Late onset Lennox-Gastaut syndrome

Late-Onset Lennox-Gastaut Syndrome, is arbitrarily defined as Lennox-Gastaut Syndrome with onset after age 8 years, as opposed to its typical age of onset between 18 months and 8 years. Little is known about this late onset form of Lennox-Gastaut Syndrome. In this article, available information about this rare population of patients is reviewed, based on English language literature, to elucidate our current knowledge about the characteristic clinical presentation, known etiologies, comorbidities and long-term outcomes, as well as to identify future needs in research. The clinical characteristics of the Late-Onset Lennox Gastaut Syndrome described in the literature included relatively high number of patients with normal development and cognition at onset, low numbers of patients with preceding infantile spasms, and possibly a better cognitive outcome than its typical early onset form. Limitations rested on the variable age criterion used in different studies, limited data on imaging and genetics in many early studies, and only small numbers of patients included in each study. A study with a large number of patients with Lennox-Gastaut Syndrome, based on a unified diagnostic criterion, with detailed information on demographics, including evaluation with modern imaging modalities and genetic testing, and with long-term follow-up data is warranted to further delineate its accurate clinical characteristics.