Birth outcomes in pregnant women with epilepsy: A Nationwide multicenter study from Türkiye.

OBJECTIVE: The present study was aimed at investigating the effects of anti-seizure medications (ASMs), patient demographic characteristics, and the seizure type and frequency on the development of congenital malformations (CMs) in the infants of pregnant women with epilepsy (PWWE). METHODS: PWWE followed up at the neurology outpatient clinic of 21 centers between 2014 and 2019 were included in this prospective study. The follow-up of PWWE was conducted using structured, general pregnant follow-up forms prepared by the Pregnancy and Epilepsy Study Committee. The newborns were examined by a neonatologist after delivery and at 1 and 3 months postpartum. RESULTS: Of the infants of 759 PWWE, 7.2% had CMs, with 5.6% having major CMs. Polytherapy, monotherapy, and no medications were received by 168 (22.1%), 548 (72.2 %), and 43 (5.7 %) patients, respectively. CMs were detected at an incidence of 2.3% in infants of PWWE who did not receive medication, 5.7% in infants of PWWE who received monotherapy, and 13.7% in infants of PWWE who received polytherapy. The risk of malformation was 2.31-fold (95% confidence interval (CI): 1.48-4.61, p < .001) higher in infants of PWWE who received polytherapy. Levetiracetam was the most frequently used seizure medication as monotherapy, with the highest incidence of CMs occurring with valproic acid (VPA) use (8.5%) and the lowest with lamotrigine use (2.1%). The incidence of CMs was 5% at a carbamazepine dose <700 mg, 10% at a carbamazepine dose ≥700 mg, 5.5% at a VPA dose <750 mg, and 14.8% at a VPA dose ≥750 mg. Thus the risk of malformation increased 2.33 times (p = .041) in infants of PWWE receiving high-dose ASMs. SIGNIFICANCE: Birth outcomes of PWWE receiving and not receiving ASMs were evaluated. The risk of CMs occurrence was higher, particularly in infants of PWWE using VPA and receiving polytherapy. The incidence of CMs was found to be lower in infants of PWWE receiving lamotrigine.

A clinical evaluation of gelastic and dacrystic seizures: a multicenter study.

BACKGROUND: Gelastic seizures are extremely rare, short-lasting, unprovoked, and uncontrollable laughing attacks. We conducted this retrospective evaluation to determine whether these symptoms, manifesting in different forms, such as cheerful laughter, laughing, smiling, and sobbing had any value in terms of etiology or localization. METHODS: A total of 31 patients who exhibited bouts of laughing or crying and who were under follow-up between 2000 and 2019 at tertiary epilepsy centers were included in the study. Laughing seizures were divided into three groups in terms of semiology (i.e., laughter with mirth, laughter without mirth, and smile). Dacrystic seizures were accompanied by some gelastic seizures and were divided into two groups in terms of semiology (i.e., weeping loudly [motor and voice-sobbing] and crying). RESULTS: Of the 27 patients with laughing seizures, 12 had seizures that manifested with smiling, 7 had seizures that manifested with laughing and mirth, and 8 had seizures that manifested with laughter without mirth. Dacrystic-gelastic seizures were observed in four patients, among whom 2 patients had crying and laughter without mirth and 2 patients had weeping loudly and laughter without mirth episodes. CONCLUSION: Gelastic and dacrystic seizures often suggest hypothalamic hamartomas, in the literature. This rare ictal behavior can originate from different cortical locations and lesions of a different nature. However, we found that gelastic seizures with smiling were a more homogenous group with regard to location in the temporal lobe, which we aimed to show by evaluating the patients included in this study.

ANTECEDENTES: Crises gelásticas são ataques de riso extremamente raros, de curta duração, não provocados e incontroláveis. Realizamos esta avaliação retrospectiva para determinar se esses sintomas, manifestando-se de diferentes formas, como riso alegre, riso, sorriso e soluço, tinham algum valor em termos de etiologia ou localização. MéTODOS: Foram incluídos no estudo 31 pacientes que apresentavam crises de riso ou choro e que estavam em acompanhamento entre 2000 e 2019 em centros terciários de epilepsia. As crises de riso foram divididas em três grupos em termos de semiologia (ou seja, riso com alegria, riso sem alegria e sorriso). As crises dacrísticas foram acompanhadas por algumas crises gelásticas e foram divididas em dois grupos em termos de semiologia (ou seja, choro alto [motor e soluçar a voz] e choro). RESULTADOS: Dos 27 pacientes com crises de riso, 12 tiveram crises que se manifestaram com sorriso, 7 tiveram crises que se manifestaram com riso e alegria e 8 tiveram crises que se manifestaram com riso sem alegria. Crises dácristico-gelásticas foram observadas em quatro pacientes, sendo 2 pacientes com choro e riso sem alegria e 2 pacientes com choro alto e riso sem alegria. CONCLUSãO: Crises gelásticas e dacrísticas frequentemente sugerem hamartomas hipotalâmicos, na literatura. Este comportamento ictal raro pode ter origem em diferentes localizações corticais e lesões de natureza diversa. No entanto, verificamos que as crises gelásticas com sorriso foram um grupo mais homogêneo quanto à localização no lobo temporal, o que buscamos evidenciar avaliando os pacientes incluídos neste estudo.

A Rare Cause of Epilepsy: Ulegyria Revisited in a Series of 10 Patients.

Introduction. Ulegyria results from perinatal hypoxic-ischemic brain injury in term infants. The specific mushroom-shaped configuration of ulegyria results from small atrophic circumvolutions at the bottom of a sulcus underlying an intact gyral apex. Clinically, ulegyria is generally associated with epilepsy. Here, we aimed to delineate the characteristics of patients with ulegyria and the epileptic seizures they experience. Material and methods. Medical records including radiology and pathology reports, video-electroencephalographic (EEG) analysis, operative notes, hospital progress and outpatient clinic notes were reviewed retrospectively in a total of 10 ulegyria patients. Results. Patients ages ranged between 24 and 58 years (mean, 32 ± 9.8 years). Past medical history was confirmed for neonatal asphyxia in 2 (20%). Neurological examination was remarkable for spastic hemiparesis in 1 (10%) patient with perisylvian ulegyria and for visual field deficits in 2 patients (20%) with occipital ulegyria. Ulegyria most commonly involved the temporoparietal region (n = 5, 50%) followed by the perisylvian area (n = 2, 20%). Except the one with bilateral perisylvian ulegyria, all patients had unilateral lesions (n = 9, 90%). Hippocampal sclerosis accompanied ulegyria in 2 patients (20%). All patients experienced epileptic seizures. Mean age at seizure onset was 8.8 ± 5.4 years (range, 2-20 years). Interictal scalp EEG and EEG-video monitoring records demonstrated temporoparietal and frontotemporal activities in 5 (50%) and 2 (20%) patients, respectively. The seizures were successfully controlled by antiepileptic medication in 8 patients (n = 8, 80%). The remaining 2 patients (%20) with concomitant hippocampal sclerosis required microsurgical resection of the seizure foci due to medically resistant seizures. Discussion. Ulegyria is easily recognized with its unique magnetic resonance imaging characteristics and clinical presentation in the majority of cases. It is highly associated with either medically resistant or medically controllable epileptic seizures. The treatment strategy depends on the age at onset and extends of the lesion that has a significant impact on the severity of the clinical picture.

The evaluation of psychogenic non-epileptic seizures (PNES) cases with saline injection method in video-EEG monitorization unit.

BACKGROUND: It has been reported that 10 to 30% of patients sent to epilepsy centers with a diagnosis of refractory epilepsy are diagnosed with psychogenic non-epileptic seizure (PNES). A wide variety of provocative methods are used to assist PNES diagnosis. OBJECTIVE: To investigate the effect of seizure induction on the diagnosis and prognosis of PNES. METHODS: We retrospectively examined 91 patients with PNES complaints in our video-EEG laboratory. Intravenous saline was administered to all patients for induction of seizures. RESULTS: Saline injection was performed in 91 patients referred to our EEG lab with PNES initial diagnosis, 57 of whom were female and 34 male. Saline injection triggered an attack in 82 patients (90%). CONCLUSIONS: In this study we have concluded that provocative methods are practical, cheap and, most of all, effective for patient diagnosis. In clinical practice, explaining the diagnosis is the first and most important step of the treatment, and careful patient-doctor communication has a positive impact on patient prognosis.

The evaluation of psychogenic non-epileptic seizures (PNES) cases with saline injection method in video-EEG monitorization unit / Avaliação de casos de crises não epilépticas psicogênicas (CNEPs) pelo método de injeção de solução salina em unidade de monitoramento por vídeo-EEG

ABSTRACT Background: It has been reported that 10 to 30% of patients sent to epilepsy centers with a diagnosis of refractory epilepsy are diagnosed with psychogenic non-epileptic seizure (PNES). A wide variety of provocative methods are used to assist PNES diagnosis. Objective: To investigate the effect of seizure induction on the diagnosis and prognosis of PNES. Methods: We retrospectively examined 91 patients with PNES complaints in our video-EEG laboratory. Intravenous saline was administered to all patients for induction of seizures. Results: Saline injection was performed in 91 patients referred to our EEG lab with PNES initial diagnosis, 57 of whom were female and 34 male. Saline injection triggered an attack in 82 patients (90%). Conclusions: In this study we have concluded that provocative methods are practical, cheap and, most of all, effective for patient diagnosis. In clinical practice, explaining the diagnosis is the first and most important step of the treatment, and careful patient-doctor communication has a positive impact on patient prognosis.

RESUMO Antecedentes: Há relatos de que 10 a 30% dos pacientes com epilepsia refratária enviados a centros de epilepsia são diagnosticados com crise não epiléptica psicogênica (CNEP). Uma ampla variedade de métodos provocativos é usada para auxiliar no diagnóstico de CNEP. Objetivo: Investigar o efeito da indução de convulsões no diagnóstico e no prognóstico de CNEP. Métodos: Examinamos 91 pacientes com queixas de CNEP em nosso laboratório de vídeo-EEG. Foi administrada solução salina intravenosa a todos os pacientes para indução de convulsões. Resultados: A injeção de solução salina foi realizada em 91 pacientes com diagnóstico inicial de CNEP encaminhados ao nosso laboratório de EEG, 57 dos quais eram mulheres e 34 homens. A injeção de solução salina desencadeou um ataque em 82 pacientes (90%). Conclusões: Neste estudo, concluímos que os métodos provocativos são práticos, baratos e, acima de tudo, eficazes para o diagnóstico de pacientes. Na prática clínica, a explicação do diagnóstico é a primeira e mais importante etapa do tratamento, e a comunicação cuidadosa entre médicos e pacientes tem um impacto positivo em seu prognóstico.

Headaches Associated with Seizure: A Prospective Comparative Cohort Study.

INTRODUCTION: The purpose of the present study is to examine the frequency of headaches based on their relationship with seizures in epileptic patients as well as types of these headaches, and their clinical characteristics. METHODS: 100 patients with epilepsy (60 female, 40 male), who applied to the epilepsy outpatient clinic of Faculty of Medicine of Uludag University, were included in the study after accepting their consent forms. Patients with symptomatic epilepsy, secondary headaches and mental retardation were excluded from the study. Patients with epilepsy were divided into two groups as the patients with or without headaches associated with seizure. In addition, according to their temporal relationships with seizures, headaches were grouped as preictal, ictal and postictal headaches and the characteristics of headaches associated with seizure were examined and the patients with and without headache associated with seizure were compared in terms of their demographic and clinical features. RESULTS: In this study, the prevalence of headache associated with seizure was found as 42%. Headaches associated with seizure were more frequent in the postictal period and they were mostly characterized as migraine-like headache. According to the seizure periods, 22 (52.3%) of the patients experienced pain during every seizure period. It was determined that preictal headache was frequently migraine-like compared to postictal headache and this headache was more frequently accompanied by aura. CONCLUSION: Headache and epilepsy are the most frequent paroxysmal neurological conditions. However, because the symptoms of epilepsy are more remarkable, and its clinical presentation has a more dramatic picture, additional neurological conditions may be overlooked. Since both epilepsy and headache symptoms decrease the quality of life, it is important to treat both conditions. Examination of the correlation between these two situations can guide the physicians for selecting the treatment type, as well as helping them to improve the quality of life.

Ictal kissing: Electroclinical features of an unusual ictal phenomenon.

PURPOSE: The study aimed to describe the electroclinical features of ictal kissing, an unusual behavior that may occur during focal seizures. METHOD: Twenty-five patients collected from four epilepsy centers and previously published reports were reviewed for their demographic, clinical, and electrophysiological features. RESULTS: Sixteen of 25 patients were female. The mean age was 32.9 years (9.9-51 years) and the average age at seizure onset was 14.5 years. All seizures were localized to the temporal lobe (TL) and lateralized to right side in 17 patients, left side in 2 patients, and unclear in 6 patients. A total of 55 ictal video electroencephalograms (EEGs) were analyzed. There were other symptoms such as speaking, spitting, hugging, and oral and upper-extremity automatisms along with different types of ictal kissing behavior during the seizures. The median duration of all seizures was 106.7±73.73s. Kissing occurred at a median time of 71s (1-95s) after the onset of seizure, and ictal epileptiform discharges usually involved TL during kissing episode. CONCLUSION: Ictal kissing is mainly associated with right TL and female gender, although association with left TL may also occurs, possibly related to the circuits involving temporo-insular structures.

Nonconvulsive status epilepticus cases arising in connection with cephalosporins.

Cephalosporins, particularly cefepime, exert neurotoxic side effects that can lead to status epilepticus. These neurotoxic side effects include myoclonus, dystonic movements, tremor, asterixis, seizure, status epilepticus, encephalopathy, and sometimes coma. Status epilepticus, particularly nonconvulsive status epilepticus (NCSE), is a well-known but unusual complication in patients with altered renal function who were receiving treatment with intravenous cephalosporins, especially cefepime. We reviewed the clinical and electroencephalographic (EEG) characteristics of 7 patients with renal failure who developed consciousness alterations with changes in EEG activity while being treated with cephalosporins. All patients developed renal failure: six patients had chronic renal failure, one patient had acute renal failure, and two patients were administered hemodialysis. Nonconvulsive status epilepticus was observed between 2 and 8 days (average of 5.6 days) after initiation of cephalosporins. Cephalosporins are epileptogenic drugs, especially when used in excessive doses or when renal function is impaired. Critically ill patients with chronic kidney disease are particularly susceptible to cefepime neurotoxicity. Clinical and electrophysiological results of patients guide the diagnosis of NCSE by healthcare providers.

Paroxysmal Amnesia Attacks due to Hashimoto's Encephalopathy.

Hashimoto's encephalopathy is a rare disease which is thought to be autoimmune and steroid responsive. The syndrome is characterized by cognitive impairment, encephalopathy, psychiatric symptoms, and seizures associated with increased level of anti-thyroid antibodies. The exact pathophysiology underlying cerebral involvement is still lesser known. Although symptoms suggest a nonlesional encephalopathy in most of the cases, sometimes the clinical appearance can be subtle and may not respond to immunosuppressants or immunomodulatory agents. Here we report a case who presented with drowsiness and amnestic complaints associated with paroxysmal electroencephalography (EEG) abnormalities which could be treated only with an antiepileptic drug.

Two Cases With Developing Neurologic Complications After Liver Transplant.

The widespread use of immunosuppressive agents has significantly increased the rates of successful solid-organ and stem cell transplants, especially with liver and kidney. Cyclosporine and tacrolimus are most commonly used for this purpose. Although these agents have different mechanisms of action, both have various adverse effects, including nausea, vomiting, headache, hypertension, nephrotoxicity, and rarely epileptic seizures. In our first case, a patient presented with epileptic seizures and hemiparesis after a liver transplant, and posterior reversible encephalopathy syndrome related to cyclosporine toxicity was considered. Once cyclosporine levels in the blood decreased, the patient had both clinical and radiologic improvements. In our second case, a patient presented with delirium after a liver transplant. Again, when cyclosporine levels in the blood decreased, the patient showed improvement in clinical findings. Neurologic complications may develop after liver transplant, and these complications are encountered most frequently within the first postoperative month. Neurologic complications are multifactorial; insufficient graft function, intracranial bleeding, cerebral infarcts, infections, and immunosuppressive drug toxicity (tacrolimus and cyclosporine) may be considered among these factors. As shown in our presented cases, most neurologic complications can be successfully treated by correcting the underlying factor.

Statistical shape analysis of temporal lobe in mesial temporal sclerosis patients.

BACKGROUND: Surgery is regarded as a common treatment option for patients with mesial temporal lobe epilepsy due to hippocampal sclerosis but sometimes deciding this diagnosis can be very difficult. We aim to investigate the shape differences in the temporal lobe of mesial temporal sclerosis epilepsy patients compared with healthy controls, investigating the side difference and, if present, assessing the clinical application of this situation. METHOD: The MRI scans of mesial TLE patients and controls were retrospectively reviewed. Temporal lobe data were collected from the two-dimensional digital images. Standard anthropometric landmarks were selected and marked on each digital image using TPSDIG 2.04 software. Eight anatomic landmarks were marked on images. A generalized Procrustes analysis was used to evaluate the shape difference. The shape deformation of the temporal lobe from control to patient was evaluated using the TPS method. RESULTS: There were statistically significant TL shape differences between groups. High level deformations for the left and right side from the control to patient group were seen in the TPS graphic. The highest deformation was determined at the inferior lateral temporal midpoint of the middle temporal gyri and superior temporal landmark points of both the right and left sides. CONCLUSION: Our study for the first time demonstrated temporal shape differences in TLE patients using a landmark-based geometrical morphometric method by taking into consideration the topographic distribution of TL.

A case of mycosis fungoides-like lesions developing after levetiracetam therapy.

Levetiracetam is a relatively new antiepileptic drug that has previously not been associated with severe dermatological side effects. We report the case of a 31-year-old male treated with levetiracetam for seizures who subsequently developed a mycosis fungoides-like drug reaction that resolved upon dosage reduction.

Sporadic Creutzfeldt-Jacob Disease: An 8-Year Experience from a Single Center in Turkey.

INTRODUCTION: Our aim was to conduct a retrospective review to demonstrate the clinical, radiological, and electrophysiological features of patients with sporadic Creutzfeldt-Jacob disease (sCJD). METHOD: A total of 10 patients (5 female and 5 male, with a mean age of 45 years from a range of 40 to 67 years) out of 8.259 adult patients hospitalized from January 2000 to December 2008 were diagnosed with sCJD. RESULTS: Eight of the patients were diagnosed on the basis of clinical, radiological, electroencephalography (EEG), and cerebrospinal fluid (CSF) findings. Two other patients also had a pathological diagnosis. The most common signs and symptoms were behavioral disturbances, movement disorders, cognitive decline, myoclonus, psychosis, focal neurological deficit, and aphasia. Nine of the patients had periodic sharp wave discharges on EEG. Seven patients were positive for the 14.3.3 protein in the CSF. Five patients had pulvinar signs-a bilateral increased signal in the pulvinar thalami-on cranial magnetic resonance imaging. Eight patients were diagnosed with probable sCJD; two were diagnosed with definite sCJD. All of the patients died as a result of the disease within 24 months after the onset of symptoms. DISCUSSION: sCJD should be considered in the diagnosis of patients who present with rapidly progressive dementia. Clinical and radiological data appear to be sufficient for the diagnosis. However, detailed molecular examinations of the subtypes of the disease may be required for early diagnosis of cases given the wide spectra of CJD.

Sociodemographics, clinical features, and psychiatric comorbidities of patients with psychogenic nonepileptic seizures: experience at a specialized epilepsy center in Turkey.

Patients with psychogenic nonepileptic seizures (PNESs) or pseudoseizures are known to have psychiatric comorbidities. In the present retrospective analysis, we examined the sociodemographics, clinical characteristics, and psychiatric diagnoses of patients with PNESs. Our aim is to demonstrate the contribution of the consulting psychiatrists to the presumed psychiatric diagnoses of the neurologists. We used data from long-term video EEG monitoring (LVEM) performed at a specialized epilepsy center in Turkey. The study group comprised 67 patients (mean age: 30 years, 75% female) diagnosed with PNESs following LVEM of approximately 5 days' duration. Two hundred thirty-three episodes were recorded. Most of the patients experienced a PNES on the first day. All of the patients were taking antiepileptic drugs (AEDs) at the time of admission; 56.7% were taking antidepressant (AD) drugs. All of the PNES patients were diagnosed with conversion disorder by both the neurologists and the psychiatrists. Most of the PNES patients were using multiple AEDs. Cooperation between neurologists and psychiatrists and ongoing education for both neurologists and psychiatrists about PNES are needed in appropriate diagnosing and management of patients with PNES.

What is the efficacy of nasal surgery in patients with obstructive sleep apnea syndrome?

PURPOSE: Obstructive sleep apnea syndrome (OSAS) is characterized by repetitive episodes of partial or complete obstruction of the upper airway during sleep and is associated with increasing respiratory efforts, with a consequent oxyhemoglobin desaturation, sleep fragmentation, and daytime symptoms, most commonly excessive sleepiness. The effectiveness of continuous positive airway pressure (CPAP) is undoubtedly high in treating those patients who use it regularly, but for those who refuse it, the success rate is 0. It is for this subset of patients that surgical therapy can be useful. The purpose of this study was to evaluate the effects of nasal surgery on nasal resistance, sleep apnea, sleep quality, and nasal volumetric measurement in adult male patients with OSAS. METHODS: Twenty male patients with complaints of hypersomnia and snoring were included in the study. Polysomnography of patients with the prediagnosis of OSAS was planned. All patients underwent CPAP treatment before and after surgery. Patients, who had anatomic structural defects causing nasal valve shrinkage, were operated on at the Plastic Reconstructive and Aesthetic Department. Volumetric measurements of the nose were obtained before and after the operation. RESULTS: In our study, it was observed that respiratory tract space of patients increased subsequent to the surgery, and thereby OSAS level decreased, and tolerating CPAP device was easier. Measurements of internal nasal valve vertex and fields and external nasal valve fields before and after operation were significant. CONCLUSIONS: Surgical relief of this nasal obstruction may improve quality of life in patients with OSAS.

Delayed diagnosis of a neuroBehçet patient with only brainstem and cerebellar atrophy: literature review.

We report a 34-year-old male neuroBehçet's Disease (NBD) patient with atypical magnetic resonance imaging (MRI) findings, whose behavioral problems were followed by progressive neurological symptoms. The patient was hospitalized due to forgetfulness, irritability, behavioral dyscontrol and a choking sensation. T2-weighted MRI showed prominent atrophy of cerebellar hemispheres, the cerebellar peduncle, the midbrain and the pons. He was diagnosed with NBD after an evaluation of his medical history together with neuropsychiatric and laboratory findings. There are few reports of NBD with only brainstem and cerebellar atrophy. We discuss our patient in the context of the four previously reported cases. In NBD without evident mucocutaneo-ocular symptoms, neurologists should always consider the medical and family history. Early diagnosis of NBD helps to initiate appropriate treatment, thereby modulating the course of the disease and preventing complications.

Proton magnetic resonance spectroscopy study of bilateral thalamus in juvenile myoclonic epilepsy.

PURPOSE: To investigate neuronal dysfunction in the thalami of juvenile myoclonic epilepsy (JME) by using proton magnetic resonance spectroscopy (MRS). METHODS: We performed single-voxel proton MRS over the right and the left thalami of 15 consecutive patients (10 women, 5 men) with JME (mean age 20.3 years) and 16 healthy volunteers (10 women, 6 men) (mean age 24.5 years). All patients had seizure onset in late childhood-teenage, normal neurologic examination, typical electroencephalogram (EEG) of JME and normal magnetic resonance imaging (MRI). We determined N-acetylaspartate (NAA) values and NAA over creatine-phosphocreatine (Cr) values. Mann-Whitney U-test was used to evaluate group differences. RESULTS: Group analysis showed that echo time (TE) 270 integral value of NAA over left thalamus were significantly decreased in JME patients as compared with controls (34.6033+/-15.8386; 48.0362+/-22.2407, respectively, P=0.019). Also group analysis showed that thalami NAA/Cr ratios were significantly decreased in JME patients (right side, 2.21+/-1.07; left side 2.00+/-0.72) as compared with controls (right side, 3.45+/-1.50; left side, 3.08+/-1.60; P=0.011 and P=0.030, respectively). CONCLUSION: In the previous studies, NAA values in patients with JME found that they were not statistically lower in thalami than control group. But, in our study, NAA value was found low as well. It has been known that NAA is a neuronal marker and hence it is a valuable metabolite in the neuron physiopathology. As a result, in the patients with JME we tried to support the theory that the underlying mechanism of the generalized seizures was the abnormal thalamocortical circuity, determining the thalamic neuronal dysfunction in MRS statistically.