Lennox-Gastaut Syndrome (LGS)
Incidence
LGS begins between 1 and 7 years with a peak at 3 to 5 years. The incidence is 2.8 per 10,000 live births. It accounts for 5 to 10% of children with seizures.
Clinical Characteristics
Lennox-Gastaut syndrome is a childhood epileptic encephalopathy characterized by a triad of polymorphic intractable seizures, cognitive and behavioral abnormalities, and an EEG with paroxysms of fast activity and slow (less than 2.5 Hz) generalized spike-wave discharges (GSWD). It begins between 1 and 7 years with a peak at 3-5 years. The incidence is 2.8 per 10,000 live births. It accounts for 5-10% of children with seizures. It is considered an epileptic syndrome. Symptoms can appear without apparent cause (cryptogenic) or result from obvious brain insult (symptomatic). Underlying pathologies include encephalitis and/or meningitis, tuberous sclerosis, brain malformations (eg, cortical dysplasias), birth injury, hypoxia-ischemia injury, frontal lobe lesions, and trauma and more rarely tumors or metabolic diseases. Infantile spasms precede the development of LGS in 9-39% of patients. Over 60% of brain MRI\\\'s are abnormal. The most common seizure types are tonic-axial, atonic, and absence seizures, but myoclonic, generalized tonic-clonic, and partial seizures can be observed. Seizures often are resistant to therapy. Behavioral abnormalities occur in half the cases. No pathognomonic physical findings are present in patients with LGS. Also consider Myoclonic-astatic epilepsy (Doose syndrome) as a differential diagnosis. The prognosis is poor. AED treatments nearly always fail to control seizures completely, although a reduction in seizures, usually temporarily, may be achieved. Surgical options include corpus callosotomy, vagus nerve stimulation, and focal cortical resection. Surgical alternatives may decrease the number of seizures but rarely lead to seizure freedom. Patients with LGS have a recognized high risk for status epilepticus. You should prescribe a medication for emergency intervention.
Precipitants
Seizures may be triggered by sleep deprivation, infections, fever, vomiting, or diarrhea.
Provocation Tests
Seizures may be triggered by sleep deprivation, infections, fever, vomiting, or diarrhea. The EEG is obligatory test to make the diagnosis.
Diagnostic Procedures
It is a clinical diagnosis. It is a triad of polymorphic intractable seizures, cognitive and behavioral abnormalities, and an EEG with paroxysms of fast activity and slow (less than 2.5 Hz) generalized spike-wave discharges (GSWD). The EEG is obligatory test to make the diagnosis.