What is the prognosis of Lennox-Gastaut syndrome (LGS)?

Updated: Nov 09, 2018
  • Author: Koshi A Cherian, MD; Chief Editor: Amy Kao, MD  more...
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Answer

Long-term prognosis overall is unfavorable but variable in LGS. [6] Longitudinal studies have found that a minority of patients with LGS eventually could work normally, but 47-76% still had typical characteristics (mental retardation, treatment-resistant seizures) many years after onset and required significant help (eg, home care, institutionalization). [7]

Patients with symptomatic LGS, particularly those with an early onset of seizures, prior history of West syndrome, higher frequency of seizures, or constant slow EEG background activity, have a worse prognosis than those with idiopathic seizures.

Tonic seizures may persist and be more difficult to control over time, while myoclonic and atypical absences appear easier to control.

The characteristic diffuse slow spike wave pattern of LGS gradually disappears with age and is replaced by focal epileptic discharges, especially multiple independent spikes.

Mortality rate is reported at 3% (mean follow-up period of 8.5 y) to 7% (mean follow-up period of 9.7 y). Death often is related to accidents. A high rate of injuries is associated with atonic and/or tonic seizures.

The severity of the seizures, frequent injuries, developmental delays, and behavior problems take a large toll on even the strongest parents and family structures. Pay attention to the psychosocial needs of the family (especially siblings). The proper educational setting also is important to help the patient with LGS reach his or her maximal potential.


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