in meningitis, encephalitis, cerebral palsy with inter-current infection and metabolic or neurodegenerative disease. Lumbar puncture may be mandatory in children who have a convulsion with fever in their first year of life (though this is still debated).

Electroencephalography

EEG in straightforward cases is not needed.¹⁰ EEG is more likely to be normal or show non-specific abnormalities that may be overemphasised by inexperienced neurophysiologists.

The reported incidence of EEG abnormalities in children with febrile seizures varies from 2 to 86%.⁴⁷ Such a wide difference is due in part to the definition of the EEG abnormalities, the time elapsed since the febrile seizure and the age of the patient at the time of the EEG.

Some authors have suggested that ‘hypnagogic paroxysmal spike wave activity’ is a special EEG feature seen in many children with febrile seizures.^48–50 This involves brief (1–2 s) generalised bursts of slow waves intermixed with small larval spikes as the child drifts to sleep. However, this is not specific and of no predictive significance.

Other authors have found that 20–50% of children who have serial EEGs starting at least 2 years after the initial febrile seizure demonstrate spikes or spike and wave abnormalities and photosensitivity.²⁰^,⁵¹ In addition, there appears to be a high incidence of Rolandic spikes in children with febrile seizures.⁵²^,⁵³

The American Academy of Paediatrics emphasises that, “No published study demonstrates that an EEG performed either at the time of presentation after a simple febrile seizure or within the following month will predict the occurrence of future non-febrile seizures. Although the incidence of abnormal EEGs increases over time after a simple febrile seizure, no evidence exists that abnormal EEGs after the first febrile seizure are predictive for either the risk of recurrence of febrile seizures or the development of epilepsy. Even studies that have included children with complex febrile seizures and/or those with pre-existing neurological disease (a group at higher risk of having epilepsy develop) have not shown EEGs to be predictive of the development of epilepsy.” ¹⁰

Prognosis

Clinical note
Overall, children with febrile seizures have a sixfold excess (3%) of subsequent non-febrile seizures and epilepsy compared with controls (Table 6.1).⁵^,²⁹^,⁵⁴ Simple febrile seizures without any of these risk factors (60%) have only a twofold excess (1%) of unprovoked seizures compared wth controls. The risk after complex febrile seizures increases from 6–8% when a single complex feature is present to 49% if all three elements (prolonged, repetitive and focal febrile seizures) are present. These non-febrile seizures occur a few months to two to three decades after the initial febrile attack, but 85% start within 4 years. The risk is 2% by age 5 years, 4.5% by age 10 years, 5.5% by age 15 years and 7% by age 25 years.⁵⁴

Table 6.1

Rates of epilepsy in children

Non-febrile seizures of the subsequent epilepsy are of any type.⁵⁴ Generalised seizures are much more common than focal seizures and tend to occur when there is a positive family history of non-febrile seizures and a large number of brief generalised febrile seizures. Focal epilepsies follow prolonged lateralised febrile seizures in approximately 20%. In addition, earlier onset and persisting neurological abnormalities favour the development of focal epilepsy.⁵⁴

The estimated risk of developing temporal lobe epilepsy (TLE) subsequent to prolonged febrile seizures is negligible, probably 1 in 75 000 children per year.⁵⁵^,⁵⁶ Conversely, one-third of patients with hippocampal epilepsy have a previous history of prolonged febrile seizures (see mesial TLE with hippocampal sclerosis on page 378).^57–61

The Risk Factors for Later Epilepsy Are:

(1) abnormal neurological or developmental status prior to the first febrile seizure,

(2) a family history of non-febrile seizures,

(3) complex febrile seizures.

The overall risk is 3%.⁵^,²⁹^,⁵⁴

Predisposition to Both Febrile Seizures and Other Types of Epilepsies

It is well known that febrile seizures precede the onset of various forms of epilepsies in 10–15% of children.⁵⁶^,⁶³ There is a predisposition to both febrile seizures and other types of idiopathic generalised epilepsies (IGEs),⁵⁴^,⁵⁶^,⁶³^,⁶⁴ benign childhood focal seizures⁶⁵ and other epilepsies such as Dravet syndrome. There is unanimous agreement that children with benign childhood focal seizures, mainly Rolandic epilepsy and Panayiotopoulos syndrome, have a high prevalence of preceding febrile seizures (10–30%).⁶⁵ One-fifth of children with benign myoclonic epilepsy of infancy suffer preceding attacks of febrile seizures.

Contrary to the above are the results of another study of children with epilepsies, 13.9% of whom also had febrile seizures.⁵⁶ Children with febrile seizures were less likely to have childhood absence epilepsy and absence seizures compared with children without febrile seizures. This was particularly true for simple febrile seizures. Complex but not simple febrile seizures were associated with a younger age at onset of epilepsy. There was no evidence that focal or prolonged febrile seizures were associated with focal epilepsies or TLE per se. Of three children whose initial MRI scans demonstrated hippocampal atrophy, none had a history of febrile seizures.⁵⁶

Intellectual and Behavioural Outcome

Children with febrile seizures perform as well as other children in terms of their academic progress, intellect and behaviour at 10 years of age.⁶⁶

The subsequent psychomotor development of children who were normal prior to the onset of febrile seizures is normal.⁶⁶^,⁶⁷ If psychomotor deficits, learning difficulties and behavioural problems are found in children with febrile seizures, they are not related to the febrile seizures, but probably reflect the overall developmental status of the child.²⁰

Management^12,^13,¹⁵

Clinical note
This is mainly based on the recent recommendations of the American Academy of Pediatrics.¹²^,¹³

Acute Management of a Child with a Febrile Seizure

Control of the seizures is paramount. Long-lasting febrile seizures (longer than 10 min) or status epilepticus (longer than 30 min) is a genuine paediatric emergency that demands appropriate and vigorous treatment, similar to non-febrile status epilepticus.¹⁵^,²⁵ Early, usually parental treatment is more effective than late emergency treatment.¹⁵ The parents of children with recurrent seizures should be advised to place the child on his/her side or stomach on a protected surface and administer a preparation of rectal benzodiazepine. In an emergency facility, the child’s airway should be kept clear, oxygenation maintained and intravenous or rectal diazepines given to halt the seizure. Diazepam intravenously at a dose of 0.2–0.3 mg/kg or in rectal preparations at a dose of 0.5 mg/kg is probably the first choice. Rectal absorption of liquid diazepam is very rapid, reaches the brain within minutes and has a near-intravenous efficacy. A disadvantage of diazepam is its short duration of action. Rectal tubes containing liquid diazepam are the most widely used formulation. Diazepam rectal gel is now available in the USA. Lorazepam administered intravenously (0.1 mg/kg), which is less likely to cause respiratory depression and probably has a longer action than diazepam, is often preferred in medical facilities. Midazolam administered by buccal or nasal application may be another alternative for terminating prolonged seizures in the home.^68–70
Treatment of the fever and, mainly, the underlying illness is also important. Sponging with tepid water, anti-pyretics or both are usually recommended for reducing high fever. Anti-pyretics have a more sustained action while tepid sponging has a quicker but shorter effect. Anti-pyretic treatment during febrile illnesses does not reduce the recurrence rate and cannot be recommended other than to make the child more comfortable and avoid dehydration. Paracetamol is more widely used than ibuprofen, while aspirin is avoided because it has been associated with the development of Reye’s syndrome.

Important note
Physical methods such as fanning, cold bathing and sponging are likely to cause discomfort and are not recommended.⁸

Prophylactic Management

Simple febrile seizures do not need prophylactic treatment. The risks are small and the potential side effects of drugs appear to outweigh the benefits.

Prophylactic treatment may be desired if a child has one or, mainly, a combination of the following features: (1) complex febrile seizures, (2) neurological abnormalities, (3) age less than 1 year and (4) frequent recurrences. Prophylactic treatment may be continuous or intermittent at the time of a febrile illness. Neither of these may be needed for the majority of children with febrile seizures, who nearly invariably do well.

Continuous treatment consists of daily administration of, mainly, phenobarbitone (which at a blood level of 15 μg/ml can effectively reduce the risk of recurrences) and, less often, valproate (fatal hepatitis in this age group or pancreatitis make valproate totally unacceptable). Carbamazepine and phenytoin are ineffective in the prevention of febrile seizures.

Intermittent treatment at the time of a febrile illness, mainly with rectal or oral diazepam, is an alternative to continuous medication (again a debated issue). There is a small reduction in the recurrence risk with a dose of 0.3 mg/kg of diazepam, although one-third of children will have significant side effects of somnolence or ataxia.

Supportive Family Management

The parents of young children should have general information provided by the family doctor about fever and febrile seizures. Parents who have watched their child during a fit need specific information in order to avoid long-term reactions.

In a study of parental reactions to a child’s first febrile convulsion⁷¹ most of the parents knew little about febrile seizures before the fit and most of them thought the child was dying (77%), suffocating or had meningitis (15%). Afterwards, the parental behaviour altered with restless sleep (60%), dyspepsia (29%) and overnight watching of the child (6%) or when feverish (8%). Parents with previous knowledge of febrile seizures took more appropriate measures but only 21% positioned the child correctly during the seizure.

Supportive family management includes education about febrile seizures, specific instructions about anti-pyretic and anti-epileptic prophylaxis and emergency procedures for possible subsequent seizures.

Benign Infantile Seizures (Familial and Non-Familial)
(Watanabe–vigevano Syndrome)

Clinical note
Benign infantile (familial and non-familial) seizures^87–98 constitute a benign age-related idiopathic syndrome of infancy. The seizures are focal and the infants are otherwise normal.

Demographic Data

Age at onset is 3–20 months with a peak at 5–6 months. The familial form mostly starts between 4 and 7 months. Boys and girls are equally affected in the non-familial form, but more girls are reported in the familial cases. Only small numbers, approximately 100 of all types, have been reported so far but this may increase with improved awareness of the condition.

Clinical Manifestations

Seizures characteristically occur in clusters of five to ten per day for 1–3 days and may recur after 1–3 months. One-third of patients have single isolated seizures 10–15 days before the clusters occur. The seizures are brief (0.5–3 min) but may be longer (3–6 min) at the beginning of the clusters and in the familial cases. They are predominantly diurnal. Inter-ictally the children remain normal.

The seizures are focal, manifesting with motor arrest, decreased responsiveness, impairment of consciousness, staring, eye and head deviation and mild unilateral clonic convulsions. Simple automatisms are frequent. Alternating from one side to the other is common. The seizures may or may not progress to hemi- or generalised convulsions.

Aetiology

The familial form is most likely autosomal dominant with genetic heterogeneity. Linkage has been found to chromosomes 19q12-13.1,¹⁰⁰ 2q24¹⁰¹ and 16p12-q12.¹⁰²

Considerations on Classification

The new ILAE diagnostic scheme recognises two types of benign infantile seizures (familial and non-familial). ²

Three types of benign infantile seizures have been described.

Benign infantile familial convulsions.⁸⁹
Benign infantile epilepsy with complex focal seizures.⁸⁷^,⁸⁸
Benign focal epilepsy with secondarily generalised seizures in infancy.⁹⁹

These have more in common than dividing features, as the main protagonists of their description, Watanabe and Vigevano, stated in a joint review.⁹⁰ Thus, all these are idiopathic seizures affecting otherwise normal infants, they:

are focal with or without secondarily generalised convulsions
appear in clusters in around the fifth to sixth months of life
have an entirely benign course.

In the dividing line is the familial or sporadic occurrence. Age at onset may be after the first year of life in the sporadic forms, while it is strictly limited to this age group (3 months-12 months) in the familial forms.

Benign familial infantile seizures do not appear to have genetic links with benign neonatal seizures though they may also prove to be channelopathies. In this respect the discovery of familial forms with seizure onset in the intermediate age (1–3 months) between benign neonatal and infantile seizures, for which the term ‘benign familial neonatal–infantile seizures’ has been coined (page 126), may be relevant.¹⁰³^,¹⁰⁴ They are caused by mutations in the sodium channel subunit gene SCN2A.¹⁰³^,¹⁰⁴ No such mutations were found in ten families with benign familial infantile seizures.¹⁰⁴

In addition, of significant genetic interest is the description of ‘familial infantile convulsions and choreoathetosis’ linked to chromosome 16.¹⁰⁵^,¹⁰⁶ In this syndrome benign infantile seizures are inherited as an autosomal dominant trait together with variably expressed paroxysmal choreoathetosis.¹⁰⁵^,¹⁰⁶

In another familial syndrome there is partial co-segregation of benign familial infantile seizures and familial hemiplegic migraine (which is linked to chromosome 19p13 in 50% of the families tested).¹⁰⁷

The sporadic cases of benign infantile seizures may be identical to the familial cases, but with reduced expressivity¹⁰⁸ or they may be due to exogenous factors such as rotavirus infections.¹⁰⁹

Diagnostic Procedures

All relevant tests performed for infantile seizures are normal. However, they are needed, particularly in sporadic cases, in order to exclude symptomatic infantile seizures.

Electroencephalography

The inter-ictal EEG is normal. The ictal EEG demonstrates focal discharges of fast activity intermixed with spikes that usually spread to neighbouring areas or the whole brain (Figure 6.1). Onset may be frontal, temporal, parietal or occipital and may vary in location and side between seizures in the same patient.⁹⁸^,¹⁰⁹

Figure 6.1

Benign infantile seizures (non-familial). An ictal EEG of a seizure in an 8-week-old baby who, at this age, had three focal seizures of right-sided convulsions involving the face and upper limbs (This is case 17.2 in reference (more...)

Differential Diagnosis

This may be difficult in the sporadic form, which requires a long follow-up before such a diagnosis can be established.⁹⁴

Prognosis

By definition seizures remit within 1–2 years from onset. Development is normal. In untreated cases there can be isolated or brief clusters within this infantile period of life.

Management

In the active seizure period, drug treatment is usually effective. Complete seizure control is achieved in nearly all cases. Recurrences after 1–2 months may occur in one-third of patients, but these are also easily controlled by drug dose adjustments. Anti-epileptic treatment is usually withdrawn after 1–3 years with no relapses.

Watanabe mainly used carbamazepine while Vigevano used valproate or phenobarbitone.

Other Hereditary Syndromes with Benign Infantile Seizures

Not Yet Recognised by the International League against Epilepsy

Benign Familial Neonatal–infantile Seizures

This syndrome has been described in a small number of families by Berkovic’s team.¹⁰³^,¹⁰⁴ The term ‘benign familial neonatal–infantile seizures’ is used to denote the fact that the onset of seizures is in the intermediate age period of when benign neonatal and benign infantile seizures occur.

Benign familial neonatal–infantile seizures is an autosomal dominant disorder presenting between day 2 and 7 months (mean 11.2 ± 9.2 weeks) with non-febrile secondarily generalised focal seizures: neonatal seizures were not seen in all families. The frequency of seizures varied, with some individuals having only a few attacks without treatment and others having clusters of many per day. Febrile seizures were rare. All cases remitted by 12 months. Ictal recordings in four subjects showed onset from the posterior quadrants.

The disorder is a sodium channelopathy caused by mutations in the sodium channel subunit gene SCN2A.¹⁰³^,¹⁰⁴ No such mutations were found in ten families with benign familial infantile seizures.¹⁰⁴

Familial Infantile Convulsions and Choreoathetosis

Familial infantile convulsions and choreoathetosis has been described in four families from France in which benign infantile convulsions were inherited as an autosomal dominant trait together with variably expressed paroxysmal choreoathetosis.¹⁰⁵ The clinical manifestations of infantile seizures are the same as those already described for benign infantile seizures. Choreoathetotic movements start during infancy or childhood, are of dystonic type, occur at rest or can be induced by exertion or anxiety. Strong evidence of linkage for the disease gene was obtained in the peri-centromeric region of chromosome 16.¹⁰⁵

In another report from Japan, Hamada et al.¹⁰⁶ reported 11 patients from five unrelated families. Paroxysmal choreoathetosis started between the ages of 5 and 17 years and was controlled with anti-epileptic drugs (AEDs) or subsided spontaneously. Eight cases also had infantile convulsions with onset between 3 and 8 months and an excellent prognosis. Four had complex focal seizures, which were characterised by staring, eye deviation, apnoea or loss of consciousness. Paroxysmal choreoathetosis was no different between those with or without infantile convulsions.¹⁰⁶

Familial Hemiplegic Migraine and Benign Familial Infantile Seizures

Terwindt et al.¹⁰⁷ reported a large Dutch–Canadian family with familial hemiplegic migraine and benign familial infantile seizures concurring and partially co-segregating. Familial hemiplegic migraine is a dominantly inherited subtype of migraine with attacks of hemiparesis linked to chromosome 19p13 in half of the families tested. Mutations in a brain-specific P/Q-type calcium channel alpha 1 subunit gene (CACNL1A4) were identified in families with chromosome 19-linked familial hemiplegic migraine. Molecular analysis of this family excluded linkage of the infantile convulsions to markers on chromosome 20q13.2, 8q or 19p13.

Idiopathic Epileptic Syndromes

Generalised Epilepsy with Febrile Seizures plus
(Autosomal Dominant Epilepsy with Febrile Seizures plus)

Clinical note
GEFS+³^,⁴^,⁴⁴^,^72–78 is the most important familial epileptic syndrome because it links febrile seizures with various other epileptic seizures/syndromes and documents genetic relations between (1) benign and severe and (2) focal and generalised epileptic disorders.
GEFS+ has been described by Berkovic and his associates⁴⁴^,⁷³ and has been recognised as a syndrome in development by the ILAE Task Force.²

Demographic Data

The age at onset, from the first months of life to childhood, varies considerably between individuals, even individuals of the same family. Both sexes are equally affected. The prevalence is unknown, but may be high considering the increasing numbers of publications and the very broad spectrum of GEFS+.

Clinical Manifestations

GEFS+ is characterised by heterogeneous clinical phenotypes ranging from classical febrile seizures, ‘febrile seizures plus’ (FS+), non-febrile generalised convulsions, absences, myoclonic or atonic seizures and, more frequently myoclonic-atonic seizures. Focal frontal and temporal lobe seizures may occur⁸⁰ in 13% of affected individuals⁴ and these focal seizures may dominate in some members of affected families.³ Within the GEFS+ spectrum, more severe syndromic phenotypes can occur including Dravet syndrome, epilepsy with myoclonic-astatic seizures (EM-AS) of Doose and TLE.⁸¹^,⁸²

GEFS+ shows marked genetic and phenotypic heterogeneity. There are extreme intra-familial and inter-familial clinical variations regarding seizure type, seizure frequency, severity and prognosis. By definition, in all families some patients suffer from FS+, which are often preceded by classical febrile seizures.

Febrile seizures may occur alone (approximately 75% of patients) or combine with other type of seizures such as the following:

Brief non-febrile generalised convulsions
Other generalised seizures, such as absences, myoclonic jerks and tonic seizures
Focal seizures of mainly frontal or temporal lobe origin
More severe forms of seizures such as those occurring in Dravet syndrome and epilepsy with myoclonic-astatic seizures. Typical febrile seizures and FS+ are the most common clinical phenotypes occurring in approximately 75% of affected patients.

Definitions and Clarifications for Febrile Seizures plus

Febrile seizures plus’ (FS+) is a term used to denote childhood onset febrile seizures, which (unlike the typical febrile seizures) start earlier (less than 6 months with mean 1 year) than the classical febrile seizures. They are often multiple and continue beyond the age of 5 years usually remitting by mid-childhood (median 11 years). Individuals with FS+ may also have additional non-febrile seizures.

According to Berkovic, the critical feature in diagnosing the ‘febrile seizures plus’ phenotype is the continuity of generalised seizures from early to mid-childhood, not the presence or absence of fever. Indeed, families often do not recognise the absence of a fever, interpreting all attacks as ‘febrile seizures’. In some children with FS+, seizures with fever did occur beyond age 6 years, whereas in others, all seizures beyond age 6 years were non-febrile.⁷⁹

Some authors have proposed that the name ‘autosomal dominant epilepsy with febrile seizures plus’ may be more appropriate than GEFS+ because recent discoveries document that the spectrum of the syndrome includes diverse types of focal and generalised seizures.³

Of nine families identified by probands suffering from myoclonic-atonic seizures genealogical information was available for 799 individuals: 91 had a history of seizures and 63 had seizures consistent with the diagnosis of GEFS+ syndrome.⁷³ Of these 63 patients 49% had classical febrile seizures only, 29% had FS+, 13% had FS+ with other seizure types such as absences (5%), myoclonic seizures (3%) and temporal lobe seizures (3%) and 14% had myoclonic-atonic seizures.⁷³ Of other members (not considered to suffer from GEFS+) one had typical childhood absence epilepsy and another three had nocturnal GTCS after a head concussion.

In another family with GEFS+, all 15 patients had typical febrile seizures.⁷⁷ Of these 40% had FS+, 13% non-febrile tonic clonic convulsions, 13% atonic seizures, 6% nocturnal tonic seizures and 6% a single focal seizure with orofacial onset.⁷⁷

The high prevalence of GEFS+ amongst patients with Dravet syndrome has only recently been realised (see Chapter 7, page 155).⁸¹^,⁸³^,⁸⁴

In seven recently described families (unrelated to SCN1A, SCN1B and GABRG2 gene mutations) of 167 individuals, 41 had epilepsy: 29 had a phenotype consistent with GEFS+, seven had IGE, in three the epilepsy type could not be classified and two were considered phenocopies.⁴ The clinical phenotypes included FS+ (29.2%), febrile seizures (29.2%), IGE (18.2%), FS+ with focal seizures (13%) or absence seizures (2.6%) and febrile seizures with absence seizures (2.6%).

Aetiology

GEFS+ is a purely genetic disorder with profound heterogeneity. Inheritance is generally autosomal dominant with incomplete penetrance,⁷³ but this may not be the only situation.³⁷

As Berkovic indicated, “GEFS+ may follow a more complex inheritance and serve as an entrée to the genetics of common forms of epilepsy. It is likely that the severe phenotypes within GEFS+ are the result of cumulative effects of multiple genes. Thus, finding one gene within a family may form a basis from which other genes may be found, entering the domain of true complex inheritance.” ³⁷

GEFS+ is genetically heterogeneous with two loci described on chromosome 19q (GEFS+) and chromosome 2q (GEFS2). Mutations were found in the SCN1A, SCN1B and SCN2A genes (encoding the alpha 1, alpha 2 and beta 1 voltage-gated sodium channel subunits) and the GABRG2 gene (GABA_A–receptor gamma 2 subunit).⁸⁵ At least nine mutations that cause GEFS+ have been identified in the SCN1A gene encoding the alpha subunit of the Na(v)1.1 voltage-gated sodium channel.⁸⁶ More recent studies have indicated that mutations of the SCN1A, SCN2A, SCN1B and GABRG2 genes in patients with GEFS+ are rare.⁴^,⁷⁷

There may be many mechanisms by which sodium channel alterations cause the various clinical phenotypes of GEFS+. To produce the different seizure types observed in families with GEFS+, seizure predisposition determined by the GEFS+ genes could be modified by other genes and/or by environmental factors.⁸²

One of the most significant recent developments is the frequent association of GEFS+ with EM-AS of Doose (Chapter 10, page 291) and Dravet syndrome. (see Chapter 7, page 152).⁸¹^,⁸³^,⁸⁴ Dravet syndrome probably represents the very severe end of the spectrum within the GEFS+ phenotype.⁸²

Diagnostic Procedures

Brain MRI when performed is within the normal limits.

EEG findings depend on the clinical phenotype. The EEG is usually normal, particularly in patients with febrile seizures only. The commonest EEG abnormality is sparse and brief generalised discharges of spike and slow wave that might require sleep EEGs for their detection. In clinical phenotypes of EM-AS or Dravet syndrome the EEG abnormalities are severe as described in the relevant chapters. In patients with focal seizures, the EEG shows focal sharp waves, which are mainly localised in the frontal and temporal regions.³^,⁴

Prognosis

In most studies, the overall picture is that GEFS+ is usually benign and self-limited.⁷³^,⁷⁷ Non-febrile seizures occur in only approximately one-quarter of the patients and these are usually infrequent and often remit by mid-childhood (median 11 years). However, this overall good prognosis is now being reconsidered with the inclusion of Dravet syndrome, a devastating epileptic encephalopathy, within the broad spectrum of GEFS+.

Differential Diagnosis

In a child presenting with recurrent convulsions precipitated by fever, the diagnostic issue is whether they are typical febrile seizures or FS+. This is because, at onset, the FS+ phenotype resembles typical febrile seizures occurring between 6 months and 5 years. ¹² The distinguishing features are the persistence of febrile seizures beyond the age of 6 years and/or the occurrence of non-febrile seizures.

Absences when they feature are usually brief and mild. They are easily distinguishable from the severe forms of childhood or juvenile absence epilepsy.

The evolution to other more severe clinical phenotypes such as EM-AS or Dravet syndrome becomes apparent only with the emergence of new types of seizures compatible with these disorders.

Management

Repetitive febrile seizures or FS+ may need prophylactic treatment. Valproate or newer drugs such as lamotrigine, levetiractetam or topiramate should be used for non-febrile generalised seizures, if these are frequent. Treatment of more severe phenotypes has been described in the chapter on Dravet syndrome (page 152) and EM-AS (page 291).

Benign Myoclonic Epilepsy in Infancy

Benign myoclonic epilepsy in infancy⁹⁰^,^110–116 is probably the earliest form of an age-dependent IGE syndrome manifested mainly or exclusively by myoclonic jerks only. The jerks may be spontaneous, reflex or both.

Demographic Data

Onset is between 6 months and 3 years, but in a few infants may start earlier (4 months) or later (2–5 years). Boys are twice as likely to be affected than girls. The prevalence may be around 1–2% of epilepsies starting before the age of 3 years.¹¹⁵^,¹¹⁶ This syndrome is based on retrospective studies and single case reports of approximately 100 patients, sometimes heterogeneous and including those with stimulus-elicited myoclonic jerks.¹¹⁵^,¹¹⁶

Clinical Manifestations

Myoclonic seizures are the predominant and often the only type of fits in benign myoclonic epilepsy in infancy.¹¹⁶

Myoclonic Jerks

Myoclonic jerks mainly affect the head, eyeballs, upper extremities and the diaphragm (Figure 6.2). The jerks are brief and singular or clusters that vary in frequency and violence. They clinically manifest with head nodding and more rarely flexion or extension of the body. The upper limbs usually fling upwards and outwards, while the eyeballs may roll upwards. A brief yell, probably resulting from the contraction of the diaphragm, sometimes accompanies the jerks. Falls may occur in the rare cases in which the lower limbs are affected.

Figure 6.2

Benign myoclonic epilepsy in infancy. This is a typical case of benign myoclonic epilepsy in infancy with myoclonic jerks only and an excellent prognosis. At age 3 years, she developed frequent and violent myoclonic jerks (more...)

Consciousness is commonly intact but clusters of jerks may be associated with mild clouding.

Myoclonic seizures are usually spontaneous, occurring randomly in alert stages and exaggerated by drowsiness and slow sleep. In some patients they tend to cluster on awakening or during the first hours of sleep. Reflex myoclonic jerks are sometimes prominent. Patients may have spontaneous or reflex-only jerks or both.

The duration of the jerks is usually brief (1–2 s). Vigevano et al.⁹⁰ reported that some children have more significant generalised clonic seizures, exclusively during sleep or on awakening, that are prolonged up to 15–20 min and can cause cyanosis without loss of consciousness.⁹⁰

Other Types of Seizures

One-fifth of patients have simple, brief and infrequent febrile seizures usually preceding the onset of myoclonias.

One-fifth of patients may develop infrequent GTCS usually in their early teens.

Non-febrile convulsions of uncertain categorisation prior to the onset of myoclonic seizures or during the clinical course of the disease have been reported.¹¹²

In one report six out of 11 children also had non-epileptic myoclonus.¹¹⁷

Precipitating Factors

One-fifth of patients have clinical and EEG photosensitivity. In 10% the myoclonic jerks are predominantly or exclusively elicited by unexpected acoustic or tactile stimuli and these may have a better prognosis.¹¹¹^,¹¹⁴^,¹¹⁸^,¹¹⁹ Single jerks or clusters of two to eight symmetric limb jerks, mainly of the arms, are elicited by sudden noise or tactile stimuli either when awake or asleep. Startle is important. If expected the stimulus is ineffective.¹¹¹

Aetiology

Benign myoclonic epilepsy in infancy is probably the earliest form of IGE. There is no evidence that it is linked with juvenile myoclonic epilepsy or indeed any other type of IGE. A family history of epilepsy or febrile seizures is present in 30% of cases.

Familial benign myoclonic epilepsy in infancy with autosomal recessive inheritance and linkage to chromosome 16p13 has been reported, but in this family myoclonic seizures persisted into adulthood and all patients developed GTCS in their early teens.¹²⁰

Diagnostic Procedures

All tests other than the EEGs are normal.

Electroencephalography

The inter-ictal EEG is normal. Spontaneous inter-ictal generalised polyspike wave discharges without associated jerks are exceptional.

The ictal EEG during jerks shows generalised polyspike or spike and slow wave discharges with a duration of 1–3 s (Figure 6.2).

Drowsiness and the early stages of sleep exaggerate the EEG discharges that may occur with or without jerks. EEG generalised discharges of mainly multiple spikes with jerks are often stimulus evoked by photic stimulation or unexpected acoustic or tactile stimuli. These occur in awake or sleep stages.

Differential Diagnosis

Benign myoclonic epilepsy in infancy should first be differentiated from non-epileptic conditions such as hypnagogic jerks and benign non-epileptic myoclonus (page 110). Hypnagogic jerks do not occur in waking states and the EEG is normal. Benign non-epileptic myoclonus resembles epileptic spasms rather than myoclonic jerks of benign myoclonic epilepsy in infancy.

It should not be difficult to differentiate benign myoclonic epilepsy in infancy from epileptic encephalopathies such as West, Dravet or Lennox–Gastaut syndromes with multiform seizures, severe EEG abnormalities and often neurodevelopmental deficits.

Prognosis

Remission usually occurs between 6 months and 5 years from onset. Patients with jerks provoked by auditory or tactile stimuli have a better prognosis and the jerks are easily controlled with anti-epileptic medication or stimulus preventive measures. Conversely, EEG photosensitivity may persist many years after clinical remission.

In general, 10–20% of all patients with benign myoclonic epilepsy in infancy develop infrequent GTCS in their early teens when medication has been withdrawn.

Psychomotor development is often normal, but 10–20% of children may later develop usually mild cognitive, behavioural or motor deficits if untreated.

Management

The response to AED treatment is usually excellent. Patients with photosensitivity are more difficult to control and EEG photosensitivity may persist several years after the remission of seizures. Patients with acoustic- and somatosensory-evoked myoclonus may not need treatment or withdrawal may be initiated after 1 year.

Valproate is considered the drug of choice by all authors. With adequate doses 80% of patients may become seizure free. However, no other suitable AEDs have been tried in this condition. Clonazepam is more effective than valproate in controlling myoclonic jerks. Levetiracetam is the most potent new anti-myoclonic drug, which also significantly suppresses photosensitivity (Table 10.8 page 317).¹²¹ Therefore, these AEDs may be more effective as monotherapy and probably in smaller doses than valproate.

Gradual and slow withdrawal of drug treatment over 6 months to a year can be initiated 3–5 years from onset.

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Footnotes

*: These are newly recognised syndromes and, although not specified by the International League Against Epilepsy (ILAE) Task Force, benign infantile seizures (familial and non-familial) are classified by name (seizures) in the group of conditions with ‘epileptic seizures that do not require a diagnosis of epilepsy’ (Table 1.6).
**: ‘GEFS+’ or ‘autosomal dominant epilepsy with febrile seizures plus’³^,⁴ has been recognised by the ILAE Task Force as a new syndrome in development.²

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Panayiotopoulos CP. The Epilepsies: Seizures, Syndromes and Management. Oxfordshire (UK): Bladon Medical Publishing; 2005. Chapter 6, Idiopathic Epileptic Seizures and Syndromes in Infancy.
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Conditions with Epileptic Seizures That Do Not Require a Diagnosis of Epilepsy
Idiopathic Epileptic Syndromes
Febrile Seizures
Benign Infantile Seizures (Familial and Non-Familial) : (Watanabe–vigevano Syndrome)
Other Hereditary Syndromes with Benign Infantile Seizures
Generalised Epilepsy with Febrile Seizures plus : (Autosomal Dominant Epilepsy with Febrile Seizures plus)
Benign Myoclonic Epilepsy in Infancy
References

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The Epilepsies: Seizures, Syndromes and Management.

Chapter 6Idiopathic Epileptic Seizures and Syndromes in Infancy

Conditions with Epileptic Seizures That Do Not Require a Diagnosis of Epilepsy

Idiopathic Epileptic Syndromes

Febrile Seizures

Demographic Data

Clinical Manifestations

Types of Febrile Seizures

Simple Febrile Seizures

ILAE and Other Formal Definitions for Febrile Seizures

Complex Febrile Seizures

Duration of Febrile Seizures

Post-Ictal Stage

Risk Factors for Febrile Seizures

Aetiology

Pathophysiology

Diagnostic Procedures

Electroencephalography

Prognosis

Table 6.1

The Risk Factors for Later Epilepsy Are:

Predisposition to Both Febrile Seizures and Other Types of Epilepsies

Intellectual and Behavioural Outcome

Management12,13,15

Acute Management of a Child with a Febrile Seizure

Prophylactic Management

Supportive Family Management

Benign Infantile Seizures (Familial and Non-Familial) (Watanabe–vigevano Syndrome)

Demographic Data

Clinical Manifestations

Aetiology

Considerations on Classification

Diagnostic Procedures

Electroencephalography

Figure 6.1

Differential Diagnosis

Prognosis

Management

Other Hereditary Syndromes with Benign Infantile Seizures

Not Yet Recognised by the International League against Epilepsy

Benign Familial Neonatal–infantile Seizures

Familial Infantile Convulsions and Choreoathetosis

Familial Hemiplegic Migraine and Benign Familial Infantile Seizures

Idiopathic Epileptic Syndromes

Generalised Epilepsy with Febrile Seizures plus (Autosomal Dominant Epilepsy with Febrile Seizures plus)

Demographic Data

Clinical Manifestations

Definitions and Clarifications for Febrile Seizures plus

Aetiology

Diagnostic Procedures

Prognosis

Differential Diagnosis

Management

Benign Myoclonic Epilepsy in Infancy

Demographic Data

Clinical Manifestations

Myoclonic Jerks

Figure 6.2

Other Types of Seizures

Precipitating Factors

Aetiology

Diagnostic Procedures

Electroencephalography

Differential Diagnosis

Prognosis

Management

References

Footnotes

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Management^12,^13,¹⁵

Benign Infantile Seizures (Familial and Non-Familial)
(Watanabe–vigevano Syndrome)

Generalised Epilepsy with Febrile Seizures plus
(Autosomal Dominant Epilepsy with Febrile Seizures plus)