Benign childhood epilepsy with centrotemporal spikes or Rolandic seizures/epilepsy is the commonest manifestation of a childhood seizure susceptibility syndrome that is age related and genetically determined.
This chapter is based on an exhaustive review of the literature regarding all aspects of Rolandic seizures and their EEG manifestations. The history of the ‘discovery of benign Rolandic epilepsy’ has been vividly described by the main French protagonists Marc Beaussart and Pierre Loiseau.
Demographic Data
Onset of RS is between 1 and 14 years; in 75% of patients, onset is between 7 and 10 years, and there is a peak at 8–9 years. There is a 1.5 male predominance. Prevalence is around 15% in children with seizures aged 1–15 years. Incidence is 10–20/100,000 children aged 0–15 years.
Clinical Manifestations
The cardinal features of RS are infrequent, often single, focal seizures consisting of unilateral facial sensorimotor symptoms, oro-pharyngo-laryngeal (OPL) manifestations, speech arrest and hypersalivation.1
Hemifacial sensorimotor seizures occur in approximately one-third of patients. These are mainly motor seizures, which may be entirely localised in the lower lip manifesting with sudden, continuous or bursts of clonic contractions, usually lasting from a few seconds to 1 minute. Ipsilateral tonic deviation of the mouth is also common. More rarely, hemifacial convulsions may appear nearly simultaneously or spread to the ipsilateral upper extremity. Involvement of the leg is rare.
Hemifacial sensory seizures are less common and consist of numbness in the corner of the mouth.
Consciousness is usually preserved.
Hemifacial sensorimotor symptoms may be the only ictal manifestations, but are often associated with an inability to speak and hypersalivation.
The left side of my mouth felt numb and started jerking and pulling to the left, and I could not speak to say what was happening to me.
Oro-pharyngo-laryngeal ictal manifestations, which occur in more than half of seizures (53%), are the most characteristic of all other ictal symptoms of RS. They consist of unilateral sensory and motor manifestations inside the mouth, tongue, inner cheek, gums, teeth and pharyngo-laryngeal regions. Sensory symptoms manifest with unilateral numbness and more commonly paraesthesias (tingling, prickling, freezing and their variations), and are usually diffuse on one side or, exceptionally, may be highly localised to even one tooth. Motor OPL symptoms produce strange sounds, such as death rattle, gargling, grunting, guttural sounds and their combinations.
In his sleep, he was making guttural noises, with his mouth pulled to the right, “as if he was chewing his tongue”.
We heard her making strange noises “like roaring” and found her unresponsive, head raised from the pillow, eyes wide open, rivers of saliva coming out of her mouth, rigid.
Arrest of speech is another common ictal symptom that occurs in more than 40% of RS. The child is inarticulate and attempts to communicate with gestures. A few mainly laryngeal sounds, not words, may be uttered, particularly at the beginning.
Benign childhood epilepsy with centrotemporal spikes (best known among paediatricians as Rolandic epilepsy) is defined by the ILAE as follows:
“Benign childhood epilepsy with centrotemporal spikes is a syndrome of brief, simple, partial, hemifacial motor seizures, frequently having associated somatosensory symptoms which have a tendency to evolve into generalised tonic clonic seizures. Both seizure types are often related to sleep. Onset occurs between the ages of 3 and 13 years (peak 9–10 years) and recovery occurs before the age of 15–16 years. Genetic predisposition is common, and there is a male predominance. The EEG has blunt high-voltage centrotemporal spikes, often followed by slow waves that are activated by sleep and tend to shift or spread from side to side.
The following should be considered in future revisions of the classification of BCECTS:
(a). Most of centrotemporal spikes are, in fact, Rolandic spikes; they are rarely located in the temporal electrodes
(b). The word ‘temporal’ is misleading because these children do not have symptoms from the temporal lobes
(c). BCECTS may occur without centrotemporal spikes and conversely centrotemporal spikes may occur in children without seizures or other clinical phenotypes of benign childhood seizure susceptibility syndrome
(d). Similar clinical features may appear in patients with spikes in other than centrotemporal locations
(e). Children with centrotemporal spikes may manifest with symptoms typical of PS.
Rolandic seizures/epilepsy may be the most appropriate nomenclature. Rolandic epilepsy is very well established and well identified by neurologists, neurophysiologists and paediatricians with this form of benign childhood focal seizures. They all understand it as a benign seizure syndrome of children with ictal symptoms, originating from a well-known anatomical region of the brain, the inferior part of the pre-and post-central gyrus.
Rolandic fissure (or central fissure) is a well-established anatomical name that can not change, though the central (or Rolandic) sulcus was probably first described by the French anatomist Vicq d’Azy and not by the Italian anatomist Luigi Rolando. Also, epileptic symptoms do not come from the Rolandic (central) fissure, but from the pre- and post-central gyrus.
In this book I use BCECTS, Rolandic seizures or Rolandic epilepsy synomymously though I would prefer the term Rolandic seizures.
There is no impairment of the cortical language mechanisms. The child is perfectly able to understand what is being said, but unable to utter a single intelligible word. Some authors call thisaphemia oraphonia. However,aphemia means motor aphasia or pure word mutism and does not appear to be correct, andaphonia is an inability to produce sounds by laryngeal mechanisms, which also does not appear to be the case in RS. The arrest of speech in RS is more of ananarthria, that is loss of the power and coordination for the articulation of words, which also explains why this is equally common in left or right sided RS.
My right hand was numb and stiff. My mouth opened and I could not speak. I wanted to say I can not speak. At the same time, it was as if somebody was strangling me.
She was trying to speak but only noises came out of her mouth as if her tongue was tied up in her mouth.
Some RS patients are dysarthric rather than anarthic that is they were able to pronounce some words but with difficulty as:
if there are stones in my mouth.
Hypersalivation is one of the most characteristic ictal symptoms of RS and probably occurs in as many as one-third of cases. It is often associated with OPL symptoms, but is also associated with pure hemifacial seizures and may be the most pronounced ictal manifestation. Hypersalivation is not just frothing:
Suddenly my mouth is full of saliva, it runs out like a river and I can not speak.
Ictal syncope may occur probably as a concurrent symptom of PS (page 237).
She lies there, unconscious with no movements, no convulsions, like a wax work, no life.
Consciousness is fully retained in more than half (58%) of RS and the patient is able to describe the events after the end of the fits well.
I felt that air was forced into my mouth, I could not speak and I could not close my mouth. I could understand well everything said to me. Other times I feel that there is food in my mouth and there is also a lot of salivation. I can not speak.
Secondarily GTCS are reported in between one- and two-thirds of children with RS. Primarily GTCS are not part of the syndrome of RS.
Duration of RS is usually brief, lasting for 1–2 min, but may become longer if seizures progress to convulsions.
Circadian distribution. Three-quarters of seizures occur during non-REM sleep, mainly at sleep onset or just before awakening. Seizures during sleep are usually longer and may progress to GTCS, which rarely occurs during wakefulness.
Status Epilepticus
Generalised convulsive status epilepticus is exceptional. Though rare, focal motor status epilepticusand hemiconvulsive status epilepticus are more likely to occur.
While skating, he felt that the left side of his tongue was numb and that he could not see well. This was followed within seconds by repetitive and continuous left-sided clonic hemifacial spasms, involving the mouth and eye that ended 40 min later with left hemiconvulsions. There was postictal Todd’s paralysis.
Hemiconvulsive status epilepticus may be more common in children aged 2–5 years; this is often associated with postictal Todd’s paralysis, which generally does not include the face.
Opercular status epilepticus usually occurs in atypical evolutions of BCECTS.or exceptionally it may be carbamazepine-induced. These are often associated with EEG continuous spikes and waves during slow-wave sleep (page 183). The status may last for hours to months and consists of continuous unilateral or bilateral contractions of the mouth, tongue or eyelids, positive or negative subtle perioral or other myoclonia, dysarthria, anarthria or speech arrest, difficulties in swallowing, buccofacial apraxia and hypersalivation.
Aetiology
Benign childhood epilepsy with centrotemporal spikes is genetically determined and there is evidence of linkage with chromosome 15q1424. The mode of inheritance is unknown. Autosomal dominant inheritance with age-dependent penetrance refers to the EEG centrotemporal spikes, and not to the clinical syndrome of BCECTS (see review in ref1).A multifactorial pathogenesis with hereditary impairment of brain maturation has been proposed by Doose and associates.However, according to a recent study conventional genetic influences may be less important than other mechanisms, which need to be explored. This study compared the concordance of twins with Rolandic epilepsy with the concordance of a twin sample of IGEs. All eight twins (six monozygous and two dizygous) with RS were discordant Monozygous pairwise concordance was 0 (95% confidence interval, 0–0.4) for Rolandic epilepsy compared with 0.7 (95% confidence interval, 0.5–0.9) for 26 IGE monozygous pairs.
Siblings or parents of patients with BCECTS may rarely have the same type of seizures or other phenotypes of BCSSS, such as PS (page 240). Febrile seizures are common (10–20%) prior to RS.
My view that RS are part of a BCSSS is detailed on page 262. All these benign childhood conditions are linked together through a common, genetically determined, mild and reversible, functional derangement of the brain cortical maturational process.
Pathophysiology
The ictal manifestations of RS agree well with he symptoms elicited by electrical stimulation of the lower part of the precentral and postcentral gyrus in ma
Hypersalivation, like other autonomic manifestations in childhood focal seizures, is difficult to explain; it is extremely rare in adults.
Diagnostic Procedures
Apart from the EEG, all tests are normal.
Brain imaging is not needed when the diagnosis of RS is certain though 15% of patients with RS may have abnormal findings because of static or other brain diseases unrelated to the pathophysiology of RS. Further, hippocampal abnormalities have been detected in some children with RS on MRI and proton magnetic resonance spectroscopywhich may be incompatible with such an age-related and benign seizure disorder.
The presence of brain lesions has no influence on the prognosis of RS
Interictal EEG
Top: High amplitude centrotemporal spikes (in fact these are central spikes) occur independently on the right or left, and are markedly exaggerated during natural sleep.
Top extreme right: Typical morphology and polarity of CTS in Laplacian montage.
Bottom: ESES, which are evoked by tapping fingers or toes. Note that their location corresponds to the location of the activating stimulus.
Centrotemporal spikes are mainly Rolandic not temporal spikes. Facing page
Top, middle and bottom: The same EEG sample is shown in 3 different montages.
This is from an 8-year-old boy referred for an EEG because of “recent GTCS and a 2-year history of unilateral facial spasms. Previously, the EEG and CT brain scan were normal. No medication. Focal seizures with secondarily generalised convulsions?”
The EEG showed frequent clusters of repetitive centrotemporal spikes on the left. Because the spikes appeared to be of higher amplitude in the temporal electrode (T3) (black arrows), the technologist rightly applied additional electrodes at C5 and C6 (Rolandic localisation). This showed that the spike is of higher amplitude in the left Rolandic region (C5) (open arrows).
Activation of functional centrotemporal and occipital spikes. Facing page
Top: Video EEG of a 6-year-old girl with headaches and abdominal pains of recent onset Neurological examination and MRI were normal. Symptoms improved over the following year. She never had seizures and her development was normal. EEG showed normal background with the following abnormalities:
(a). Spontaneously central spikes, occurring independently on the right or left.
(b). High amplitude central spikes elicited by somatosensory stimulation of the contralateral side. Simultaneous stimulation of the fingers of the hands by the patient herself elicited simultaneous bilateral central spikes.
(c). Brief, mainly anterior, bursts of polyspikes.
(d). Brief and high amplitude generalised discharges of 3–5 Hz slow waves interspersed with small spikes or small polyspikes.
Note that the ESES are bilateral and synchronous when the stimulus is also bilateral and synchronous (tapping together and simultaneously the palmar tips of her fingers). Unilateral tapping evoked contralateral ESES.
Middle: ESES of a patient with PS (case 17 in ref). At the age of 8 years, this boy had a single nocturnal seizure, which started with repetitive vomiting and “he was lost”. He then clenched his teeth and became rigid, but there were no clonic convulsions. The last follow-up, at the age of 14 years, disclosed no further seizures and normal development, though school performance was moderate. Three EEGs showed right CTS that were also evoked by somatosensory stimuli. Occipital spikes were never observed.
Top: High amplitude right-sided centrotemporal spikes (C5 and C6 electrodes were not applied).
Bottom: Onset of ictal discharge in the right centrotemporal regions during sleep. Arrow shows onset of clinical manifestations that started with contractions of the left facial muscles (note muscle artefacts on the left),
Centrotemporal spikes are the hallmark of the syndrome of BCECTS They are characterised by their morphology, amplitude and duration, location and field distribution, frequency and pattern of occurrence, reactivity to external stimuli and the sleep-wake cycle, as well as age-dependence and evolution.
Although called centrotemporal spikes, these are mainly high amplitude sharp and slow wave complexes localised in the C3/C4 (central) or C5/C6 (midway between central and temporal) electrodes The main spike (sharp wave) component is diphasic with a maximum surface, negative, rounded peak that is followed by a smaller positive peak ). This is followed by a negative or negative-positive slow wave. A relatively minute positive spike often precedes this spike–slow wave complex ). The amplitude of the main spike (or sharp wave) component often exceeds 200 μV, though it may be much smaller or much higher. The negative phase is larger than the positive phase of the spike, as well as the preceding or following components of the spike–slow wave complex. CTS may be unilateral, but are more often bilateral, independently right or left. They are abundant (4–20/min) and usually occur in clusters.
CTS increase during stages I–IV of sleep by a factor of 2–5 times without disturbing the sleep organisation
Rarely, children with RS may have a normal EEG, the spikes may be very small or CTS appear only during sleep stages (3–35%).1 In serial EEG, CTS may appear right or left, infrequent or abundant, small or giant, alone or with functional spikes in other locations.
In this book, I comply with the nomenclature “centrotemporal spikes”, though they are rarely temporal; “Rolandic spikes” or simply “central spikes” would be a more accurate name
Dipoles of Centrotemporal Spikes
The main negative spike component of CTS can usually be modelled by a single and stable tangential dipole source along the Rolandic region, with the negative pole maximum in the centrotemporal region and the positive pole maximum in the frontal regions (Figure 9.3 The tangential dipole and the location of CTS have been confirmed with magnetoencephalography.
Concurrent Spikes in Locations Other than the Centrotemporal Region
CTS may occur simultaneously in the same EEG with morphologically similar sharp and slow waves in other locations, such as the midline, parietal, frontal and occipital regions. These multifocal sharp waves are more frequently seen in serial EEGs. Occipital spikes are usually the first to appear.
The frequency, location and persistence of CTS do not determine the clinical manifestations, severity and frequency of seizures or the prognosis.
Centrotemporal Spikes in Normal and Children without Rolandic Seizures
CTS occur in 2–3% of normal school-age children, of whom less than 10% develop RS. CTS are age-dependent, appearing at a peak age of 7–10 years, often persisting despite clinical remission, and usually disappearing before the age of 16 years. They are common among relatives of children with RS. Age-dependent CTS frequently occur in a variety of organic brain diseases with or without seizures, such as cerebral tumours, Rett syndrome, fragile X syndrome and focal cortical dysplasia. Furthermore, CTS may incidentally be found in non-epileptic children with various symptoms, such as headache speech, behavioural and learning difficulties.
Sharp wave: A transient, clearly distinguished from background activity, with pointed peak at conventional paper speeds and duration of 70–200 ms i.e. over 1/14–1/5 s approximately. The main component is generally negative relative to other areas. Amplitude is variable.
Spike: A transient, clearly distinguished from background activity, with pointed peak at conventional paper speeds and duration of 20 to under 70 ms i.e. over 1/50–1/14 s approximately. The main component is generally negative relative to other areas. Amplitude is variable.
According to the above definition what we call centrotemporal spikes are centrotemporal sharp waves because their duration is usually more than 70 ms.
Niedermeyer explained: “EEG spikes should be differentiated from sharp waves (i.e. transients having similar characteristics but longer duration). However, it is well to keep in mind that that this distinction is largely arbitrary and serves primarily descriptive purposes. It is certainly not incorrect to use the term ‘spike’ and ‘sharp wave’ synonymously when a local paroxysmal event is discussed, although purists of nomenclature would regard this as a breach of etiquette.
Extreme Somatosensory Evoked Potentials/Spikes
After sleep, the most common form of activation of CTS (10%–20%) is somatosensory stimulation mainly of the fingers or toes These are called extreme somatosensory evoked spikes (ESES), extreme somatosensory evoked potentials or giant somatosensory evoked spikes. Like normal somatosensory evoked potentials, their location depends on the site and side of stimulation but their size and morphology is identical to that of CTS. ESES correspond to mid- or long-latency somatosensory evoked potentials with peaks at 35–80 ms depending on the height of the individual and the site of the stimulation ESES persist during sleep. ESES, like spontaneous CTS, occur in children with or without seizures and disappear with age. They may be detected in EEGs with or without spontaneous CTS or other functional spikes of childhood.
Techniques to Elicit Extreme Somatosensory Evoked Spikes
Any type of mechanical or electrical stimulus can elicit ESES in susceptible children providing that it is properly applied. It must be abrupt and strong enough (without being uncomfortable), and delivered to the appropriate sensitive body region. Percussion of the distal parts of the legs (toes and heels) or arms (palms and mainly tips of fingers) with a reflex hammer or with the plantar tips of the examiner’s fingers is very effective in eliciting ESES A hammer can also be connected to a channel of the EEG in order to mark the exact timing of the stimulus and measure the latency of the evoked spike. Electrical stimulation with digital electrodes as in orthodromic sensory nerve testing, or electrical stimulation of the nerve as in antidromic sensory nerve testing, is equally effective for eliciting ESES, but this is used only for research purposes It may not be necessary for routine EEG, because it is no more efficient than the mechanical stimulation described above, which is a more child-friendly method.
In clinical EEG practice, asking the child to tap together the palmar surface of the tips of his/her fingers of both hands is an easy method of testing for ESES ). The child should be instructed to strike them with sufficient strength and at random intervals of varying frequency.
EEG variability in Panayiotopoulos syndrome. EEG variability in Panayiotopoulos syndrome.
Samples from EEGs of six children with typical clinical manifestations of PS. Spikes may occur in all electrode locations, and they are usually of high amplitude and frequent or repetitive (cloned-like repetitive multifocal spike wave complexes). but may also be small and sparse. Brief generalised discharges of small spikes and slow waves may be present.
The reported prevalence of generalised discharges in RS varies from as low as 0% to as high as 54%.In my studies, generalised discharges occurred in about 4% of patients with RS and consisted of brief 1–3 s generalised bursts of 3–5 Hz slow waves intermixed with small spikes.These brief generalised discharges are identical to those seen in PSThe combination of a normal child with infrequent seizures and an EEG showing disproportionately severe focal epileptogenic activity is highly suggestive of benign childhood seizure susceptibility syndrome
Ictal EEG
There are very few reports of ictal EEG of RS. One example captured with video EEG is shown in.1 There is an initial paucity of spontaneous CTS prior to the onset of the ictal discharge, which appears in the ipsilateral Rolandic regions and consists of slow waves intermixed with fast rhythms and spikes.
Evolution and Prognosis
Remission occurs within 2–4 years of onset and before the age 16 years. The total number of seizures is low. The majority of patients have less than 10 seizures; 10–20% have a single seizure only. Around 10–20% may have frequent seizures, but these also remit with age.
Children with RS may develop reversible linguistic abnormalities during the active phase of their disease.Hospital-based studies emphasise learning or behavioural problems that require intervention. A few patients (< 1%) may progress to atypical evolutions of more severe syndromes of linguistic, behavioural and neuropsychological deficits, such as Landau-Kleffner syndrome, atypical focal epilepsy of childhood, or epilepsy with continuous spikes and waves during slow wave sleep.
The prognosis of RS is invariably excellent, with a less than 2% risk of developing infrequent generalised seizures in adult life; absence seizures may be more common than GTCS.
Development, social adaptation and occupation of adults with a previous history of RS is normal.
The only problem was with five patients who had difficulties in obtaining their driving licences and one patient who despite a 15-year seizure-free period was still on phenytoin because of concerns of her physician regarding her driving licence.
For unknown reasons social levels of patients with Rolandic epilepsy seem to be even higher than for non-epileptic controls.
Management
Children with RS may not need antiepileptic medication, particularly if the seizures are infrequent, mild or nocturnal, or the onset is close to the age of natural remission of this age-limited disorder. Patients with frequent seizures and secondarily GTCS or with comorbid conditions (tics, attention-deficit hyperactivity disorder, learning disability) may need medication. In a recent study, AEDs significantly reduced GTCS, but did not reduce focal seizures. On an empirical basis, carbamazepine is the preferred AED. However, some children might experience particular learning difficulties and exaggeration and new types of seizures while receiving carbamazepine.
Within days after re-introduction of carbamazepine, she suffered nearly continuous, brief atonic attacks of head and arm drop and also absences (case 17.3 in ref
Lamotrigine may be contraindicated in RS, because of case reports with exacerbation of the condition and new types of seizures.
See details in “Management of benign childhood focal seizures” (page 257).
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