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Abstract
BackgroundAutism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15–35% of the patients with epilepsy, more frequently in those with intellectual disability (ID). Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are two typical forms of intractable epileptic encephalopathy associated with ID. We previously reported that ASD was diagnosed in 24.3% of patients with DS, higher in those with profound ID. Given the severe epilepsy and high frequency of ID in LGS, it is necessary to know whether ASD is a common psychomotor co-morbidity of LGS. This study evaluated the autistic behaviors and intelligence in patients with LGS and further compared that between LGS and DS, aiming to understand the complex pathogenesis of epilepsy-ASD-ID triad.MethodsA total of 50 patients with LGS and 45 patients with DS were enrolled and followed up for at least 3 years. The clinical characteristics were analyzed, and evaluations of ASD and ID were performed.ResultsNo patients with LGS fully met the diagnostic criteria for ASD, but three of them exhibited more or less autistic behaviors. Majority (86%) of LGS patients presented ID, among which moderate to severe ID was the most common. Early onset age and symptomatic etiology were risk predictors for ID. The prevalence of ASD in LGS was significantly lower than that in DS (0/50 vs. 10/45, p < 0.001), while the prevalence and severity of ID showed no significant difference between the two forms of epileptic encephalopathy.ConclusionsThis study demonstrated a significant difference in the co-morbidity of ASD between LGS and DS, although they had a similar prevalence and severity of ID, refuting the proposal that the prevalence of ASD in epilepsy is accounted for by ID. These findings suggest that the co-morbidity of ASD, ID, and epilepsy may result from multifaceted pathogenic mechanisms.
Highlights
Autism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15–35% of the patients with epilepsy, more frequently in those with intellectual disability (ID)
Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy, which accounts for approximately 1–10% of childhood epilepsies [1]
We previously reported that ASD was diagnosed in 24.3% of patients with Dravet syndrome (DS), which is another form of epileptic encephalopathy with ID, and ASD was more often in those with profound ID [8]
Summary
Autism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15–35% of the patients with epilepsy, more frequently in those with intellectual disability (ID). We previously reported that ASD was diagnosed in 24.3% of patients with Dravet syndrome (DS), which is another form of epileptic encephalopathy with ID, and ASD was more often in those with profound ID [8]. Given the severe epilepsy and high frequency of ID in LGS, it is necessary to know whether autism or autistic behavior is a common psychomotor co-morbidity of LGS. As little is known about the relative contributions of epilepsy itself, ID, or other underlying factors to the occurrence of ASD in different forms of epileptic encephalopathy, a comparison between LGS and DS will help to understand the complex pathogenesis of epilepsy-ASD-ID triad
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