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LENNOX-GASTAUT SYNDROME (LGS)

  Mandatory Alerts Exclusionary
Seizures Tonic seizures and ≥1 of:
Atypical absences
Atonic seizures
Myoclonic seizures
Focal impaired awareness seizures
Generalized tonic-clonic seizures
Epileptic spasms
Nonconvulsive status epilepticus
   
EEG Generalized slow (<2.5Hz) spike-wave
Generalized paroxysmal fast activity in sleep
Photoparoxysmal response at low flash frequency (consider CLN2)  
Age at onset <18 years >8 years  
Course of illness Drug resistant epilepsy
Intellectual impairment
   
Are MRI or ictal EEG required for diagnosis?
An MRI is not required for diagnosis but is usually performed to look for a cause
An ictal EEG is not required, however should be strongly considered where alerts are present or if epilepsy with myoclonic atonic seizures is a differential
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome cannot be diagnosed without EEG showing at least generalised slow spike-wave in wakefulness.

NOTE Alert criteria are absent in the vast majority of patients with the syndrome, but rarely can be seen. Their presence should result in caution in diagnosing the syndrome and consideration of other conditions

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