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Aim:To analyze trends in the prophylaxis of febrile convulsions (FC) in childhood by comparing two cohorts from the previous two decades (2007-2008 versus 2017-2018).Materials and Methods:The cohort consisted of 272 children with FC who were followed up during the 2007-2008 (n=105) and 2017-2018 (n=167) periods in Ege University Faculty of Medicine Children’s Hospital. The following clinical parameters were analyzed: demographic data, FC types, prophylaxis types, selected anti-seizure medications (ASM), recurrence risk factors, and electroencephalography (EEG) characteristics.Results:We defined two secular trends for the prophylaxis of FC in children in the last decade: (1) a reduced rate of FC prophylaxis (22.1%) in the period of 2017-2018 compared with a rate of 63.8% in 2007-2008, p

Aim:To evaluate the association of etiology, continuous electroencephalography (cEEG) findings and neuroimaging findings with short-term outcomes for patients admitted to a pediatric intensive care unit (PICU) for acute encephalopathy.Materials and Methods:A total of 24 children admitted to a PICU for acute encephalopathy were enrolled into this study. The etiology, treatment, duration of stay in the PICU, their demographic information and their past medical history were recorded. cEEG was initiated as quickly as possible following admission to the PICU and continued for at least 24 hours. Their short-term prognosis was evaluated by the Pediatric Cerebral Performance Category score (PCPC) at PICU discharge.Results:The most common cause was traumatic brain injury comprising 25% (n=6) of all cases. Other common causes were asphyxia (hanging, foreign body aspiration, drowning) (n=4, 16.67%) and intoxication (n=3, 12.5%). Twenty-two patients underwent cranial imaging. The most common findings in CT were hemorrhage (n=6, 30%) and ischemia/edema (n=6, 30%). Fourteen patients had unfavorable PCPC outcome scores. There was a tendency for poorer outcomes in those patients with hemorrhage/fracture or ischemia/edema in the imaging and for those patients who needed either pre-hospital CPR or had non-convulsive seizures but without statistical significance.Conclusion:cEEG in critically ill children is useful for detecting both epileptic and non-epileptic events. The use of cEEG in PICUs can be helpful for the better management of cases.

Transient lesions involving the splenium of corpus callosum is defined as reversible splenial lesion syndrome (RESLES). Benign convulsions with mild gastroenteritis is a rare condition which may be associated with RESLES. Since the prognosis is excellent, the awareness of this association is important to prevent unnecessary investigations and anti-epileptic drug therapy.

Aim:A brief resolved unexplained event (BRUE) is characterized by sudden alterations in an infant’s breathing, color, tone, or responsiveness. The aim of this study was to evaluate the necessity of electroencephalography (EEG) in high-risk BRUE cases.Materials and Methods:Fifty-one patients’ cases were analyzed retrospectively. All of these patients were younger than 1 month so considered to be a high-risk group. The gestational week, the age of the patient, the duration of the event, the conditions related to the event, whether or not any intervention was needed, the type of intervention if done, if the event recurred, the number of recurrences, the state of consciousness during the event, respiratory pattern, muscle tone, sleeping position, suspicion of trauma, use of medication by mother and/or baby and smoking exposure were evaluated in detail.Results:The mean age of the patients was 15.74±14.96 days, 31 (60.78%) were male and 20 (39.21%) were female. The mean gestational age was 37.64±2.35 weeks. The neurological examinations of the patients were evaluated as normal. EEG was performed in 36 (70.58%) of the 51 patients and only one patient had sharp waves in the left hemisphere central region. In 11 patients hospitalized with a preliminary diagnosis of BRUE, final diagnoses were found as congenital pyloric stenosis, dehydration, fetal myocarditis, patent ductus arteriosus, lower respiratory tract infection and gastroesophageal reflux.Conclusion:It would be more appropriate to plan the tests to be carried out in the high-risk BRUE group by evaluating many factors such as recurrence of the event, family history, and neurological examination findings. As a result, even in the high-risk BRUE group, it would be cost effective to perform an EEG if only the clinical cues are strongly suggestive for the diagnosis of epilepsy.

Congenital myasthenic syndromes (CMS) are neuromuscular hereditary diseases with the symptoms of fatigue, weakness, ptosis, ophthalmoparesis and respiratory problems. This disease group is classified as CMS originating from the presynaptic region, synaptic gap and postsynaptic region according to the origin of the neuromuscular junction. Most of these patients are affected by receptor defects originating from the postsynaptic gap. Here, we present a case who was thirteen years old and had a CHRNE genotype p.Y124 *(c.372C> G) homozygous mutation, which is associated with weakness, low voice, ophthalmoparesis and frequent respiratory infection since birth. Our patient has been diagnosed with non-kinetic AChR deficiency and the case is important with the detection of a new mutation.

INTRODUCTION: Acquired demyelinating syndromes are immune-mediated demyelinating disorders of the central nervous system. Clinical spectrum includes acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, clinically isolated syndrome, and neuromyelitis optica. Aim of this study is to present the clinical features of patients demyelinating disease other than multiple sclerosis and our therapeutic experience. METHODS: Data of 30 patients with acquired demyelinating disease followed up in Ege University Department of Child Neurology between January 2013 and January 2018 were evaluated retrospectively. Demographic data, complaints, clinical and neuroimaging findings, laboratory results, ophthalmologic findings and treatment were recorded. RESULTS: The age of the patients ranged between 3-15 years and mean age was 8.76 +- 3.59 years. The most common diagnosis was acute disseminated encephalomyelitis. The most common complaints were ataxia, headache, encephalopathy, pleome (mono + paraplegia), blurred vision and visual loss respectively. Tests were negative for oligoclonal banding in all patients. Anti-myelin oligodendrocyte antibody was positive in seven patients. One of these patients was double positive for both anti-aquaporin 4 and anti-myelin oligodendrocyte antibodies. Neuroimaging revealed findings consistent with cerebral white matter, spinal cord and optic nerve involvement. All patients were given intravenous pulse methylprednisolone therapy for the acute period DISCUSSION AND CONCLUSION: Since childhood acquired demyelinating diseases may be the first episode of multiple sclerosis, it is important to perform detailed serological tests and CSF examinations for follow-up and differential diagnosis.

Aim: The aim of this study is to determine the risk factors of epilepsy development in children with cerebral palsy. Materials and Methods: Data of 234 cerebral palsy patients treated at Ege University Pediatrics Department, Child Neurology Division between January 2008 and December 2015 were evaluated retrospectively. All patients were classified into two groups; Group I: cerebral palsy without epilepsy (n=116) and Group II: cerebral palsy with epilepsy (n=118). The clinical and laboratory findings of the groups were compared to each other, a p value of less than 0.05 was considered as statistically significant. Results: There was no significant difference between the two groups in terms of gender, gestastional age, birth type, birth weight, risk factors for cerebral palsy development (pre-/peri-/postnatal), duration of neonatal intensive care stay and the need for mechanical ventilation (p>0.05). The risk factors of epilepsy were determined as the following; the presence of neonatal convulsions, focal clonic and generalized tonic neonatal seizures, an abnormal baseline rhythm on neonatal electroencephalography (EEG), discharge from neonatal intensive care unit with at least one antiepileptic drug, spastic bilateral (tetraplegic) cerebral palsy, epileptic activity on the sixth month EEG, abnormal cranial magnetic resonance imaging findings, mental retardation, microcephaly and visual problems. Conclusion: Epilepsy is a common problem in children with cerebral palsy. Therefore, cases of cerebral palsy, especially those with the determined risk factors should be closely monitored for epilepsy in order to ensure a timely diagnosis and proper treatment.

Hereditary neuropathy with a liability to pressure palsies (HNPP) represented by recurrent focal pressure neuropathies is rare in childhood. Here we present a 10-year-old girl admitted to our hospital with a recurrent weakness in her foot and diagnosed as HNPP with a quick Multiplex Ligation-dependent Probe Amplification test revealing PMP22 deletion.

Hyperinsulinemic hypoglycemia (HH) is the most common reason for persistent and recurrent hypoglycemia in the neonatal and infancy periods. We presented a case diagnosed with HH on the first day of life and who underwent near-total pancreatectomy because of the unresponsiveness to the diazoxide treatment. Despite early diagnosis and management, complications developed due to hypoglycemia and surgery.

Aim: To evaluate the value of vagal nerve stimulation (VNS) in improving the quality of life, seizure frequency and mood in children with pharmacoresistant epilepsy. Materials and Methods: Eleven pharmacoresistant epileptic children implanted with the VNS therapy device between 2010-2014 were included in this prospective longitudinal study. Clinical assessment for the Diagnostic and Statistical Manual of Mental Disorders-IV psychiatric disorders, schedule for affective disorders, and schizophrenia for school children were applied. Childhood Depression Inventory (CDI), Beck Depression Inventory (BDI) were filled. Child Behavior Checklist (CBCL) and the Pediatric Quality of Life Inventory (PedsQL) were filled in by parents before the implantation. All of these tests were repeated in the first and second year of implantation. Results: Mean age at the first implantation of the VNS therapy device was 11.5 (2.5-16). Mean age was 13.9 (2.5-18) years. Mean duration of epilepsy prior to VNS implantation was 6.6 years, and VNS usage period was 30.4 months (10-96). International League Against Epilepsy classification of predominant seizure type was partial in seven, and generalized in four patients. Decrease in the number of seizures was 42% and 43% in the first and the second year respectively. While four of 11 patients had no psychopathology, different problems were determined in seven patients. There was no statistically significant difference between the scores of CDI/BDI, scores of CBCL and PedsQL prior to implantation, and also did not differ 1 year and 2 years after implantation. Conclusion: VNS is partially effective in controlling drug-resistant epilepsy. Although a positive effect on depression and quality of life is reported in literature, the results of this study were not statistically significant. We think that the scores of depression inventory should gradually decrease in years as it is reported for seizure frequency.

Sturge-Weber syndrome (SWS) is a neurogenetic disease with an incidence of 1 in 20.000-50.000 live births. The less common form, which can be difficult to diagnose and only involves leptomeningeal angioma, has been defined as Type III SWS. A 5.5-month-old male patient with normal neuromotor development presented with right sided partial seizures, which had been occurring frequently for the previous two days and could not be controlled. A cranial magnetic resonance imaging showed pathological contrasts in the cortical regions involving the left hemisphere and in the leptomeningeal structures. We aim to present the case of an infant with SWS, which unlike the classical form was unidentifiable in physical examination and diagnosed using imaging methods.

Today rapidly increasing obesity causes significant morbidity and mortality. Gastric by-pass is frequently used for the treatment of morbid obesity, which cannot be controlled with diet and exercise. Although this treatment modality achieves rapid success, it also has several complications such as nutritional deficiency and its clinical results. We aimed to present a patient with polyneuropathy after having undergone gastric by-pass surgery for morbid obesity.

Branchial anomalies are observed as cists, fistulas and sinuses. Branchial cleft fistulas are rare congenital anomalies that appear in the neck area. These are clinically diagnosed. Similar fistulas could be seen at every age from birth to adolescence. These kinds of anomalies are not selective for gender, and localization in the neck area. Such disorders are due to the incomplete obliteration of branchial apparatus during fetal development. As mentioned branchial cleft fistulas are not commonly observed, whereas bilateral types are even rare than unilateral types. Diagnosis and treatment of the bilateral branchial fistulas is presented in this article with the review of the literature.

Hyperekplexia is a rare, nonepileptic, genetic, or sporadic neurologic disorder characterized by startle responses to acoustic, optic, or tactile stimuli. Genetic defects in glycine receptors as well as encephalitis, tumors, inflammation, and disgenesis are among the etiologic causes of the disease. The main problem in hyperekplexia is the incomplete development of inhibitory mechanisms or exaggerated stimulation of excitatory mediators. Hyperekplexia is often confused with epileptic seizures. Here we present a case with hypoglycemic convulsions coexisting with hyperekplexia, causing diagnostic difficulty.