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Öğe Abnormal Findings in Neonatal Electroencephalography(Turkiye Klinikleri, 2019) Simsek, Erdem; Tekgul, HasanThe electroencephalography (EEG) of the neonatal period differs significantly from the EEG of the older child and adult. The electrical activity also varies depending on the conceptual age reflecting the maturation of the brain. This variability presents various evaluation challenges for neurologists. in this article, pathological findings (ground rhythm abnormalities and paroxysmal abnormalities) of EEG in newborn period are described.Öğe Acquired Demyelinating Syndrome: Single Center Experience(Dr Behcet Uz Cocuk Hastaliklari Ve Cerrahisi, 2019) Serin, Hepsen Mine; Simsek, Erdem; Kanmaz, Seda; Yilmaz, Sanem; Aktan, Gul; Tekgul, Hasan; Gokben, SarenurObjective: 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 followed up with the diagnosis of demyelinating disease other than multiple sclerosis and also our therapeutic experience. Method: 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, admission complaints, clinical and neuroimaging findings, laboratory results, ophthalmologic findings and treatments used were recorded. Results: The age of the patients ranged between 3, and 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, plegia (mono+paraplegia), blurred vision and visual loss in order of decreasing frequency. Oligoclonal banding, and anti-aquaporin 4 antibody negativities were detected in all patients. Anti-myelin oligodendrocyte antibody-positivity was found in seven patients. In one of these patients tests were 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 started on intravenous pulse methylprednisolone therapy during the acute episode 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 of patients, and differential diagnosis.Öğe Assessment of Prognostic Factors and Validity of Scoring Models in Childhood Autoimmune Encephalitis(AVES, 2023) Kanmaz, Seda; Yilmaz, Sanem; Toprak, Dilara Ece; Atas, Yavuz; Ince, Tugce; Simsek, Erdem; Dokurel, IpekObjective: The aim of this study is to evaluate the prognostic factors in a single-center pediatric cohort with autoimmune encephalitis. Materials and Methods: The study group consisted of 23 pediatric autoimmune encephalitis patients (seropositive autoimmune encephalitis: 15, seronegative autoimmune encephalitis: 8). Five group prognostic parameters were evaluated: clinical manifestations, electroenc ephalography features, magnetic resonance imaging characteristics, biomarkers, and treatment modalities. Three scoring models were applied: the Antibody Prevalence in Epilepsy and Response to Immunotherapy in Epilepsy for predicting autoimmune-related epilepsy in the whole cohort and the anti-N-methyl-d-aspartate receptor Encephalitis 1-Year Functional Status score for overall outcome in patients with anti-N-methyl-d-aspartate receptor encephalitis. Results: The initial clinical spectrum of the disease was similar in the seronegative and seropositive groups. Almost half of the patients (48%) recovered without any complications with first-line immunotherapy. The patients with movement disorders in the acute phase of the disease needed more likely second-line immunotherapy (P =.039). The presence of status epilepticus at admission was significantly associated with adverse outcomes and the development of autoimmune-related epilepsy (P =.019). Autoimmune-related epilepsy was defined in an equal proportion of patients (91.5%) with 2 immune epilepsy scores (Antibody Prevalence in Epilepsy and Response to Immunotherapy in Epilepsy). The N-methyl-d-aspartate receptor Encephalitis 1-Year Functional Status score and the modified Rankin score assessed for the first-year prognosis were strongly correlated among the patients with anti-N-methyl-d-aspartate receptor encephalitis (P =.03, Spearmen's rho = 0.751). Conclusions: The presence of status epilepticus was the most important prognostic factor in the patients with the adverse outcome. The studied scoring models (Anti-N-methyl-d-aspartate receptor Encephalitis 1-Year Functional Status, Antibody Prevalence in Epilepsy, and Response to Immunotherapy in Epilepsy) have also been proven to be applicable to the pediatric age group for predicting overall outcome and autoimmune-related epilepsy.Öğe Cerebral folate transporter deficiency: a potentially treatable neurometabolic disorder(Springer Heidelberg, 2021) Kanmaz, Seda; Simsek, Erdem; Yilmaz, Sanem; Durmaz, Asude; Serin, Hepsen Mine; Gokben, SarenurCerebral folate deficiency (CFD) syndrome is a rare treatable neurometabolic disorder with low levels of the active form of folaten in cerebrospinal fluid (CSF) arising from different causes such as FOLR1 gene mutations or autoantibodies against the folate receptor-alpha (FR) protein that can block folate transport across the choroid plexus. It is characterized by late infantile onset refractory seizures, ataxia, movement disorder, and unexplained global developmental delay. Here, we report a patient diagnosed with autistic spectrum disorder, followed by refractory myoclonic-atonic seizures, ataxia, and loss of motor skills over time. A homozygous missense (c.665A > G) mutation in FOLR1 gene and extremely low CSF 5-methyltetrahydrofolate level led to the diagnosis of CFD. Although she was initiated on combined oral and intravenous high doses of folinic acid treatment at 6 years of age, mild improvement was achieved in terms of epileptic seizures and motor skills. It is important that CFD should be kept in mind in cases with refractory myoclonic-atonic seizure and folinic acid treatment should be started as soon as possible.Öğe Cerebral sinovenous thrombosis in children: A single-center experience(Aves, 2021) Cetin, Ipek Dokurel; Eraslan, Cenk; Simsek, Erdem; Kanmaz, Seda; Serin, Hepsen Mine; Karapinar, Deniz Yilmaz; Yilmaz, Sanem KeskinObjective: The study aimed to evaluate the patients with a diagnosis of cerebral sinovenous thrombosis in terms of clinical findings, etiology and underlying risk factors, imaging findings, treatment, and prognosis in the long term. Materials and Methods: Medical records of 19 patients whose ages ranged between 0 days and 17 years with clinical and radiological cerebral sinovenous thrombosis in Ege University Department of Child Neurology were retrospectively evaluated. Results: Nine of nineteen cases were female (47.3%). The median age was 84 months (0-201 months). The most common complaint at the presentation was headache (n=12) and the most common physical examination finding was papilledema (n=11). In etiology, otitis/mastoiditis in three cases, iron deficiency anemia in three cases, sinusitis in two cases, catheter use in four cases, Behcet's disease in three cases were determined. The most common observed genetic factors causing thrombosis was methylenetetrahydrofolate reductase mutation. The transverse sinus (68.4%) is the sinus where thrombosis is most frequently observed. As a result of an average follow-up of 12 months (2-72 months), hemiparesis (n=3/19, 15.7%) and epilepsy (n=5/19, 26.3%) were recorded as sequelae findings, and no mortality was observed. Conclusion: In cases presenting with headache, evaluation of papilledema on funduscopic examination should not be skipped. Neurological imaging should be performed in the change of consciousness of poor feeding infants and children with infections in the head and neck area or underlying chronic diseases. When cerebral sinovenous thrombosis is detected, anticoagulant therapy should be started immediately.Öğe Clinical spectrum, treatment and outcome of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease in children: a tertiary care experience(Springer Heidelberg, 2021) Serin, Hepsen Mine; Yilmaz, Sanem; Simsek, Erdem; Kanmaz, Seda; Eraslan, Cenk; Aktan, Gul; Gokben, SarenurAnti-myelin oligodendrocyte glycoprotein antibodies have been associated with a wide range of clinical presentations including monophasic and relapsing disease courses. Lack of a definitive marker for predicting further relapses and the final diagnoses complicates the clinical follow-up and treatment decisions for patients with the first episode. This study retrospectively analyzed the clinical spectrum, treatment protocols and outcome of nine children with MOG antibody-associated demyelinating disease. Diagnoses at first presentation were acute disseminated encephalomyelitis (ADEM) in six cases (67%), optic neuritis in two cases (22%), and clinically isolated syndrome in one case (11%). The disease remained monophasic in five (56%) cases. All cases with a monophasic disease course were negative for anti-MOG antibody titers in the third month. The initial diagnosis of all relapsing cases was ADEM. Three of the four cases with a relapsing disease course were available for anti-MOG antibody testing at the third month and all were positive, however, antibody titers at the sixth month were inconsistent. Cases with a relapsing disease course had no further attacks after monthly intravenous immunoglobulin treatment. Relapsing disease course is not rare in childhood MOG-antibody associated demyelinating disease. Monthly IVIG treatment may be a good alternative for the long-term treatment of relapsing cases with a low side effect profile. Anti-MOG antibody serostatus at remission periods should be interpreted cautiously. Further studies are needed to better understand and predict the clinical course of pediatric patients with MOG-antibody associated diseases.Öğe Continuous EEG Monitoring in Critically Ill Children and Prognostic Factors for Short-term Outcome: An Observational Study(Galenos Publ House, 2022) Balci, Ozlem Ozdemir; Simsek, Erdem; Ozkaya, Pinar Yazici; Kanmaz, Seda; Dokurel, Ipek; Serin, Hepsen Mine; Yilmaz, SanemAim: 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.Öğe CSF levels of a set of neurotrophic factors (brain-derived neurotrophic factor, nerve growth factor) and neuropeptides (neuropeptide Y, galanin) in epileptic children(Elsevier Sci Ltd, 2020) Tekgul, Hasan; Serin, Hepsen Mine; Simsek, Erdem; Kanmaz, Seda; Gazeteci, Hande; Azarsiz, Elif; Gokben, SarenurThis paper aims to investigate the possible roles of a set of neurotrophic factors (brain-derived neurotrophic factor-BDNF, nerve growth factor-NGF) and neuropeptides (neuropeptide Y-NPY, and galanin) in children with active epileptogenesis. the cerebrospinal fluid (CSF) levels of BDNF, NPY, NGF and galanin were measured with enzyme-linked immunosorbent assays in epileptic children (n = 73) and controls (n = 64). There were no significant alterations in the CSF levels of BDNF, NPY and NGF in epileptic children with active clinical seizures compared with the levels of controls. However profoundly depressed galanin levels were found in infants with epileptic encephalopathy (mean +/- SD:0.63 +/- 0.19 pg/ml) and significantly increased galanin levels were measured in children with drug resistant epilepsy during the period of status epilepticus (mean +/- SD: 6.92 +/- 1.19, pg/ml pg/ml) compared with the levels of controls. Depressed levels of galanin might reflect a defective anti-epileptogenic effect of galanin in infants with epileptic encephalopathy. on the contrary, increased CSF levels of galanin might be a result of antiepileptogenic effects of this peptide in epileptic children with status epilepticus. (C) 2020 Elsevier Ltd. All rights reserved.Öğe Efficacy of levetiracetam as first-line therapy for neonatal clinical seizures and neurodevelopmental outcome at 12 months of age(Springer Heidelberg, 2020) Kanmaz, Seda; Koroglu, Ozge Altun; Terek, Demet; Serin, Hepsen Mine; Simsek, Erdem; Cetin, Ipek Dokurel; Tekgul, Hasan L.Appropriate treatment of neonatal seizures with an effective therapy is important in reducing long-term neurologic disabilities. Sixty-seven neonates, who received intravenous (IV) levetiracetam (LEV) as first-line therapy for treating seizures between 2013 and 2017 were evaluated retrospectively to investigate the efficacy of LEV and its neurodevelopmental outcome at 12 months of age. of the 67 neonates (44 preterm and 23 term babies) evaluated for seizures, 55 (82%) had a defined etiology. EEG confirmation was obtained in 36 (57.1%) of the neonates with clinical seizures. on the 7th day of the treatment (mean seizure control time 7.4 +/- 15.1 days), LEV was effective as monotherapy in 43 (64%), whereas add-on therapy was required in 24 (36%) neonates. At the 1-year follow-up, 76% of infants achieved drug-free state, nine (18%) infants remained on LEV monotherapy and three (6%) needed add-on therapy. Neurodevelopmental outcome of the infants was assessed with Ankara Development Screening Inventory and results suggested favorable neurodevelopmental outcome in 69.7% of the infants with at the end of the 1-year follow-up with LEV monotherapy. in conclusion, this retrospective cross-sectional study demonstrated that IV LEV is an effective first-line therapy for treating neonatal clinical seizures and LEV monotherapy effect was sustained during the first year follow-up.Öğe Melatonin Versus Chloral Hydrate for Sleep Electroencephalography Recording in Children: A Comparative Study Using Bispectral Index Monitoring Scores and Electroencephalographic Sleep Stages(Lippincott Williams & Wilkins, 2022) Yilmaz, Sanem; Simsek, Erdem; Tekin, Hande Gazeteci; Aktan, Gul; Gokben, Sarenur; Tekgul, HasanPurpose: To compare the effects of chloral hydrate and melatonin on sleep EEG recordings in children by using standard EEG sleep stages and the bispectral index scores (BIS). Methods: A total of 86 children were randomly assigned to two groups: (1) melatonin group (n = 43) and (2) chloral hydrate group (n = 43). BIS monitoring scores and sleep EEGs were recorded simultaneously. The effect of two drugs on sleep EEG recording was evaluated with sleep stages of EEG and BIS. Results: There was no statistically significant difference between the groups with regard to time to sleep onset and the need for a second drug (P = 0.432; P = 1.000). Eight patients (18.6%) in chloral hydrate group reported side effects while there were no reported side effects in the melatonin group (P = 0.006). Mean BIS values during EEG recordings were similar in both groups (59.72 +/- 18.69 minutes and 66.17 +/- 18.44 minutes, respectively, P = 1.000). The average time to achieve N2 sleep was 32.38 minutes in the chloral hydrate group and 43.25 minutes in the melatonin group (P < 0.001). Both time spent in wakefulness and N1 sleep were found to be significantly higher in the melatonin group (P < 0.001 and P = 0.005). BIS scores higher than 75 were found to be suggestive for wakefulness; 75 to 66 for N1, 65 to 46 for N2, and values lower than 46 were found to be indicative for N3 sleep with a good strength of agreement in weighted Kappa analysis (95% confidence interval; weighted Kappa = 0.67). Conclusions: Melatonin is reliable and at least as effective as chloral hydrate for sleep EEG acquisition in children.Öğe Neuropsychological outcome in cases with acute disseminated encephalomyelitis(Turkish J Pediatrics, 2020) Kanmaz, Seda; Kose, Sezen; Eraslan, Cenk; Simsek, Erdem; Serin, Hepsen Mine; Yilmaz, Sanem; Gokben, SarenurBackground and objectives. Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory and demyelinating disorder of the central nervous system. There have been a few studies in recent years on the fact that these cases have neurocognitive impairment. the purpose of this study is to evaluate the neurocognitive outcome and quality of life in cases with ADEM. Methods. Eleven cases who were on follow-up between 2008 and 2017 were included in the study, systemic, neurological and psychiatric examinations were done. All magnetic resonance images were re-evaluated. the neuropsychiatric evaluation was performed by clinical examination and psychometric scales; (1) the Pediatric Quality of Life Inventory 4.0, (2) Child Behavior Checklist, (3) Children's Depression Inventory, (4) the Wechsler Intelligence Scale for Children-Revised and (5) Continuous Performance Test. the cases in our study underwent neuropsychiatric evaluation 3-42 months after the diagnosis of ADEM had been established. Results. Nine cases (81.8%) fully recovered without neurologic deficit. One case (9.1%) had a psychiatric disorder. During follow-up, cognitive and psychiatric problems were encountered in half of the cases (54.5%). Most of the cases with basal ganglia involvement (80%) displayed attention deficit and cognitive problems. Conclusion. in particular, cases with basal ganglia involvement should be followed carefully in terms of attention and cognitive problems.Öğe The potential effects of anticonvulsant drugs on neuropeptides and neurotrophins in pentylenetetrazol kindled seizures in the rat(Taylor & Francis Ltd, 2020) Tekgul, Hasan; Simsek, Erdem; Erdogan, Mumin Alper; Yigitturk, Gurkan; Erbas, Oytun; Taskiran, DilekPurpose: Neuropeptides and neurotrophic factors are thought to be involved in epileptogenesis. This study aims to investigate the potential effects of anticonvulsant drugs on neuropeptides (galanin and neuropeptide Y) and neurotrophic factors (BDNF and NGF) in pentylenetetrazol (PTZ)-kindled seizures in the rat. Methods: Forty-eight adult male Sprague?Dawley rats were included in the study. the animals were divided into 8 groups of six rats. Group 1 was defined as na?ve control, and received no medication. Group 2 (PTZ?+?saline) was treated with sub-convulsive doses of PTZ (35?mg/kg) and saline i.p. for 14?days. For anticonvulsant treatments, Groups 3?8 were treated with 200?mg/kg levetiracetam (PTZ?+?LEV), 1?mg/kg midazolam (PTZ?+?MDZ), 80?mg/kg phenytoin (PTZ?+?PHT), 80?mg/kg topiramate (PTZ?+?TPR), 40?mg/kg lamotrigine (PTZ?+?LMT) and 50?mg/kg sodium valproate (PTZ?+?SV), respectively. All anticonvulsant drugs were injected 30 min prior to PTZ injection throughout 14?days. Following treatment period, behavioral, biochemical and immunohistochemical studies were performed. Results: PTZ?+?saline group revealed significantly decreased galanin, NPY, BDNF and NGF levels compared to control. PTZ?+?MDZ group had significantly increased galanin, BDNF and NGF levels compared to saline group. Also, PTZ?+?LEV group showed increased BDNF levels. PTZ?+?saline group revealed significantly lower neuron count and higher GFAP (+) cells in hippocampal CA1?CA3 regions. All anticonvulsants significantly reduced hippocampal astrogliosis whereas only midazolam, levetiracetam, sodium valproate and lamotrigine prevented neuronal loss. Conclusion: Our results suggested that anticonvulsant drugs may reduce the severity of seizures, and exert neuroprotective effects by altering the expression of neuropeptides and neurotrophins in the epileptogenic hippocampus.Öğe A rare case of peripheral nerve hyperexcitability in childhood: Isaacs syndrome(Wolters Kluwer Medknow Publications, 2020) Kanmaz, Seda; Ozcan, Muhittin; Simsek, Erdem; Serin, Hepsen M.; Aydogdu, Ibrahim; Gokben, Sarenur; Tekgul, HasanIsaacs syndrome is rare disorder with peripheral nerve hyperexcitability syndromes with acquired neuromyotonia in childhood. We present a 13-year-old girl with muscle stiffness and neuromyotonia diagnosed Isaac syndrome with spontaneous discharge potentials on motor unit in electromyography and the diagnosis supported by the presence of antinuclear antibodies. A successful treatment was obtained using low-dose carbamazepine. Cause of Isaacs syndrome is unknown, generally thought to be an autoimmune etiology with voltage-gated potassium channelopathy; it sometimes occurs as a paraneoplastic syndrome. Early use of electromyography has critical role in the differential diagnosis with certain muscle disorders and peripheral nerve hyperexcitability syndromes.Öğe A Rare Cause of Diplopia: Idiopathic Orbital Myositis(Galenos Yayincilik, 2019) Gokcel, Sule; Simsek, Erdem; Yilmaz, Sanem Keskin; Aydogdu, SemaOrbital myositis is an entity affecting the ocular muscles, especially the medial rectus. These cases are usually referred to clinics with complaints such as diplopia, orbital/periorbital pain, limitation in ocular movements, increased pain with eye movements, proptosis, swelling of the eyelid and/or hyperemia in the conjunctiva. Orbital myositis is usually idiopathic and autoimmunity is often suspected in etiology. In this article, we present a 15-year-old girl who presented with diplopia, pain in both eyes, anomalous head posture, periorbital edema and was diagnosed with idiopathic orbital myositis through history, clinical findings and imaging methods.Öğe A rare cause of ischemic stroke in childhood: spontaneous long segment intracranial dissection(Springer, 2020) Simsek, Erdem; Yilmaz, Sanem; Oran, Ismail; Aktan, Gul; Tekgul, Hasan; Gokben, SarenurCraniocervical arterial dissection is an important cause of arterial ischemic stroke in children. Recognition of dissections is of particular importance both in determining the risk of recurrence and in bringing about different treatment alternatives. We report a 10-year-old girl who presented with acute ischemic stroke due to spontaneous long segment dissection involving the parasellar internal carotid artery up to the distal M1 portion of the middle cerebral artery. Three-dimensional digital subtraction angiography with flat panel detector revealed the presence of major vessels originating from both true and false lumens and had a critical role in the treatment decision of the case.Öğe A retrospective study with ICAST-R (ispcan child abuse screening tools-retrospective) questionnaire for determination of child abuse in first year medical students in Turkish population(Pergamon-Elsevier Science Ltd, 2017) Simsek, Erdem; Guney, Sevay Alsen; Baysal, Serpil UgurChild Abuse and Neglect (CAN) is an important problem both in Turkey and worldwide. Unfortunately, we still don't have enough and reliable data on this important subject. In this study, we aimed to investigate the prevalence of childhood maltreatment in a population of first year medical students in a Turkish university using the retrospective version of the ISPCAN Child Abuse Screening Tool (ICAST-R). One hundred seventy three first year medical students, aged between 18 and 24 years, who agreed to complete the ICAST-R questionnaire, were recruited in our study. Of 173 students who completed the survey, 51.4% are found to be subjected to at least one type of child abuse (physical, emotional or sexual). The prevalence is higher in male gender and the difference is statistically significant. Physical, emotional and sexual abuse exposure rates are found to be 23.1%, 40.5% and 11% respectively. Physical and emotional abuse rates are higher in male gender and the difference is statistically significant. We did not find any statistically significant association between gender and sexual abuse. Efforts to prevent child abuse and neglect are growing in our country, as they are worldwide. However, child abuse is still common and a problematic issue. It is important to determine the prevalence of child abuse in order to raise awareness. We need further studies investigating not only prevalence but also risk factors to have reliable data for our country.Öğe Sulthiame add-on treatment in children with epileptic encephalopathy with status epilepticus: an efficacy analysis in etiologic subgroups(Springer-Verlag Italia Srl, 2020) Kanmaz, Seda; Simsek, Erdem; Serin, Hepsen Mine; Yilmaz, Sanem; Aktan, Gul; Tekgul, Hasan; Gokben, SarenurPurpose Sulthiame (STM) has been recommended as an effective antiepileptic drug (AED) in children with epileptic encephalopathy with status epilepticus in sleep (ESES). the aim of this study is to evaluate the efficacy of STM add-on treatment in children with pattern of ESES with respect to the etiologic subgroup. Methods Twenty-nine children with ESES pattern with three different etiologic subgroups (epileptic syndromes: 14, structural/infectious: 9, unknown: 6) who were given STM as add-on treatment were included into the study. the efficacy of STM was evaluated in terms of seizure control, electroencephalography (EEG) findings, need of the new AEDs after add-on STM, and behavioral and cognitive improvement. Results the range of the follow-up duration after add-on STM treatment was between 5 and 51 months. At the end of 1 year of STM treatment, the most successful electrophysiologic improvement was identified in the well-defined epileptic syndrome group; epileptic syndrome, 71.4% (10/14); structural/infectious, 33.3% (3/9); and unknown, 0% (0/6). Patients who had complete response or persistent ESES pattern at the 3rd month were still in the same condition at the 6th and 12th months. However, the ESES pattern reappeared in 35.2% of the patients who had partial electrophysiological improvement at the 3rd month. in the epilepsy syndrome group, eight out of ten patients who had either complete or partial EEG response after 1 year of STM treatment displayed behavioral and cognitive improvement. Conclusion Sulthiame might be a valid add-on treatment of ESES especially in children with epilepsy syndromes.Öğe What are the predominant predictors of seizure relapse following discontinuation of anti-seizure medication in epileptic children?(Wiley, 2023) Kanmaz, Seda; Toprak, Dilara Ece; Olculu, Cemile Busra; Dokurel, Ipek; Simsek, Erdem; Serin, Hepsen Mine; Yilmaz, SanemObjective: The aim of the study was to identify the predominant predictors of seizure relapse following discontinuation of ASM in epileptic children. Methods: The study cohort consisted of 403 epileptic children who had a with-drawal process of ASM (monotherapy: 344; dual therapy or polytherapy: 59) after at least a 2- year seizure- free period. Patients were categorized if they had a well-defined epileptic syndrome. Epileptic children with ongoing ketogenic diet, vagal nerve stimulation, or surgery were excluded from the cohort due to the additional withdrawal process related to other therapy modalities. Results: The cohort's seizure relapse rate was 12.7% (51/403). The highest rates of seizure relapse were defined for genetic etiology at 25% and structural etiology at 14.9%. An epilepsy syndrome was defined in 183 of 403 children (45.4%). There was no difference in the seizure relapse rate between the subgroups of well-defined epileptic syndromes; 13.8% for self-limited focal epileptic syndromes, 11.7% for developmental and epileptic encephalopathies, and 7.1% for generalized epileptic syndromes. Five predictors were defined as the most powerful predictors of seizure relapse in univariate analysis: age at epilepsy diagnosis >2 years (hazard ratio [HR]: 1.480; 95% confidence interval [CI]: 1.134- 1.933), defined etiology (HR: 1.304; 95% CI: 1.003- 1.696), focal seizure (HR: 1.499; 95% CI: 1.209- 1.859), =3 months duration of the withdrawal process (HR: 1.654; 95% CI: 1.322- 2.070), and a history of neona-tal encephalopathy with or without seizures (HR: 3.140; 95% CI: 2.393- 4.122). In multivariate analysis, the main predictor of seizure relapse was a history of neonatal encephalopathy with or without seizures (HR: 2.823; 95% CI: 2.067- 3.854). Significance: The duration of seizure freedom before discontinuation of ASM was not a predominant risk factor for seizure relapse: 2- 3 years versus >3 years. The predictive values of five predictors of seizure relapse rate should be evaluated for patients with different epilepsy subgroups.Öğe WWOX-associated encephalopathies: identification of the phenotypic spectrum and the resulting genotype-phenotype correlation(Springer-Verlag Italia Srl, 2018) Serin, Hepsen Mine; Simsek, Erdem; Isik, Esra; Gokben, SarenurEpileptic encephalopathies are a group of disorders in which epileptiform abnormalities cause progressive deterioration in cerebral function. Genetic causes have been described in several of the epileptic encephalopathies, and many previously unknown genes have been identified. WW domain-containing oxidoreductase (WWOX) has recently been implicated in autosomal recessive spinocerebellar ataxia type 12 (SCAR12) and severe early-onset epileptic encephalopathy. With whole-exome sequencing, we identified a homozygous WWOX missense mutation, p.Leu239Arg, in a girl from a consanguineous family with psychomotor developmental delay, acquired microcephaly, and epileptic seizures. WWOX-related epileptic encephalopathy is a rare condition but it should be considered in cases having early epileptic spasms and parental consanguinity.
