Details

Autor(en) / Beteiligte

• Alsini, Hanin
• Alghamdi, Abdulaziz
• Alshafi, Shatha
• Hundallah, Khalid
• Almehmadi, Sameer
• Alsowat, Daad
• Al-Yamani, Suad
• Almuzaini, Hanin
• Alwadie, Ali
• Al-Otaibi, Ali
• Jad, Lamyaa
• Almadhi, Asma
• Bashiri, Fahad
• Kentab, Amal
• Hamad, Muddathir H
• Baarmah, Duaa
• Alrifaie, Mohammed
• Almuqbel, Mohammed
• Baradie, Raidah Al
• Mir, Ali
• Jan, Mohammed
• Muthaffar, Osama
• Aljabri, Mohammed
• Ali, Elsayed
• Saeed, Mohammed
• Matar, Abeer
• Tabarki, Brahim

Titel

Developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep in Saudi Arabia: Electroclinical, etiologic, genetic, and outcome multicenter study

Ist Teil von

• Seizure (London, England), 2023-04, Vol.107, p.146-154

Ort / Verlag

England: Elsevier Ltd

Erscheinungsjahr

2023

Quelle

ScienceDirect

Beschreibungen/Notizen

• •We evaluated the electro clinical characteristics of the Saudi Arabian children with D/EE-SWAS and compared their treatment outcomes.•The most prevalent etiologies of D/EE-SWAS were genetic/metabolic.•Children with genetic or metabolic diseases are more likely to experience D/EE-SWAS at a younger age than those with other etiologies.•Benzodiazepines and steroids were the most common treatment, and sodium valproate was the most frequently used antiseizure medication.•The combination therapy with steroids and benzodiazepines has a superior efficacy in terms of reduction of SWI and seizure response. To investigate the clinical features of developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (D/EE-SWAS), its electrographic characteristics, and etiology and to compare the effects of different treatment strategies on the outcomes using a Saudi Arabian database. This multicenter study included children with D/EE-SWAS who were evaluated between 2010 and 2020 at 11 tertiary centers. Data were collected on their baseline clinical features, etiologies, and treatment modalities. Seizure reduction, spike-wave index, and cognitive state were examined as potential therapeutic outcomes. Ninety-one children were diagnosed with D/EE-SWAS, with a median age of 7 years (IQR: 3–5) and an almost equal sex distribution. The average age at which epilepsy was diagnosed was 3 years (IQR: 5–2). A genetic/metabolic etiology was found in 35.1% of the patients, and a structural etiology was found in 27.4%. Children with underlying genetic/metabolic diseases exhibited an earlier seizure onset (P = 0.001) than children with other etiologies. Benzodiazepines (76.6%) were the most common treatment, followed by steroids (51.9%). Sodium valproate (75%) was the most frequently used antiseizure medication, followed by levetiracetam (64.9%). Children with a later seizure onset were more likely to have better clinical responses (P = 0.046), EEG responses (P = 0.012), and cognitive outcomes (P = 0.006) than children with an earlier onset. Moreover, better seizure response and electrographic response were seen in patients with bilateral interictal discharges on the EEG than otherwise. Children had a higher likelihood of both clinical and electrographic improvement with combination therapy of benzodiazepines (P = 0.001) and steroids (P = 0.001) than with other therapies. This study shows a higher prevalence of genetic/metabolic causes and suggests the superior efficacy of combination therapy with steroids and benzodiazepines in D/EE-SWAS. Prospective studies that strictly assess the treatment protocols and outcomes are needed.