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Historical note and nomenclature

An EEG with a 2 Hz ("slow") spike-and-wave pattern was first described in 1939 (Gibbs et al 1939). It was associated with a special type of absence seizure characterized by incomplete loss of consciousness. By contrast, classic petit mal absence seizures had been known to be associated with rhythmic 3 Hz spike-and-waves. The term "petit mal variant" was therefore used to describe the EEG pattern and the clinical seizure complex (Gibbs et al 1948).

Lennox and Davis first correlated the slow spike-and-wave EEG pattern with a distinctive group of clinical manifestations (eg, mental retardation and specific seizure types), including myoclonic jerks, atypical absences, and astatic seizures (drop attacks) (Lennox and Davis 1950). Gastaut and his colleagues described the clinical manifestations and EEG patterns of 100 patients with slow spikes-and-waves (Gastraut et al 1966). They called this syndrome "Lennox syndrome" or "childhood epileptic encephalopathy with diffuse slow spike-and-waves." The term "Lennox-Gastaut syndrome" first appeared in the literature in 1969 (Niedermeyer 1969). The International Classification of Epilepsies, Epileptic Syndromes, and Related Seizure Disorders classifies the Lennox-Gastaut syndrome as a cryptogenic or symptomatic generalized epilepsy (Anonymous 1989).

The concept of the myoclonic variant of Lennox-Gastaut syndrome was developed in the late 1980s (Aicardi 1995), but there is growing evidence that these patients are affected by the severe form of myoclonic-astatic epilepsy identified in the early 1970s (Doose 1985), thus illustrating the difficulties in distinguishing both conditions (Dulac et al 1998). Previous clinical descriptions differentiated these syndromes--myoclonic-astatic epilepsy rarely being identified as an exclusion criteria or a differential diagnosis.

Clinical manifestations

The Lennox-Gastaut syndrome may result from a variety of diffuse encephalopathies. It is characterized by the clinical triad of diffuse slow spikes-and-waves on EEG, mental retardation, and multiple types of generalized seizures, including especially atypical absences and tonic and atonic seizures (Beaumanoir 1985; Niedermeyer and Degen 1988; Aicardi and Levy Gomes 1992; Dulac and N'Guyen 1993). The Lennox-Gastaut syndrome is, with rare exception, a condition of children. The age of onset is between 2 and 8 years in most cases. Boys are affected more frequently than girls. Symptoms can appear de novo without apparent cause (cryptogenic Lennox-Gastaut syndrome) or result from obvious brain insult (symptomatic Lennox-Gastaut syndrome). Cryptogenic cases start later than symptomatic cases do.

In young children, the Lennox-Gastaut syndrome usually begins with episodes of sudden falls. In the school-age group, behavioral disturbances may be the heralding signs, along with drop attacks. This is soon followed by frequent seizures, episodes of status epilepticus, progressively deteriorating intellectual functions, personality disturbances, and chronic psychosis (Roger et al 1989).

Tonic seizures occur in most affected children. They are usually brief, lasting only seconds. Depending on the extent and groups of muscles involved, they may appear as axial (characterized by flexor movements of the head and trunk), axial rhizomelic (characterized by elevation and adduction of proximal upper limbs, stiffening of posterior neck muscles, elevation of shoulders, opening of the mouth, upward deviation of the eyes and brief apnea), or global, leading to sudden falls if the patient is in an upright position (Gastraut et al 1966). The seizures can be asymmetrical or predominantly unilateral. Sometimes automatic behaviors follow the tonic stage. Tonic seizures occur most frequently when falling asleep, but they can occur at any time of the day. They may occur subclinically and may be revealed only by a polygraphic sleep EEG tracing with simultaneous EMG recording. They may also be precipitated by stimuli such as noise, contact, or movement (Roger et al 1989).

Atypical absence seizures occur in approximately two-thirds of patients. Both the onset and the termination are gradual in contrast to the strikingly sudden lapses in typical absence. Whereas typical absences are usually brief (less than 10 seconds) and consciousness returns immediately and completely afterward, atypical absences are usually longer and are often followed by some postictal cognitive impairment. During the atypical absence there is "clouding" rather than loss of consciousness so that patients can continue their activity to some degree (Gastraut et al 1966). Associated manifestations are more common in atypical than in typical absences and include eyelid or perioral myoclonus, progressive flexion due to loss of postural tone, and localized motor phenomena, such as neck-stiffening or head-nodding (Roger et al 1989).

Atonic seizures are characterized by sudden, intense loss of postural tone that involves either the whole body or only the head (Roger et al 1989).

Other kinds of seizures, including partial and generalized tonic-clonic seizures, are less frequent (Roger et al 1989). Myoclonic seizures in which consciousness usually remains clear, enabling patients to arise without assistance promptly after falling, are rare in Lennox-Gastaut syndrome. They are a distinctive feature of myoclonic-astatic epilepsy, however (Doose 1985).

Myoclonic, myoclonic-atonic, atonic, and tonic seizures all cause falls (drop attacks) and are difficult to differentiate clinically from one another without polygraphic recording (Ikeno et al 1985). The falls result in recurrent injury, including lacerations that leave disfiguring scars (Roger et al 1989).

Most patients with the Lennox-Gastaut syndrome have 1 or more episodes of status epilepticus (Dulac and N'Guyen 1993). A variety of forms of status epilepticus occur that represent a continuum from absence status, consisting of an insidious confused state that can last for days or weeks, to pure tonic status epilepticus, which is more often seen in adolescents or adults than in children. Absence status may be difficult to recognize, particularly in children with severe mental retardation. Tonic status has been reported to be occasionally elicited by intravenous administration of benzodiazepines (Tassinari et al 1972).

In young children, slowing or even complete arrest of psychomotor development occurs. In cases of later onset, the intellectual impairment may be less pronounced than in cases of early onset (Gastraut et al 1966). In addition, behavioral abnormalities occur in half the cases, including hyperactivity (most commonly), emotional instability, aggressiveness, destructive behavior, autism, antisocial personality, or hypersexuality (Markand 1977). Such abnormalities and the arrest of educational progress are more prominent in older children and adolescents than in younger children. Chronic psychosis with episodes of acute exacerbation may also occur (Roger et al 1989; Jambaque et al 2002).

Interictal neurologic symptoms are not specific for Lennox-Gastaut syndrome, but they are determined by the location and extent of the underlying pathology. Motor signs occur in 59% of cases. Cerebellar signs may be difficult to differentiate from effects of antiepileptic drugs (Markand 1977). Seventeen percent of patients have normal neurologic examinations.

Clinical vignette

No information was provided by the author.

Etiology

In about 30% of cases, the symptoms appear without antecedent history or evidence of brain pathology. Such cases are called "cryptogenic" Lennox-Gastaut syndrome. It is this group that is difficult to distinguish from myoclonic-astatic epilepsy. There is no evidence of a genetic predisposition even though previous studies may have reported a higher incidence of epilepsy or febrile convulsions in these patients than in the general population before the distinction between Lennox-Gastaut Syndrome and myoclonic-astatic epilepsy was made.

The other 70% of cases are "symptomatic." They are associated with preexistent brain damage, usually acquired in the prenatal or neonatal periods or in infancy. Pre and perinatal factors comprise ABO blood group incompatibility, prematurity, abnormal presentation, prolonged labor, cord prolapse, respiratory depression, and various malformations including tuberous sclerosis and porencephaly. With the advent of high resolution MRI, cortical dysplasias are being identified as an increasingly common substrate of the Lennox-Gastaut syndrome (Ricci et al 1992). Band heterotopia is a newly identified cause of Lennox-Gastaut syndrome (Barkovich et al 1994; Granata et al 1994). Postnatal factors include central nervous system infection, degenerative or metabolic disorders of the nervous system, head injury, anoxic encephalopathy, cerebrovascular accident, hypoglycemia, and irradiation leukoencephalopathy (Markand 1977; Mitsufuji et al 1996). An autoimmune mechanism has been proposed for some cases, but there still is little evidence to support this concept (van Engelen et al 1995).

Approximately one-third of patients with symptomatic Lennox-Gastaut syndrome represent evolution from infantile spasms, occurring in infants and young children (Gastraut et al 1966).

The age range of occurrence suggests that some maturational, yet unidentified, factor plays a determinant role in the development of Lennox-Gastaut Syndrome.

Pathogenesis and pathophysiology

No specific pathophysiological mechanisms have been demonstrated to underlie the Lennox-Gastaut syndrome; however, the frontal lobes appear to play a predominant role in the epileptic symptoms, and the usual age of appearance of this syndrome corresponds with maturation of the frontal lobes (Dulac and N'Guyen 1993). There is evidence that subcortical structures are involved in the epileptogenic process (Velasco et al 1991).

The major so-called ”interictal” spike-wave activity seems to play a major role in the development of cognitive troubles compared to the seizures themselves, which are “minor” as commonly mentioned. Lennox-Gastaut syndrome is one of the epileptogenic encephalopathies, a condition between epilepsy and status epilepticus that also comprises infantile spasms and continuous spike waves in slow sleep.

The relative role of spike waves and fast activity is probably related to the ability of neuronal network to produce slow waves that are related to GABA release and to interrupt the fast activity (Blume 2001; Halasz and Janszky 2002).

Epidemiology

Although the incidence of the Lennox-Gastaut syndrome is low, the intractable nature of this disorder accounts for a prevalence of 5% of epileptic patients of all ages and about 10% of epileptic patients under 15 years of age (Gastaut et al 1975; Heiskala 1997; Trevathan et al 1997).

Prevention

No information is available.

Differential diagnosis

Drop attacks and slow spike-and-wave discharges on EEG may also be seen in patients who have conditions other than Lennox-Gastaut syndrome, including overdose of certain antiepileptic medication and partial seizures secondary to structural lesions. Both may produce continuous spike waves in slow sleep (Patry et al 1971). Posttraumatic epilepsies and encephalopathy with multifocal epilepsy associated with secondary generalization may produce frontal lobe epilepsy with secondary generalization of the spike-wave activity (Roger et al 1989). In these patients, the tonic seizures are usually missing, and onset is at a later age.

Three other epilepsy syndromes may produce drop attacks. Epilepsy with myoclonic-astatic seizures begins between 2 years and 5 years of age with generalized tonic-clonic seizures. This is followed, several months later, by frequent daily drop attacks due to myoclonic-astatic seizures with atypical absences (Doose 1985). The EEG reveals 2 Hz to 3 Hz spike-and-wave discharges and a slow baseline activity.

The syndrome of continuous spike-waves in slow sleep begins between 3 years and 6 years of age with atonic seizures and atypical absences with slow spike-waves, but there are no tonic seizures (Patry et al 1971).

Infantile spasms that produce drop attacks may begin between 1 year and 4 years of age. The drop attacks can be identified as the result of epileptic spasms only through video-EEG recordings of the seizures (Bednarek et al 1998).

Diagnostic workup

The diagnosis is based on the combination of several types of generalized seizures. Tonic and atypical absences may need ictal EEG recording to be properly identified; irregular slow spike-and-waves can be identified with interictal awake tracing; whereas polyspikes-and-waves are recorded during sleep. Cognitive and behavior deterioration may be missing at onset.

The slow spike-and-wave consists of a blunt, slow spike (approximately 150 msec), followed by a slow wave (approximately 350 msec). The amplitude ranges from 200 microvolts to 800 microvolts. When 2 spikes to 3 spikes precede the slow wave, they constitute a polyspike-and-wave complex. The frequency (1.5 Hz to 2.5 Hz) is relatively slow and arrhythmic compared to that of classical absence seizures (rhythmic 3 Hz). Occasionally, bursts of rapid spike-and-waves at 3 Hz or even 4 Hz may occur in combination with the slow spike-and-waves (Gastraut et al 1966). Generalized polyspike discharges or lower voltage fast activity (generalized paroxysmal fast activity) lasting 1 second or more without obvious clinical correlates are common during slow wave sleep. Relaxation, drowsiness, sleep, and hyperventilation facilitate the appearance of slow spike-and-waves. During sleep, the abnormal patterns become more prominent, symmetrical, and synchronous, with even slower spike-and-waves or polyspikes-and-waves. Photic stimulation, on the other hand, has no effect on these EEG events (Markand 1977). When evolution in EEG patterns occurs in patients previously affected by infantile spasms, the direction is from hypsarrhythmia to multifocal interictal spikes to generalized spike discharges to slow spike-and-waves, with the last representing a stable pattern that characterizes children with the Lennox-Gastaut syndrome (Kotagal 1995).

The interictal EEG discharges are usually generalized, bilateral, synchronous, and symmetrical. But they may also be asymmetrical and predominant in 1 hemisphere or 1 region. The EEG patterns differ among individuals and change from day to day and even moment to moment (Gastraut et al 1966).

The EEG correlate of tonic seizures consists of a 10 Hz to 13 Hz recruiting rhythm, usually followed by high amplitude slow activity rather than postictal EEG depression (Dulac and N'Guyen 1993). Although the EEG during absence seizures often shows irregular spike-and-wave discharges, these patterns are not necessarily different from interictal EEG discharges and do not clearly demarcate the occurrence of an ictal event. Similarly, there is no characteristic pattern of absence status; this prolonged confused state may be associated with an increase in slow spike-and-wave activity or with irregular slowing resembling hypsarrhythmia (Dulac and N'Guyen 1993).

A variety of multifocal or diffuse abnormalities on MRI are found in the symptomatic cases, but this finding concerns etiology, not diagnosis, of the epilepsy syndrome.

Prognosis

The prognosis is poor, and the syndrome does not evolve into a localization-related epilepsy (Yagi 1996). Remission with preserved mentation occurs in very few patients; rather, IQ tends to deteriorate with age (Oguni et al 1996), and tonic seizures persist, but the slow spike-and-wave pattern does tend to resolve. Patients with underlying brain damage particularly preexistent West syndrome, early onset, frequent and slow evolution of seizures, and repeated episodes of status have a relatively worse prognosis (Roger et al 1989).

Management

The treatment of Lennox-Gastaut syndrome has been difficult and disappointing. Carbamazepine and phenytoin can control generalized tonic-clonic convulsions and reduce tonic seizures, but these drugs can also exacerbate atypical absence seizures. Valproate offers a chance for improvement of all seizure types with a single drug but soon loses its effect.

Lamotrigine has also shown a significant effect on drop attacks although atypical absences were not significantly affected (Motte et al 1997). However, this drug should be introduced slowly in patients receiving valproate in order to prevent serious rash (Dulac and Kaminska 1997).

Felbamate has been reported to reduce the frequency and severity of drop attacks (Felbamate Study Group 1993; Avanzini et al 1996) and other seizure types (Jensen 1994) and can improve behavior in some patients (Gay et al 1995). However, this drug is associated with a high incidence of serious side effects and should be used with caution. It should be used with bimonthly follow-up of transaminases and blood cell counts; it should be restricted to cases intractable by other compounds; and it should be given for no longer than 2 months in case there is no clear response.

Topiramate significantly reduces the frequency of drop attacks, but in 1 trial, drop attacks persisted daily in most patients. In addition, the cognitive side effects of the drug may cause serious concerns (Glauser 1998). Thus, the future place of this compound in the treatment of Lennox-Gastaut syndrome still needs to be evaluated.

Other drugs that have been effective in uncontrolled trials include nitrazepam (Chamberlain 1996). Vigabatrin has yielded variable results (Feucht and Brantner-Inthaler 1994). In 1 case report, seizures were made worse by gabapentin (Vossler 1996). Corticosteroid treatment with gradually tapered dosages may be helpful in idiopathic cases at onset, in status epilepticus, or during periods of worsening in the course of evolution (Roger et al 1989). Clinical trials of intravenous immunoglobulin have yielded equivocal results (Illum et al 1990; van Engelen et al 1994). Ketogenic diet, amantadine, tryptophane, flumazenil, imipramine, and many other treatments have had limited success in some patients (Dulac and N'Guyen 1993).

Although promising, vagal nerve stimulation still awaits further research (Lundgren et al 1998; Frost et al 2001).

Corpus callosotomy can reduce or abolish drop attacks in many patients with Lennox-Gastaut syndrome provided there is no major diffuse brain malformation, but it influences the pattern of other seizure types only rarely (Gates et al 1993). With the advent of new microsurgical techniques and the realization that anterior two-thirds section is sufficient in most patients, adverse side effects of corpus callosotomy have been minimized.

Pregnancy

Although no information is available that is specific to this syndrome and pregnancy, information is available on epilepsy and pregnancy.

Anesthesia

No information is available.

References cited

Aicardi J. Myoclonic epilepsies difficult to classify as either Lennox-Gastaut or myoclonic-astatic epilepsy. In: Wallace S, editor. Epilepsy in children. London: Chapmann and Hill, 1995: 271-3.

Aicardi J, Levy Gomes A. Clinical and electroencephalographic symptomatology of the 'genuine' Lennox-Gastaut syndrome and its differentiation from other forms of epilepsy of early childhood. Epilepsy Res 1992;6(Suppl):185-93.

Anonymous. Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30(4):389-99.

Avanzini G, Canger R, Dalla Bernardina B, Vigevano F. Felbamate in therapy-resistant epilepsy: an Italian experience. Felbamate Italian study group. Epilepsy Res 1996;25(3):249-55.

Barkovich AJ, Guerrini R, Battaglia G, et al. Band heterotopia: correlation of outcome with magnetic resonance imaging parameters. Ann Neurol 1994;36(4):609-17.

Beaumanoir A. The Lennox-Gastaut syndrome. In: Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P, editors. Epileptic syndromes in infancy, childhood and adolescence. London: John Libbey, 1985:89-99.

Bednarek N, Motte J, Soufflet C, Plouin P, Dulac O. Evidence of late-onset infantile spasms. Epilepsia 1998;39(1):55-60.

Blume WT. Pathogenesis of Lennox-Gastaut syndrome: considerations and hypotheses. Epileptic.Disord. 2001;3:183-96.

Chamberlain MC. Nitrazepam for refractory infantile spasms and the Lennox-Gastaut syndrome. J Child Neurol 1996;1:31-4.

Doose H. Myoclonic astatic epilepsy of early childhood. In: Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P, editors. Epileptic syndromes in infancy, childhood and adolescence. London: John Libbey Eurotext, 1985:103-14.

Dulac O, Kaminska A. Use of lamotrigine in Lennox-Gastaut and related epilepsy syndromes. J Child Neurol 1997;12(suppl 1): S23-8.

Dulac O, N'Guyen T. The Lennox-Gastaut syndrome. Epilepsia 1993;34(Suppl 7):S7-17.

Dulac O, Plouin P, Shewmon A, Contributors to the Roymont workshop. Myoclonus and epilepsy in childhood. 1996 Royaumont meeting. Epilepsy Res 1998;30:91-106.

Felbamate Study Group in Lennox-Gastaut Syndrome. Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). N Engl J Med 1993;328:29-33.

Feucht M, Brantner-Inthaler S. Gamma-vinyl-GABA (vigabatrin) in the therapy of Lennox-Gastaut syndrome: an open study. Epilepsia 1994;35:993-8.

Frost M, Gates J, Helmers SL, et al. Vagus nerve stimulation in children with refractory seizures associated with Lennox-Gastaut syndrome. Epilepsia 2001;42(9):1148-52.

Gastaut H, Gastaut JL, Goncalves E, Silva GE, Fernandez Sanchez GR. Relative frequency of different types of epilepsy: a study employing the classification of the International League Against Epilepsy. Epilepsia 1975;16:457-61.

Gastraut H, Roger J, Soulayrol R, et al. Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as "Petit mal variant") or Lennox syndrome. Epilepsia 1966;7(2):139-79.

Gates JR, Wada JA, Reeves A, et al. Re-evaluation of corpus callosotomy. In: Engel J Jr, editor. Surgical treatment of the epilepsies. 2nd edition. New York: Raven, 1993:637-48.

Gay PE, Mecham GF, Coskey JS, Sadler T, Thompson JA. Behavioral effects of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). Psychol Rep 1995;77:1208-10.

Gibbs FA, Gibbs EL, Lennox WG. Electroencephalographic classification of epileptic patients and control subjects. Arch Neurol Psychiatry 1948;50:111-28.

Gibbs FA, Gibbs EL, Lennox WG. Influence of blood sugar level on wave and spike formation in petit mal epilepsy. Arch Neurol Psychiatry 1939;41:1111-6.

Glauser TA. Topiramate use in pediatric patients. Can J Neurol Sci 1998;39:874-77.

Granata T, Battaglia G, D'Incerti L, et al. Double cortex syndrome: electroclinical study of three cases. Ital J Neurol Sci 1994;15(1):15-23.

Halasz P, Janszky J. Paroxysmal fast activity and Lennox-Gastaut syndrome. Epileptic Disord. 2002;4(2):163.

Heiskala H. Community-based study of Lennox-Gastaut syndrome. Epilepsia 1997;38:526-31.

Ikeno T, Shigematsu H, Miyakoshi M, Ohba A, Yagi K, Seino M. An analytic study of epileptic falls. Epilepsia 1985;26:612-21.

Illum N, Taudorf K, Heilmann C, et al. Intravenous immunoglobulin: a single-blind trial in children with Lennox-Gastaut syndrome. Neuropediatrics 1990;21(2):87-90.

Jambaque I, Lassonde M, Dulac O. The neuropsychology of childhood epilepsy. New York: Plenum Press, 2002.

Jensen PK. Felbamate in the treatment of Lennox-Gastaut syndrome. Epilepsia 1994;35(Suppl 5):S54-7.

Kotagal P. Multifocal independent spike syndrome: relationship to hypsarrythmia and the slow spike-wave (Lennox-Gastaut) syndrome. Clin Electroencephalogr 1995;26:23-9.

Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 1950;5:626-44.

Lundgren J, Amark P, Blennow G, Stromblad LG, Wallstedt L. Vagus nerve stimulation in 16 children with refractory epilepsy. Epilepsia 1998;39(8):809-13.

Markand ON. Slow spike-wave activity in EEG and associated clinical features: often called "Lennox" or "Lennox-Gastaut" syndrome. Neurology 1977;27:746-57.

Mitsufuji N, Ikuta H, Yoshioka H, Sawada T. Lennox-Gastaut syndrome associated with leukoencephalopathy. Pediatr Neurol 1996;15(1):63-5.

Motte J, Trevathan E, Arvidsson JF, Barrera MN, Mullens EL, Manasco P. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group. N Engl J Med 1997;337(25):1807-12.

Niedermeyer E. The Lennox-Gastaut syndrome, a severe type of childhood epilepsy. Electroencephalogr Clin Neurophysiol 1969;24:283.

Niedermeyer E, Degen R, editors. The Lennox-Gastaut syndrome: neurology and neurobiology. Vol. 45. New York: Alan R. Liss, 1988:484.

Oguni H, Hayashi K, Osawa M. Long-term prognosis of Lennox-Gastaut syndrome. Epilepsia 1996;37(Suppl 3):44-7.

Patry G, Lyagoubi S, Tassinari CA. Subclinical electrical status epilepticus induced by sleep in children. Arch Neurol 1971;24:242-52.

Ricci S, Cusmai R, Fariello G, Fusco L, Vigevano F. Double cortex. A neuronal migration anomaly as a possible cause of Lennox-Gastaut syndrome. Arch Neurol 1992;49:61-4.

Roger J, Dravet C, Bureau M. The Lennox-Gastaut syndrome. Cleve Clin J Med 1989;56(Suppl Pt 2):S172-80.

Tassinari CA, Dravet C, Roger J, Cano JP, Gastaut H. Tonic status epilepticus precipitated by intravenous benzodiazepines in five patients with Lennox-Gastaut syndrome. Epilepsia 1972;13:421-35.

Trevathan E, Murphy CC, Yeargin-Allsopp M. Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia 1997;38(12):1283-8.

van Engelen BG, Renier WO, Weemaes CM, Strengers PF, Bernsen PJ, Notermans SL. High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome: an add-on study. Eur J Pediatr 1994;153:762-9.

van Engelen BG, Weemaes CM, Renier WO, Bakkeren JA, Borm GF, Strengers PF. A dysbalanced immune system in cryptogenic Lennox-Gastaut syndrome. Scand J Immunol 1995;41:209-13.

Velasco M, Velasco F, Alcala H, Davila G, Diaz-de-Leon AE. Epileptiform EEG activity of the centromedian thalamic nuclei in children with intractable generalized seizures of the Lennox-Gastaut syndrome. Epilepsia 1991;32:310-21.

Vossler DG. Exacerbation of seizures in Lennox-Gastaut syndrome by gabapentin. Neurology 1996;46(3):852-3.

Yagi K. Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study. Epilepsia 1996;37(Suppl 3):48-51.

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Abbreviations

EEG:electroencephalogram

EMG:electromyography

MRI:magnetic resonance imaging

ICD-9 code

345.0

Synonyms

Akinetic epilepsy

Akinetic petit mal

Childhood epileptic encephalopathy with diffuse slow spike-and-waves

Lennox syndrome

Minor motor epilepsy

Mixed seizure disorder

Petit mal variant

Propulsive petit mal

Severe myokinetic epilepsy of early childhood with slow spike-and-waves

Subtopics

Cryptogenic Lennox-Gastaut syndrome

Secondary generalized epilepsy

Symptomatic generalized epilepsy

Symptomatic Lennox-Gastaut syndrome

Associated disorders

Anoxic encephalopathy

Brain malformations

Central nervous system infection

Degenerative or metabolic disorders of the nervous system

Encephalopathy with multifocal epilepsy

Head injury

Porencephaly

Stroke

Tuberous sclerosis

West syndrome

Major keyword descriptors

ABO blood group incompatibility

absence seizures

aggressiveness

astatic seizures

atonic seizures

atypical absence seizures

autism

automatic behavior

cerebellar signs

clonic seizures

corpus callosotomy

cortical dysplasias

corticosteroids

drop attacks

felbamate

generalized tonic-clonic seizures

head nodding

hyperactivity

hypoglycemia

intellectual deterioration

intravenous immunoglobulin

lamotrigine

multiple seizure types

myoclonic seizures

myoclonus

personality changes

prematurity

prolonged labor

psychosis

rhizomelic seizures

sleep

slow spike-and-wave

tonic seizures

valproate

Minor keyword descriptors

epilepsy

mental retardation

Age of presentation

02-05 years

06-12 years

Age of typical presentation

02-05 years

Population group(s) preferentially affected

none selectively affected

Occupation group(s) preferentially affected

none selectively affected

Sex

male>female, >2:1

Family history

none

Heredity

none

Glossary

Lennox-Gastaut syndrome: A childhood-onset epileptic syndrome characterized by the clinical triad of diffuse slow spike-and-waves on EEG, mental retardation, and multiple seizure types in the same patient, including atypical absences and tonic and atonic seizures.

Permuted topic, synonyms, subtopics

Lennox-Gastaut syndrome

Gastaut syndrome, Lennox-

epileptic encephalopathy with diffuse slow spike-and-waves, Childhood

encephalopathy with diffuse slow spike-and-waves, Childhood epileptic

diffuse slow spike-and-waves, Childhood epileptic encephalopathy with

slow spike-and-waves, Childhood epileptic encephalopathy with diffuse

spike-and-waves, Childhood epileptic encephalopathy with diffuse slow

myokinetic epilepsy of early childhood with slow spike-and-waves, Severe

epilepsy of early childhood with slow spike-and-waves, Severe myokinetic

epilepsy, Akinetic

motor epilepsy, Minor

epilepsy, Minor motor

generalized epilepsy, Minor

epilepsy, Secondary generalized

generalized epilepsy, Secondary

petit mal, Akinetic

petit mal, Propulsive

seizure disorder, Mixed

generalized epilepsy, Symptomatic

epilepsy, Symptomatic generalized

Lennox-Gastaut syndrome, Cryptogenic

Lennox-Gastaut syndrome, Symptomatic

Related topics

Epilepsy

Epilepsy with continuous spike-and-waves during slow wave sleep

Sleep disorders associated with epilepsy

Differential diagnosis

antiepileptic medication overdose

partial seizures secondary to structural lesions

posttraumatic epilepsies

encephalopathy with multifocal epilepsy

epilepsy with myoclonic-astatic seizures

epilepsy with myoclonic absences

continuous spike waves in slow sleep

late onset infantile spasms