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

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. SIGNIFICANCE: 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.

Utility of single-photon emission computed tomography (SPECT) in presurgical evaluation of children: A single-center experience.

OBJECTIVE: Single-photon emission computed tomography (SPECT) is an ancillary noninvasive test commonly used to identify the epileptogenic zone. However, its proper utilization may be limited depending on the resources available at each center. This study aimed to investigate the utility of SPECT in presurgical evaluation of children at our center. METHODS: This was an observational retrospective study in 150 children who were admitted to the Epilepsy Monitoring Unit for presurgical evaluation between 2012 and 2019. The utility of interictal and ictal SPECT in different clinical cohorts was analyzed. RESULTS: Only interictal SPECT was performed on 87 patients (58 %), while ictal SPECT was performed on 62 (41.3 %), and interictal SPECT alone was considered of low diagnostic value. Ictal SPECT was unremarkable in 27 of 62 patients and abnormal in 35. Ictal SPECT was localized to the temporal lobe in 62.8 % and to the extratemporal lobe in 25.7 % of the patients and lateralized to one hemisphere in 11.4 % of the patients. In the abnormal SPECT group, ictal SPECT was considered unnecessary in 7/35 (20 %) patients with a single lesion and 3/35 (8.5 %) patients with a hemispheric lesion. In the remaining 25 patients, surgery was recommended more frequently than invasive EEG monitoring (IEM) for diffuse lesion cases (P = 0.03), while IEM was recommended more frequently than surgery for MRI-negative cases (P = 0.03), and in this group none of the MRI-negative patients underwent surgery. In our entire cohort, epilepsy surgery was performed on 24.4 % of the patients, 64 % with a single lesion, 7.6 % with a hemispheric lesion, 25.6 % with a diffuse lesion, and only one MRI-negative patient (2.5 %). Surgery was performed in 48.7 % of single lesion cases and 20.5 % of diffuse lesion cases with either unremarkable or no ictal SPECT. Engel class I outcome was achieved in 62 % and class II outcome in 33 % of the patients. In the single lesion etiology, 72 % (18/25) patients achieved excellent outcome and within this group, 22 % (4/18) patients had a positive ictal SPECT whereas 78 % (14/18) patients either did not get an ictal SPECT or it was unremarkable. SIGNIFICANCE: Based on our findings, we suggest carefully selecting patients for SPECT imaging to improve its utility and prevent overutilization and potential harm to children.