Lennox–Gastaut syndrome (LGS) is a severe pediatric epilepsy syndrome characterized by the presence of multiple pharmaco-resistant seizure types, including tonic, atypical absences, and tonic or atonic drop attacks, and the presence of electroencephalographic abnormalities, such as slow-spike waves and paroxysmal fast rhythms.
People with LGS begin having frequent seizures in early childhood, usually between ages 3 and 5. LGS is also a physically dangerous epilepsy syndrome of childhood because of the frequent falls, injuries, and cognitive impairment that can severely limit the quality of life. Children affected might previously have infantile spasms or underlying brain disorder, but etiology can be idiopathic also.
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In general, the causes of LGS is divided into two broader groups i.e, Identifiable causes and Cryptogenic (non-identifiable) causes. Identifiable causes include brain damage (e.g., head trauma), perinatal complications (e.g., birth asphyxia, intrauterine growth retardation, kernicterus), congenital central nervous system malformations (e.g., tuberous sclerosis), infections (e.g., meningitis, sepsis), or metabolic disorders.
Although EEG features are central to an accurate diagnosis of LGS, it is imperative to consider that they can take time to develop, particularly in young children. The SSW EEG pattern might not be present, initially, in patients who transforms to LGS from another epilepsy syndrome, such as West syndrome.
Therefore, for patients presenting with the seizure types associated with LGS, particularly tonic or atonic seizures, clinicians should be alert to the possibility of an LGS diagnosis and proactively monitor for EEG abnormalities to confirm its diagnosis.
While many underlying causes are still elusive, understanding of specific rare variations of epilepsy has recently enabled developers to test novel mechanisms in high-need patient populations like LGS.
Currently, the AEDs available for LGS is symptomatic drugs, used to treat the symptom “epilepsy” itself, but not to modify the natural history of the disease that so severely affects the patients’ QoL. The challenge for the near future will be to guide therapeutic choices based on etiology.
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The three main forms of treatment of Lennox-Gastaut syndrome are anti-epileptic drugs (AEDs), dietary therapy (typically the ketogenic diet) or device/surgery (VNS therapy or corpus callosotomy). In order to treat various types of seizures, treatment often revolves around the combination of multiple types of anti-epileptic drugs, which themselves can be associated with serious side effects in multidrug, high-dose regimens.
While many underlying causes are still elusive, understanding of specific rare variations of epilepsy has recently enabled developers to test novel mechanisms in high-need patient populations like LGS. Currently, the AEDs available for LGS are symptomatic drugs, used to treat the symptom “epilepsy” itself, but not to modify the natural history of the disease that so severely affects the patients’ QoL. The challenge for the near future will be to guide therapeutic choices based on etiology.
Valproate is considered as the first line of therapy for LGS as it is effective against a wide spectrum of seizures, the drug is administered as a monotherapy and if ineffective another drug such as lamotrigine, topiramate, rufinamide or clobazam may be added. It also has significant side effects ranging from tremor to reduction in platelet count and function as well as hyperammonemia.
A number of drugs have been approved by the US Food and Drug Administration (FDA) for the treatment of Lennox-Gastaut syndrome including Epidiolex, Topamax, Banzel, Lamictal, Felbatol, Onfi and Klonopin. Interestingly, none of these are the typical first choice of treatment.
The dynamics of Lennox Gastaut Syndrome market is anticipated to change in the coming years owing to the expected launch of emerging therapies during the forecasted period 2020–2030 such as Perampanel (Eisai Pharmaceuticals), ZX-008 (Zogenix International Limited) and TAK-935.
Next to the pharmacological approach, great emphasis should be given to a selective, surgical approach that would allow a more precocious control of epileptogenesis. Indeed, early seizures control has been associated with better neurocognitive endpoints. Other potential fields of future research will include other non-AED treatments and their potentiality to protect against cognitive impairment and even improve intellectual functions.
Various therapies are expected to enter the LGS market in the near future. Companies such as Eisai, Zogenix, and Takeda/Ovid and others are actively engaged in developing therapies for LGS.
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