ArticleTherapeutics and clinical risk management2017
Clinical patterns and outcomes of status epilepticus in patients with tuberous sclerosis complex.
Article in Therapeutics and clinical risk management, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 18 citations in OpenAlex.
- Genotypic and phenotypic features of 23 Egyptian patients with tuberous sclerosis complex.BMC pediatrics · 2026Observational
- A Cost-Utility Analysis of Add-On Cannabidiol Versus Usual Care Alone for the Treatment of Seizures Associated with Tuberous Sclerosis Complex in England and Wales.PharmacoEconomics - open · 2024Article
- Successful use of Fenfluramine in super-refractory status epilepticus in a patient with tuberous sclerosis complex and Lennox-Gastaut syndrome.Epilepsy & behavior reports · 2024Article
- Aberrant DJ-1 expression underlies L-type calcium channel hypoactivity in dendrites in tuberous sclerosis complex and Alzheimer's disease.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- Tuberous Sclerosis Complex in a 17-month-old: A Case Report.JNMA; journal of the Nepal Medical Association · 2023Article
- Perfect match: mTOR inhibitors and tuberous sclerosis complex.Orphanet journal of rare diseases · 2022Review
- Acute encephalopathy in children with tuberous sclerosis complex.Orphanet journal of rare diseases · 2021Article
- Infantile Hemiconvulsion-Hemiplegia and Epilepsy (IHHE) in a boy with tuberous sclerosis complex.Epilepsy & behavior reports · 2021Article
- Article
- High glucose concentrations mask cellular phenotypes in a stem cell model of tuberous sclerosis complex.Epilepsy & behavior : E&B · 2019Article
- Non-convulsive status epilepticus in two patients with tuberous sclerosis.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2019Article
- Factors affecting epilepsy prognosis in patients with tuberous sclerosis.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2019Article
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionRefractory epilepsy is a common clinical manifestation in patients with tuberous sclerosis complex (TSC), which can be complicated by many life-threatening conditions, such as status epilepticus (SE). However, very few reports mention the patterns and semiology of SE in those patients.
objectiveTo study the clinical characteristics and outcomes of SE in TSC patients. MATERIALS AND
methodsThis observational, prospective study was carried out on 36 Egyptian children with definite TSC. Clinical history, general and neurological examination and psychometric evaluation by standard questionnaires were used to explore characteristics of epileptic manifestations and clinical patterns of SE. All included patients were required to have long-term video electroencephalograms (EEGs) and brain MRI performed.
resultsA total of 32 attacks of SE were recorded in 21 patients (58.3%) in our cohort during a follow-up period of 2.8±1.1 years; of those patients, 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). The duration of status ranged from 40 to 150 min (mean ± standard deviation: 90±15). Fourteen patients with SE had severe mental retardation, 9 had autistic spectrum disorder and 22 had severe epileptogenic EEG findings. 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.
conclusionsThe incidence of SE in our patient sample is high (>50%); severe mental retardation, autistic features, history of infantile spasm (epileptic spasms) and high tuber burden are risk factors for developing SE.
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