Self-injurious behavior and tuberous sclerosis complex: Frequency and possible associations in a population of 257 patients

• Published in: Epilepsy & behavior Vol. 13; no. 4; pp. 650 - 653
• Main Authors: Staley, Brigid A, Montenegro, Maria A, Major, Philippe, Muzykewicz, David A, Halpern, Elkan F, Kopp, Claudine M.C, Newberry, Peter, Thiele, Elizabeth A
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.11.2008
• Subjects: Adolescent; Adult; Behavioral problems; Child; Electroencephalography; Epilepsy; Female; Frontal lobe; Humans; Male; Middle Aged; Neurology; Psychiatric Status Rating Scales; Self-injurious behavior; Self-Injurious Behavior - epidemiology; Self-Injurious Behavior - etiology; Tuber location; Tuberous Sclerosis - complications; Tuberous Sclerosis - epidemiology; Tuberous sclerosis complex; Young Adult; Frontal lobe; Tuber location; Behavioral problems; Self-injurious behavior; Epilepsy; Tuberous sclerosis complex
• ISSN: 1525-5050; 1525-5069
• DOI: 10.1016/j.yebeh.2008.07.010

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.