Lennox-gastaut syndrome

Intellectual disability, the presence of multiple mixed seizure types, and characteristic EEG features including abnormal background activity and slow spike-waves that represents the triad to identify patients with the Lennox-Gastaut syndrome (LGS). Epileptic spasms and an EEG demonstrating hypsarrhythmia in West's syndrome may precede LGS in about 40% of cases. LGS, like many epileptic encephalopathies and other developmental disorders, manifest uncontrolled seizures stemming from many different structural-metabolic and less frequently unknown etiologies. The best course of treatment is the use of broad-spectrum anti-seizure medications (ASM), though most patients with LGS are drug resistant and subject to recurrent injury from recurrent uncontrolled seizures. Seizure types consist of tonic, tonic-clonic, atypical absence, myoclonic, and focal seizures. Balancing ASM in polytherapy with clinical tolerability of the medication optimizes overall quality of life in patients with LGS. Rescue benzodiazepine ASM may help limit the number of emergency department visits for serial seizures and/or status epilepticus. Judicious use of neuromodulation and dietary methods can be very helpful for some patients. Surgery is palliative, and involves disconnection surgeries such as corpus callosotomy. The overall prognosis for normal neurological development in patients with LGS and the likelihood to achieve seizure freedom from ASM treatment is poor.