Shehata HS, et al. – Physicians designed this study to investigate the clinical characteristics and outcomes of status epilepticus (SE) in tuberous sclerosis complex (TSC) patients. In this patient sample, the incidence of SE was high (>50%). Risk factors for developing SE were severe mental retardation, autistic features, history of infantile spasm (epileptic spasms) and high tuber burden.

• The physicians carried out this observational, prospective study on 36 Egyptian children with definite TSC.
• They used clinical history, general and neurological examination and psychometric evaluation by standard questionnaires to explore characteristics of epileptic manifestations and clinical patterns of SE.
• They required all included patients to have long-term video electroencephalograms (EEGs) and brain MRI performed.

• In this cohort, 32 attacks of SE were recorded in 21 patients (58.3%) during a follow-up period of 2.8±1.1 years.
• Out of them, 15 had convulsive status, 7 had non-convulsive SE, 6 had refractory/super-refractory SE and 14 patients had a history of infantile spasms (epileptic spasms).
• In this study, the duration of status ranged from 40 to 150 min (mean ± standard deviation: 90±15).
• The physicians observed severe mental retardation in 14 patients with SE, 9 had autistic spectrum disorder and 22 had severe epileptogenic EEG findings.
• As per the outcomes, patients with SE had higher tuber numbers (mean: 9.6), 5 patients had subependymal giant cell astrocytomas and 2 patients had their SE after receiving everolimus.