Specific Epileptic Syndromes

In contrast to seizure location and the resultant clinical manifestations, differences exist in the occurrence of epileptic syndromes in adult and pediatric epileptology. Most specific epileptic syndromes begin in childhood and may continue into adulthood. We shall start with the benign epileptic syndromes and then the malignant syndromes. Descriptions of the epileptic syndromes come from the ICES. Additional points within each syndrome are specifically referenced. The following specific epileptic syndromes are those associated with specific EEG findings.

3.1. Benign Epileptic Syndromes

3.1.1. Febrile Seizures

The conventional wisdom regarding the EEG in benign febrile seizures is that the EEG should be normal, whereas, in a child with epilepsy, the EEG will be abnormal, with epileptiform features. The ICES considers febrile seizures a situation-related seizure: the situation being the illness with fever. However, there may not be such a clear distinction. Alvarez and Lombroso reported hypnagogic paroxysmal spike wave activity (minimal epileptiform features, sharp waves embedded into hyperventilation, or hypnagogic hypersynchrony) occurring with a higher incidence in children with febrile seizures. This is similar to epileptiform activity admixed into vertex waves and sleep spindles, called dyshormia by Niedermeyer (Fig. 1). Dyshormia refers to an abnormal paroxysmal arousal features during sleep. These features may indicate a lower seizure threshold and explains why they occur in both the normal population and in those with epilepsy.

The recent practice parameter (1999) on benign febrile seizures does not require an EEG for evaluating a benign febrile seizure. This is defined as a short (<15 min) GTCS without significant postictal depression, in a child with a nonfocal neurological examination without a family history of epilepsy.

3.1.2. Benign Myoclonic Epilepsy in Infancy

This disorder is characterized by brief bursts of generalized myoclonus that begin in otherwise normal children during the first 2 yr of life. There is often a family history of epilepsy. EEG shows brief bursts of generalized spike waves during early sleep. No other seizures occur at onset, but GTCS may occur during adolescence and developmental delay may be present.

3.1.3. Childhood Absence Epilepsy

The peak age of onset of childhood absence epilepsy is from 6 to 7 yr. The seizures consist of an abrupt cessation of ongoing activity, with a change of facial expression and a blank gaze. The duration is short, rarely lasting longer than 30 s, there is no preceding aura or subsequent postictal depression, and there may be frequent automatisms. Seizures may be activated by hyperventilation and photic stimulation.

Absence seizures are divided into typical absence and atypical absence seizures. Typical absence seizures are classified as simple or complex; a simple absence has a sudden onset without motor activity; complex absences have associated motor activity or autonomic activity. The atypical absence seizure has a less clearly defined onset or cessation, so that it may be difficult to identify the beginning or the end of the seizure without EEG. Atypical absence seizures may have more pronounced tone changes, a longer duration, and are typically associated with other seizure types and mental retardation.

Penry et al. studied 374 absence seizures with video EEG in 48 patients; a simple absence occurred in only 9% of patients; automatisms occurred in at least one episode in 88% of patients, clonic components occurred in 41% of patients, and seizures lasted less than 20 s in duration in 85% of patients.

Automatisms may be perseverative or de novo, and autonomic abnormalities may consist of pupillary dilatation, pallor, flushing, salivation, or incontinence.

The EEG in a typical absence seizure shows a symmetric 3-Hz generalized spike-and-wave discharge, which has a faster frequency at the beginning and a slower frequency at the end (Fig. 2). The atypical absence has a frequency between 1.5 and 2.5 Hz (usually slower), may be asymmetric, and usually has a slow background.

A pattern reported in children is hyperventilation-induced high-amplitude rhythmic slowing (HIHARS), in which altered awareness may occur with HIHARS on EEG. Clinically, eye opening and eyelid flutter occur more frequently in absence seizures, whereas fidgeting, smiling, and yawning occurred more frequently with HIHARS. Arrest of activity, staring, and oral and manual automatisms were observed in both groups. A similar pattern has been described in adults. These two disorders emphasize the importance of clinical observation by the EEG technologist during the study.

3.1.4. Intermittent Rhythmic Delta Activity

Intermittent rhythmic delta activity (IRDA) consists of rhythmic, sinusoidal, delta activity, which may be notched and even have low amplitude admixed spikes. IRDA may be generalized or have an age-dependent specific location: an occipital location (OIRDA) is more common in children (Fig. 3), whereas a frontal location is more common in older children and adults. Although IRDA itself is nonspecific for etiology, it typically occurs in disorders associated with acute altered awareness. In children, OIRDA is seen most commonly in epilepsy, especially generalized absence epilepsy. In our study of IRDA in children, 80% had epilepsy and the majority had absence epilepsy. This was referred to as posterior paroxysmal activity by Holmes et al. OIRDA occurs usually as an interictal pattern, but may be an ictal pattern; we have seen OIRDA evolve into a 3-Hz spike-and-wave discharge.

3.1.5. Juvenile Absence Epilepsy

The onset is around puberty, but the seizures may be less frequent than in the childhood absence syndrome. GTCS and myoclonic seizures may occur and the EEG shows a fast spike-and-wave discharge, similar to that seen in juvenile myoclonic epilepsy (JME). There may also be electrographic discharges that are more prolonged, with less altered awareness.

3.1.6. Juvenile Myoclonic Epilepsy

JME of Janz is characterized by mild myoclonic, generalized tonic-clonic, sometimes clonic-tonic-clonic seizures, and absence seizures. The onset is typically around puberty. JME is also referred to as impulsive petit mal.

The myoclonic seizures are usually mild to moderate in intensity, may involve the entire extremity, rather than isolated muscles, and generally are bilateral. These occur after awakening and are aggravated by fatigue, sleep deprivation, or alcohol. The patient may drop objects or fall. Myoclonic status epilepticus has occurred. GTCS are frequent. Delgado-Escueta et al. reported that GTCS occurred in 41 of 43 patients, and Asconape and Penry reported GTCS in 83% of patients. Myoclonic jerks usually precede the GTCS and may evolve into a GTCS. GTCS usually occur shortly after awakening. Absence seizures occurred in 40% in the series of Delgado-Escueta et al. These usually occur in association with GTCS, and most commonly occur shortly after awakening.

The EEG in JME is characterized interictally by a fast spike-and-wave discharge, 3.5- to 6-Hz spike-and-wave and polyspike-and-wave complexes (Fig. 4). Ten- to 16-Hz rapid spikes with slow waves occur with myoclonic seizures, photosensitivity is common, and abnormalities may occur in sleep.

3.1.7. Epilepsy With GTCS on Awakening

GTCS occur exclusively or predominantly shortly after awakening. The onset is usually in the second decade. Absence or myoclonic seizures may occur. The EEG shows the fast

FP1-F7, oo

FP1-F7,

Eeg Vertex Waves Sleep Eeg Vertex Waves SleepEeg Vertex Waves SleepGtcs Seizure
Childhood Absence Epilepsy
Fig. 1. Dyshormia. Six-year-old girl with childhood absence epilepsy. Note the spike component embedded with a vertex wave (A) and within spindles (B) during sleep.
Hypnagogic Hypersynchrony Adults
Fig. 2. Absence epilepsy. Twelve-year-old girl with seizures denoted by staring and a family history of seizure. Note the 3-Hz spike-and-wave pattern with responsiveness after its cessation.
Hypnagogic Hypersynchrony Sleep Children
Fig. 3. OIRDA. Eight-year-old girl with childhood absence epilepsy. Note the high amplitude, notched, 3- to 3.5-Hz occipital activity that attenuates with eye opening.
4hz Spike And Wave
Fig. 4. Juvenile myoclonic epilepsy (JME). Seventeen-year-old girl with JME. Note the generalized fast spike-and-wave discharge at approx 4 Hz.

spike-and-wave discharge, similar to JME. Photosensitivity may also occur. This syndrome may represent JME without myoclonus.

3.1.8. Benign Focal Epilepsy of Childhood

This disorder is also called benign childhood partial seizures. There are different clinical syndromes, which depend on discharge location, centro-temporal (Rolandic, benign Rolandic epilepsy [BRE], or benign childhood epilepsy with centrotemporal spikes), occipital, or frontal. The centro-temporal location is the most common, followed by the occipital location. BRE is a common epileptic syndrome, occurring in 16 to 24% of childhood epilepsies. In Iceland, the incidence of BRE is 4.7 in 100,000 people. Although considered a benign syndrome, cognitive dysfunction may occur.

The centro-temporal location, or BRE, has an age of onset of 3 to 13 yr, with a peak at 9 to 10 yr, and recovery occurs before the ages of 15 to 16 yr. According to Lerman, the seizure frequency is low, 13% may have just one seizure, 66% have infrequent seizures, and 21% have frequent seizures. The typical seizure characteristics consist of oral-buccal-lingual paresthe-sias, speech arrest, preservation of consciousness, sialorrhea, and tonic or clonic facial movements. The seizures are nocturnal in 50% of patients, occur in both the waking and sleep states in 15% of patients, and the waking state in only 10 to 20% of patients. Status epilepticus is rare. The prognosis is excellent, seizures are usually well-controlled, and the seizure frequency, recurrence, and duration are similar in treated and untreated children. Lerman and Kivity reported EEG normalization by adulthood in all patients. Lombroso referred to these as "sylvian" seizures, given the location of the discharges near the Sylvian fissure.

The EEG in BRE has distinctive, high-amplitude, diphasic spikes or sharp waves with a prominent slow wave, in the midtemporal (T3, T4) and central (C3, C4) regions (Fig. 5). However, when additional scalp electrodes are placed, maximum negativity was in the central region, either in a "high" (C3, C4) or "low" (C5, C6) location. Marked sleep activation occurs, and a horizontal dipole is present. There is a common misconception that the presence of a horizontal dipole is pathognomonic of a benign focal epilepsy. Similar findings have been reported in different pathological processes, including tumors. Massa et al. identified the following EEG characteristics as predictive of a "complicated" course: intermittent slow wave focus, multiple, asynchronous spike wave foci, spike wave clusters, generalized 3-Hz spike wave discharges, discharges with positive or negative myoclonia, or abundance of interictal abnormalities during waking or sleep.

There are now two defined occipital epilepsy syndromes in children, also called childhood epilepsy with occipital spikes or childhood epilepsy with occipital paroxysms: the early onset form (Panayiotopoulos type) and the late-onset (Gastaut type). Panayiotopoulos estimates that the early onset form comprises approx 6% of childhood epilepsies. The typical age of onset is 2 to 12 yr, with a median onset of 5 yr. The typical seizure consists of autonomic and behavioral disturbances, with vomiting, eye deviation, and altered awareness. Headaches and vomiting may occur, with migraines in the differential. The late-onset type consists of visual seizures with elementary visual hallucinations, which may evolve to a feeling of ocular movements or pain, eye deviation, ictal blindness, or focal or secondarily generalized seizures. The typical age of onset is 3 to 16 yr, with a mean age of 8 yr. Migraine is in the differential diagnosis. Neuropsychological dysfunction is also seen in these two disorders. On EEG, the giant spikes typical for benign occipital epilepsy are similar morphologically to those seen in BRE (Fig. 6). These occur with closed eyes, or have "fixation-off' sensitivity; fixation-off refers to blocking central vision or closing the eyes.

Benign Rolandic Epilepsy Eeg

1 sec

Fig. 5. Benign Rolandic epilepsy. Nine-year-old boy with nocturnal seizures, consisting of focal facial contractions and drooling. His EEG shows prominent triphasic sharp waves in the centro-parietal region, occurring independently bilaterally, and temporal sharp waves. In addition, a horizontal dipole is seen, with frontal positivity and posterior negativity.

1 sec

Fig. 5. Benign Rolandic epilepsy. Nine-year-old boy with nocturnal seizures, consisting of focal facial contractions and drooling. His EEG shows prominent triphasic sharp waves in the centro-parietal region, occurring independently bilaterally, and temporal sharp waves. In addition, a horizontal dipole is seen, with frontal positivity and posterior negativity.

Horizontal Dipole And Eeg
Fig. 6. Benign occipital epilepsy. Four-year-old girl with seizures. Note the independent, bi-occipital sharp and slow wave complexes with eyes closed.

Table 2

Neonatal Seizures: Classification System of Volpe

Electrographic Seizure

Clinical Seizure Common Uncommon

Subtle +

Clonic

Focal +

Multifocal +

Tonic

Focal +

Generalized +

Myoclonic

Focal, multifocal +

Generalized +

With a frontal location, seizures are infrequent in the waking state, are characterized by staring, with automatisms or version, and may generalize during sleep. The EEG shows the typical, repetitive frontal sharp waves with the characteristic "benign" morphology.

3.2. Malignant Epileptic Syndromes

The malignant epilepsy syndromes include disorders most likely associated with a poor prognosis. The EEG findings typical in these disorders include background discontinuity, burst suppression (BS), hypsarrhythmia, electrodecremental events, beta bursts, and the generalized paroxysmal fast activity.

3.2.1. Neonatal Seizures

There is no specific EEG pattern present in neonatal seizures. Neonatal seizures are classified by Volpe essentially using the semiology of the clinical seizure (Table 2).

Mizrahi et al. found that certain seizure types have a close association with an electro-graphic seizure, such as focal clonic seizures, whereas other seizure types, such as subtle seizures, may not have as high an association with an electrographic seizure (referred to as having an electrographic signature). These are referred to as clinical seizures with a consistent electrocortical signature or clinical seizures without a consistent electrocortical signature. There are also electrographic seizures without clinical seizure activity.

The background activity is very important for prognosis. Rose and Lombroso reported a good outcome with a normal EEG background in 86% of patients. A poor prognosis was associated with an abnormal background, especially the BS or low-voltage invariant patterns, or electrocerebral inactivity. The burst-suppression pattern is the most extreme manifestation of background discontinuity and is measured by the interburst interval (IBI). However, neonatal EEG may have features suggestive of dysmaturity, such as an increased duration of the IBI. Hahn et al. studied the duration of the IBI in normal premature infants and found a correlation with the duration of the IBI with the gestational age. Neonatal seizures do not all fit under malignant epileptic syndromes, but these set the stage for the malignant neonatal epileptic syndromes.

3.2.2. Neonatal Myoclonic Encephalopathy

Neonatal myoclonic encephalopathy (NME) is also known as early myoclonic encephalopathy. The onset is typically within the neonatal period, but before 3 mo of age. The seizures are characterized by partial or fragmentary myoclonus, massive myoclonias, and tonic spasms developing later. EEG consists of complex bursts of spikes and sharp waves, with periods of discontinuity; and the burst-suppression pattern, which may later evolve into a hypsarrhythmia pattern. All patients with NME are severely retarded and there is a high mortality during the first year of life. The metabolic disorder nonketotic hyperglycinemia needs to be excluded in this group.

3.2.3. Early Infantile Epileptic Encephalopathy

Early infantile epileptic encephalopathy with suppression bursts is also known as Ohtahara's Syndrome. The onset is within the first few months of age, typically with tonic seizures (spasms), focal seizures, and a burst-suppression EEG. The prognosis is similar to that of NME, and these patients may evolve into infantile spasms (IS; West Syndrome).

3.2.4. Severe Myoclonic Seizures of Infancy

In Severe myoclonic seizures of infancy, there is typically a family history of epilepsy or febrile seizures, no antecedent neurological history, and seizures beginning in the first year of life with generalized or unilateral febrile clonic seizures, with myoclonic seizures typically occurring between 1 and 4 yr of age. Focal seizures may also occur. The febrile seizures tend to be long and recurrent. EEG shows generalized spike waves and polyspike waves, early photosensitivity, and focal abnormalities. Psychomotor retardation occurs, usually beginning in the second year of life, with ataxia and interictal myoclonus. Seizures are typically very resistant to treatment.

The EEG may be normal at the time of the initial febrile seizures, but when the myoclonus starts, generalized spike-and-wave or polyspike-and-wave discharges occur. It may be difficult early on to differentiate the benign from the malignant syndrome, although febrile seizures do not typically occur in the benign form, in which seizures respond well to valproic acid and psychomotor retardation is absent.

3.2.5. Infantile Spasms (West Syndrome)

IS is an epileptic syndrome characterized by the triad of myoclonic or tonic seizures, hyp-sarrhythmia, and mental retardation. This is also called West Syndrome. IS is considered an age-dependent malignant epileptic syndrome, with a peak onset between 4 and 7 mo and always before 12 mo of age.

IS is divided into idiopathic, symptomatic, and cryptogenic forms. Symptomatic refers to IS secondary to a known neurological insult; cryptogenic refers to a suspected, but not definitely identified neurological insult; idiopathic is used when no specific insult has been identified. The prognosis is worse if the spasms are either symptomatic or cryptogenic.

Clinical manifestations include brief head nods, with quick extension and flexion movements of the trunk, arms, and legs, occurring in clusters and during transitions from sleeping to awaking. These are also described as flexor, extensor, or mixed. The actual spasms may resolve, but other seizure types occur later. The prognosis depends on the etiology.

Evaluation includes neuroimaging to exclude anatomic lesions, but other disorders, especially metabolic and infectious, need to be excluded. Focal spasms may occur secondary to focal lesions, such as tumor, stroke, or focal cortical dysplasia. Treatment is with steroids or standard anticonvulsant medications. Adrenocorticotropic hormone has been considered the drug of choice by many, although benzodiazepines, especially nitrazepam, and valproic acid, and now vigabatrin have been used. Vigabatrin may be especially effective if spasms are caused by tuberous sclerosis, and recent studies show a similar efficacy between vigabatrin and ACTH, with a lower incidence of acute side effects from vigabatrin. However, vigabatrin has been associated with retinal dysfunction, and visual field defects have been reported. Vigabatrin has not yet been released in the United States because of the retinal dysfunction.

If a focal lesion is responsible, then resection may be curative. Even in the absence of a frank mass lesion, surgery may be performed on refractory cases if an epileptogenic focus has been identified. Most of these cases result from focal cortical dysplasias. Functional neu-roimaging, especially positron emission tomography scan, have been used to localize the epileptogenic region.

The classic interictal EEG finding is the hypsarrhythmia pattern, which consists of a highvoltage, disorganized background with multifocal spike and sharp waves. This pattern may first occur during non-rapid eye movement sleep and may disappear during rapid eye movement (active sleep) sleep or the waking state. The typical ictal pattern is the electrodecremental event, in which a generalized spike or sharp wave is followed by a flattening of EEG activity (Fig. 7).

Hrachovy et al. described the variations of the pattern: increased interhemispheric synchronization, asymmetrical hypsarrhythmia, hypsarrhythmia with a consistent focus of abnormal discharge, hypsarrhythmia with episodes of attenuation, and hypsarrhythmia comprising primarily high-voltage slow activity with little sharp-wave or spike activity. From our department, Kramer et al. studied the hypsarrhythmia variant patterns: electrodecremental discharges, absence of normal sleep activity, relative normalization, hemihypsarrhythmia, BS, occipital hypsarrhythmia, interhemispheric asymmetry, and interhemispheric synchronization, and devised a scoring system. The variant patterns occurred in 69% of 53 consecutive EEGs. A better prognosis was not related to the presence of any one variant pattern, but was associated with faster background activity (<75% delta), a lower total hypsarrhythmia score (<10), and with absence of electrodecremental discharges on the pre-ACTH EEG.

3.2.6. Lennox-Gastaut Syndrome

Lennox-Gastaut Syndrome (LGS) is a severe, mixed seizure disorder, with tonic, atonic, and myoclonic seizures, mental retardation, and a slow spike-and-wave pattern on the EEG. This is a typical example of what has been termed an "epileptic encephalopathy." The majority of children with LGS have frequent intractable seizures, starting between 1 to 8 yr.

Tonic seizures are a major component of LGS, and criteria that are more restrictive for LGS require tonic seizures for the diagnosis. Tonic seizures are typically sleep activated and repetitive, although long-term monitoring may be needed to discover these. Benzodiazepines may induce tonic status epilepticus in LGS.

The EEG hallmark of LGS is the slow spike-and-wave discharge (1.5-2.5 Hz) superimposed on a slow background (Fig. 8). Bursts of fast rhythms occur during sleep (Fig. 9). These discharges may be frequent, and may be very sleep activated.

LGS is typically resistant to therapy with standard AEDs. Alternative therapies, such as corticosteroid therapy, the ketogenic diet, or vagal nerve stimulation, may be helpful.

3.2.7. Atypical Partial Benign Epilepsy of Childhood

Aicardi and Chevrie described the atypical partial benign epilepsy of childhood syndrome, which has some features of BRE; nocturnal focal seizures and focal EEG abnormalities, with

Idren's Hospital - Boston CLINIC = Division Of Epilepsy And Clinical Neurophysiology

Idren's Hospital - Boston CLINIC = Division Of Epilepsy And Clinical Neurophysiology

Lennox Gastaut Syndrome Lgs Eeg
Fig. 7. Infantile spasms (IS). Seven-month-old child with IS. Note the high-amplitude, disorganized slow activity with multifocal spikes with an electrodecremental response. This sort of electrodecremental event may be associated with a clinical spasm.
Atypical Spike Wave
Fig. 8. Slow spike and wave of Lennox-Gastaut syndrome (LGS). Thirty-six-year-old man with LGS. Note the bilaterally synchronous 1.5- to 2-Hz spike-and-wave discharges.
Polyspike Slow Wave Posterior Blinking
Fig. 9. Slow spike and wave with fast activity in sleep. Eleven-year-om gm with LGS. Her EEG shows slow spike-and-wave discharges, interrupted by a generalized burst of beta activity. Some of these beta bursts may be associated with tonic seizures.

initial seizures similar to BRE, but then evolving into periodic atonic seizures, with brief absences and GTCS. Atonic episodes correlate with the slow wave discharges on EEG. The EEG can be very sleep activated, similar to that seen in electrical status epilepticus of sleep (ESES), and time periods with marked sleep activation correlate with atonic seizures and intellectual regression. This fluctuates with the EEG findings. This disorder has been referred to as "pseudo-LGS," because EEG findings are similar to those of LGS.

3.2.8. Multiple Independent Spike Syndrome

Multiple independent spikes are defined as three or more independent spike foci, with at least one originating in both the right and left hemispheres. It is related to hypsarrhythmia and the slow spike-and-wave pattern seen in LGS. Background slowing and sleep-activated discharges are common, and similar to IS, increased synchrony may occur during sleep.

3.2.9. Epilepsy With Myoclonic-Astatic Seizures

Myoclonic astatic epilepsy is also called Doose Syndrome. Onset is typically between 7 mo and 6 yr of age. The seizures consist primarily of generalized myoclonic, astatic, or myoclonic-astatic seizures, short absences, and mostly GTCS, typically without tonic seizures or drop attacks. However, tonic seizures may occur in cases with a poor outcome. There are generalized spike-and-wave patterns on EEG with the background activity characterized by a 4- to 7-Hz rhythm with a parietal accentuation (Fig. 10). There is a high incidence of seizures or EEG abnormalities in relatives. Before the onset of the seizures, which may start as febrile seizures, development is normal. Valproic acid and ethosuximide are the drugs of choice. The prognosis is variable, some having a spontaneous remission.

3.2.10. Eyelid Myoclonia With Absences (Jeavons Syndrome)

Eyelid myoclonia with absences consists of eyelid myoclonia and absences, which may occur independently. The eyelid myoclonia typically occurs immediately after eye closure and is associated with brief bilateral spike-and-wave activity (Fig. 11). Eyelid myoclonia looks like rapid blinking, with upward eye deviation, and is more frequent in bright light and does not occur in the dark. However, eyelid myoclonia occurs in other idiopathic generalized epilepsies, especially with photosensitive generalized epilepsy, and has been reported in family members without seizures. These were called paroxysmal eyelid movements by Camfield et al. The frequency may help to differentiate these: the eyelid flutter with an absence seizure has a frequency of 3 Hz, eyelid myoclonia has a frequency of 4 to 6 Hz, and paroxysmal eyelid movements have a frequency of 10 Hz.

3.2.11. Photosensitive Epilepsy (A Reflex Epilepsy)

Photosensitive epilepsy is a specific reflex epilepsy, which refers to the induction of a seizure associated with a discharge of spikes or spike and slow waves with photic stimulation (referred to in the EEG laboratory as intermittent photic stimulation). The term photoparox-ysmal is used if there is only an electrographic discharge, whereas photoconvulsive is used if there is an actual clinical event associated with the discharge. Photosensitive seizures are usually associated with idiopathic generalized epilepsy, but may also occur with focal or symptomatic epilepsies. Seizures may be self-induced, and this may be difficult to treat. Avoidance of precipitating stimuli may be important (e.g., polarized sunglasses).

3.2.12. LKS (Acquired Epileptic Aphasia)

LKS is a rare epileptic syndrome characterized by language regression and an abnormal EEG. Language regression usually begins in those older than 4 yr and may first manifest as

FACILITY ■= Children's Hospital - Boston CLINIC = Division Of Epilepsy And Clinical Neurophysiology

FACILITY ■= Children's Hospital - Boston CLINIC = Division Of Epilepsy And Clinical Neurophysiology

Myoclonic Seizures
Fig. 10. Myoclonic astatic epilepsy (Doose syndrome). Five-year-old boy with generalized tonic-clonic seizures and myoclonic-astatic seizures. Note the generalized spike-and-wave discharge, with a frontal predominance, with a background of 6 Hz.
Eyelid Myoclonia
Fig. 11. Eyelid myoclonia with absences (Jeavons syndrome). Twelve-year-old girl with eyelid myoclonia associated with absence seizures and a rare, generalized tonic-clonic seizure, responsive to valproic acid. Note the generalized polyspikes induced by eye closure.
Continuous Spike And Wave During Sleep
Fig. 12. Landau-Kleffner syndrome (LKS). Four-year-old girl with LKS. Language regression occurred at 3 yr of age. Note the generalized, high-amplitude, slow spike-and-wave discharge during sleep, representing electrical status epilepticus of sleep.

an apparent word deafness, called a "verbal auditory agnosia." Seizures and behavior disturbances, particularly hyperactivity, each occur in approximately two-thirds of children with LKS.

The EEG in LKS shows bilateral, multifocal spikes and spike-and-wave discharges, occurring usually in the posterior regions, especially the temporal region, with a marked activation during sleep (Fig. 12). However, discharges occur in many locations, and may even be generalized. Some authorities require the presence of ESES to make the diagnosis.

3.2.13. Epilepsy With CSWS

CSWS is an epileptic syndrome characterized by continuous spike and waves during slow wave sleep. This has also been called ESES. CSWS consists of a sleep-activated EEG pattern with paroxysmal features present during 85% of slow wave sleep, and is divided into symptomatic and cryptogenic groups, determined by whether normal neurological or psychomotor development was present before the onset of the CSWS. Seizures are common but may not be frequent. Regression occurs in a more global manner, rather than predominantly the language regression seen in LKS. The sleep activated EEG may also occur in LKS, and some require the presence of CSWS for making the diagnosis of LKS. Guilhoto and Morrell reported that a more focal EEG pattern is associated with language regression, whereas, if the EEG pattern is more generalized, the regression is more global, and not predominantly in language skills.

LKS and CSWS have similar treatments: anticonvulsants are used for seizures and corticosteroids are used for the language or neurobehavioral difficulties. Valproic acid, the benzodiazepines, and ethosuximide have been the most successful anticonvulsants, and there is data suggesting that carbamazepine may worsen the EEG. Steroids may have a better therapeutic efficacy than standard AEDs for the aphasia in LKS.

It is important to distinguish a sleep-activated EEG pattern from the epileptic syndromes of LKS and CSWS. The EEG could show the pattern of marked sleep activation but the patient need not have the specific epileptic syndromes with either language or global regression.

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    What is a focal spike typical of a seizure?
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    What is body dyshormia?
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