Background Although it is a valuable option for children with drug-resistant epilepsy, ketogenic diet (KD) therapy is associated with several side effects. The frequency of kidney stones and risk factors for their development in epileptic children receiving KD is unclear. The aim of this study was to determine the frequency and risk factors for the development of renal stones in children receiving KD therapy. Methods A total of 95 patients receiving KD were identified. Of these, seven patients were excluded from the study due to the duration of KD being less than 12 months. The remaining 88 children were enrolled in the study. Results Renal stones were detected in 15 patients (17%), of which 12 (73.3%) received potassium citrate treatment. Two (13.3%) patients needed lithotripsy despite receiving potassium citrate treatment, and one of these, who received potassium citrate treatment for 5 months, developed acute vesicourethral reflux and underwent surgery. No patient discontinued KD due to renal stone development. The serum uric acid concentrations and urine calcium/creatinine ratio did not change significantly over the 24-month follow-up period. Age, gender, etiology, age at seizure onset, duration of KD, mobility status, use of topiramate or zonisamide, and the number of antiepileptic drugs used were not significantly different between patients with and without kidney stones. Conclusion Renal stone appears to be a common adverse effect of KD therapy. Although adequate hydration and potassium citrate treatment are effective in most patients, lithotripsy and surgery may be required in a minority of patients.

Purpose: Electroencephalography (EEG) recording in pediatric patients is difficult because of patient compliance, and children who are unsuitable for behavioral training usually require sedation for EEG recordings. The aim of this study was to examine the effects of agents commonly used in daily practice for sleep induction on sleep transition, sleep architecture, and frequency of movement artifacts on EEG recordings. Methods: A retrospective analysis was made of the demographic data and sleep EEG recordings of patients who underwent sleep EEG because of suspected seizure between 2021 and 2022. The study included patients aged 4 to 18 years, and patients with a new or previous diagnosis of epilepsy were excluded from the study. Results: Evaluation was made of 88 patients, comprising 35 (39.8%) girls and 53 (60.2%) boys with a mean age of 10.6 ± 4.3 years. In the analysis of the patients who did not sleep at all during the EEG recording, the drugs administered to the patients for sedation were observed to be hydroxyzine in 4 (14.8%) patients, chloral hydrate in 1 (2.8%) patient, and melatonin in 5 (20%) patients. Sleep duration was seen to be longer and the rates of motion artifacts and awakening during filming were lower in the patients treated with chloral hydrate. Conclusions: The results of this study showed that chloral hydrate was the most effective drug for inducing sleep. There is a need for further similar prospective studies to be performed on patients diagnosed with epilepsy and those aged <4 years. Possible complications should be considered before using each agent for sleep induction.

Background. Spinal muscular atrophy (SMA) is an inherited disease with progressive muscle weakness and atrophy. Despite the new treatments developed recently, primary and secondary effects of muscle weakness in patients with SMA cause mortality and morbidity. The aim of this study is to identify common problems in the follow-up of patients after new treatment modalities and to examine the difficulties in management of these problems. Methods. The study included 16 patients diagnosed with SMA type 1 according to clinical findings and genetic results between 2017 and 2022. The patients were divided into two groups as living and deceased, and complications were examined and compared between the groups. Results. The patients comprised 8 (50%) females and 8 (50%) males with a median age at diagnosis of 3 months. The patients had a history of gastrointestinal problems, orthopedic problems, infection and sepsis, and especially respiratory distress. Death occurred in 8 (50%) patients during follow-up (median age 38 months). Mortality was higher in patients who needed tracheostomy and had gastroesophageal reflux. The survival rate was better in patients who received more nusinersen treatment and had a higher CHOP-INTEND score. Conclusions. Despite new-generation treatments for SMA type 1, morbidity and mortality rates remain very high. As the survival rate in SMA type 1 increases, the incidence of complications similar to those frequently seen in SMA type 2 and type 3 patients also increases. The follow-up and treatment of patients with SMA should be undertaken by a multidisciplinary team.

Objectives Status epilepticus (SE) is associated with significant morbidity and mortality in children. SE in the pediatric intensive care unit (PICU) are not well characterized. The aim of this study is to retrospectively investigate the clinical features and treatment of seizures in children admitted to the PICU of our hospital. Methods We retrospectively examined the clinical characteristics of patients aged between 1 month and 18 years who were admitted to our hospital with SE or who were diagnosed with SE after hospitalization and were followed up with continuous electroencephalographic monitoring between January 2015 and December 2019 Results A total of 88 patients with SE, 50 (56.8%) boys and 38 (43.2%) girls, were included. The median age was 24 months (interquartile range, 12–80 months). When we evaluate the continuous electroencephalographic monitoring data, 27 (30.7%) were lateralized, 20 (22.7%) were multifocal, 30 (34.1%) were generalized, and 11 (12.5%) were bilateral independent epileptic activity. Seventy nine patients (89.8%) were evaluated as convulsive status epilepticus (CSE) and 9 (10.2%) as nonconvulsive status epilepticus (NCSE). Pediatric Risk of Mortality (PRISM III) score and mortality of patients with NCSE were higher (P = 0.004 and P = 0.046, respectively). Thirteen eight patients (43.1%) were diagnosed as SE, 38 patients (43.1%) as refractory SE, and 12 patients (13.6%) as super-refractory SE. The overall mortality rate was 10.2%. Conclusions Status epilepticus is a neurological emergency that causes mortality and morbidity. Electroencephalographic monitoring is important for the recognition of seizures and rapid intervention. No superiority of second-line treatments or combined treatments was demonstrated in patients with SE.

Purpose Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent episodes of fever and serositis, caused by mutations in the MEFV gene. Inflammatory pathways associated with FMF are linked to increased proinflammatory cytokines, which may be related to primary headaches, including migraine. The aim of this study was to evaluate the frequency of migraine and other primary headaches in FMF patients. Methods In this retrospective study, the medical records of FMF patients were analyzed. Demographic data, MEFV gene mutations, and headache histories were collected. The frequency of migraine was compared among patients with these mutations, and statistical analyses were conducted. Results The study included 148 FMF patients, comprising 56.1% females and 43.9% males, with a mean age of 11.3 ± 3.7 years. A family history of FMF was reported in 77.7% of patients, and 35.8% had a family history of migraine. Headaches were reported in 52.7% of patients: 24.3% non-specific, 15.5% tension-type, and 12.8% migraine. Of those with migraine, 8.1% had migraine with aura, and 4.7% without aura. Headaches were more frequently frontal in patients under 12 years of age and temporal in those aged ≥12 years (p = 0.011). The most common genetic mutations were M694V heterozygous and homozygous, with M694V and E148Q mutations linked to more frequent migraines, although not statistically significant. Conclusion FMF patients should be screened for primary headaches, particularly migraine. The high frequency of migraine observed in this study suggests that clinicians should particularly consider migraine as a diagnosis in headache episodes experienced by FMF patients.