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Clinical patterns and outcomes of status epilepticus in patients with tuberous sclerosis complex

Authors Shehata HS, AbdelGhaffar HM, Nasreldin M, Elmazny A, Abdelalim A, Sabbah A, Shalaby NM

Received 1 April 2017

Accepted for publication 25 May 2017

Published 30 June 2017 Volume 2017:13 Pages 779—785

DOI https://doi.org/10.2147/TCRM.S138576

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Hoa Le

Peer reviewer comments 3

Editor who approved publication: Professor Garry Walsh

Hatem S Shehata,1 Hadeer Mahmoud AbdelGhaffar,2 Mohammed Nasreldin,3 Alaa Elmazny,1 Ahmed Abdelalim,1 Asmaa Sabbah,1 Nevin M Shalaby1

1
Department of Neurology, Cairo University, Giza, 2Department of Paediatrics, Fayoum University, Fayoum, 3Department of Psychiatrics, Cairo University, Giza, Egypt

Introduction: Refractory 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.
Objective: To study the clinical characteristics and outcomes of SE in TSC patients.
Materials and methods: This 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.
Results: A 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.
Conclusions: The 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.

Keywords: Egyptian children, tuberous sclerosis, status epilepticus, infantile spasms, autistic spectrum

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