Self-injurious behavior and tuberous sclerosis complex: frequency and possible associations in a population of 257 patients |
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| Authors: | Staley Brigid A Montenegro Maria A Major Philippe Muzykewicz David A Halpern Elkan F Kopp Claudine M C Newberry Peter Thiele Elizabeth A |
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| Institution: | aDepartment of Neurology, Massachusetts General Hospital, Boston, MA, USA;bMassachusetts General Hospital Institute for Technology Assessment, Boston, MA, USA;cDepartment of Psychiatry, Massachusetts General Hospital, Boston, MA, USA |
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| Abstract: | Self-injurious behavior (SIB) has been observed in people with tuberous sclerosis complex (TSC), although the frequency of SIB in TSC is largely unknown. SIB is associated with intellectual and developmental disabilities, but there is no single cause of SIB. We retrospectively examined the frequency of SIB in a population of 257 patients with TSC and determined possible associations with SIB. We found a 10% frequency of SIB in our TSC population. When compared with patients without psychiatric symptoms, we identified a significantly higher rate of electroencephalographic interictal spikes in the left frontal lobe and a significantly lower number of tubers in the left occipital, parietal, and posterior temporal lobes. We also found that frequency of TSC2 mutation, history of infantile spasms, history of seizures, mental retardation, and autism are significantly associated with SIB. |
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| Keywords: | Tuberous sclerosis complex Self-injurious behavior Epilepsy Frontal lobe Tuber location Behavioral problems |
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