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Synonyms: childhood epileptic encephalopathy
The Lennox-Gastaut syndrome is characterised by multiple types of epileptic seizures, a characteristic electroencephalogram (EEG) with generalised slow spike-and-wave discharges, psychomotor delay and behavioural disorders. The onset is usually before the age of 8, with a peak between the ages of 3 and 5 years. Late cases occurring in adolescence and early adulthood have rarely been reported.
- The most common seizure types are tonic-axial, atonic, and absence seizures; however, myoclonic, generalised tonic-clonic, and partial seizures may also occur.
- Drop attacks are a frequently recognisable seizure type and also the most dangerous physically, severely limiting quality of life.
- Seizures are often resistant to treatment.
- Lennox-Gastaut syndrome can be classified as either idiopathic (25% of the total) or symptomatic (75%). In idiopathic, normal psychomotor development occurs prior to the onset of symptoms and no neurological or neuroradiological abnormalities are found.
- Symptomatic cases are due to diverse cerebral conditions, which are usually bilateral, diffuse, or multifocal, involving cerebral grey matter.
- Examples of underlying disorders responsible for symptomatic Lennox-Gastaut syndrome include encephalitis, meningitis, tuberous sclerosis, brain malformations, birth injury, frontal lobe lesions and trauma.
- There is a prevalence of about 2 per 10,000 in Europe.
- The number of children affected is not known with certainty but it may account for about 3% of all childhood epilepsies.
- Males are affected five times more often than females.
Language is frequently affected, with both slowness in ideation and expression in addition to difficulties of motor dysfunction. Severe behavioural disorders (eg, hyperactivity, aggressiveness and autistic tendencies) and personality disorders are nearly always present. There is also a tendency for psychosis to develop with time.
- 20% of all patients with Lennox-Gastaut syndrome have prior infantile spasms with hypsarrhythmia.
- Although Lennox-Gastaut syndrome typically begins during childhood, it frequently persists through adolescence and on into adulthood. It may also, rarely, have late onset during adolescence or adulthood.
- Young children may show mood instability, personality disturbances, or slowing of psychomotor development.
- Older children experience personality problems, acute psychotic episodes, or chronic psychosis with aggressiveness, irritability, or social isolation.
- Mental deterioration leads to apathy and memory disorders.
- Physical examination can be important in helping to identify specific aetiologies although there are no pathognomonic physical findings.
- Other epileptic encephalopathies.
- Epilepsy with general learning disability.
- Infantile spasms.
- Juvenile myoclonic epilepsy.
- Myoclonic-astatic epilepsy.
- Electroencephalogram (EEG):
- The EEG features are not pathognomonic of the disorder.
- Interictal EEG is characterised by a slow background that can be either constant or transient.
- The characteristic interictal EEG pattern is 1.5-2.5 Hz slow spike-wave activity, which is bilaterally synchronous, dominant over the fronto-central regions, and usually symmetrical.
- The characteristic diffuse slow spike-wave pattern gradually disappears with age and is replaced by focal epileptic discharges.
- MRI scan is important in order to search for an underlying aetiology.
- Positron emission tomography or single-photon emission computed tomography may be useful during evaluation for epilepsy surgery.
A ketogenic diet appears useful in a minority of patients. Potentially serious adverse effects include dehydration, metabolic acidosis when the diet is initiated, renal stones, cardiac abnormalities and an abnormal lipid profile.
- Anticonvulsants are the mainstay of therapy but the optimum treatment for Lennox-Gastaut syndrome remains uncertain. A combination of drugs is usually required.
- The majority of cases remain refractory to medical management and require polytherapy, and only 10% of cases are estimated to undergo full seizure remission with available therapies.
- The optimum treatment remains uncertain and no study has shown any one drug to be highly effective; rufinamide, lamotrigine, topiramate and felbamate may be helpful as add-on therapy; clobazam may be helpful for drop seizures.
- Ethosuximide is also worth trying for atypical absences.
- Benzodiazepines can be effective in the remainder of seizure types but unfortunately the effect is often transient.
- Tonic seizures may persist and be more difficult to control over time, while myoclonic and atypical absences appear easier to control.
- Corpus callosotomy is effective in reducing drop attacks but typically is not helpful for other seizure types and is considered palliative rather than curative.
- Seizure freedom following corpus callosotomy is rare but can occur.
- Vagus nerve stimulation has been reported to reduce seizure frequency but the results are anecdotal.
- Injuries or death resulting from a seizure.
- Renal, cardiac, or metabolic complications resulting from a ketogenic diet.
- Long-term prognosis overall is unfavourable but variable.
- The epilepsy often improves but complete resolution of seizures is rare and the mental and psychiatric disorders tend to worsen with time.
- A minority of patients can eventually work normally, but many still have typical characteristics (intellectual disability, treatment-resistant seizures) many years after the onset.
- A worse prognosis is associated with symptomatic Lennox-Gastaut syndrome, early onset of seizures, prior history of infantile spasms, higher frequency of seizures, or constant slow EEG background activity.
Further reading & references
- Hancock EC, Cross JH; Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev. 2013 Feb 28;2:CD003277. doi: 10.1002/14651858.CD003277.pub3.
- VanStraten AF, Ng YT; Update on the management of Lennox-Gastaut syndrome. Pediatr Neurol. 2012 Sep;47(3):153-61. doi: 10.1016/j.pediatrneurol.2012.05.001.
- Markand ON; Lennox-Gastaut syndrome (childhood epileptic encephalopathy).; J Clin Neurophysiol. 2003 Nov-Dec;20(6):426-41.
- Epilepsy Society
- Kerr M, Kluger G, Philip S; Evolution and management of Lennox-Gastaut syndrome through adolescence and into adulthood: are seizures always the primary issue? Epileptic Disord. 2011 May;13 Suppl 1:S15-26. doi: 10.1684/epd.2011.0409.
- McTague A, Cross JH; Treatment of epileptic encephalopathies. CNS Drugs. 2013 Mar;27(3):175-84. doi: 10.1007/s40263-013-0041-6.
- Kaminska A, Oguni H; Lennox-Gastaut syndrome and epilepsy with myoclonic-astatic seizures. Handb Clin Neurol. 2013;111:641-52. doi: 10.1016/B978-0-444-52891-9.00067-1.
- Arzimanoglou A, French J, Blume WT, et al; Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, Lancet Neurol. 2009 Jan;8(1):82-93.
- Purcarin G, Ng YT; Experience in the use of clobazam in the treatment of Lennox-Gastaut syndrome. Ther Adv Neurol Disord. 2014 May;7(3):169-76. doi: 10.1177/1756285614521314.
- British National Formulary
- Aneja S, Sharma S; Newer anti-epileptic drugs. Indian Pediatr. 2013 Nov 8;50(11):1033-40.
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Dr Colin Tidy
Dr Colin Tidy
Dr John Cox