Betül Tekin Güveli

Stigmatization and social impacts of epilepsy in Turkey

OBJECTIVE Stigma associated with epilepsy has negative effects on psychosocial outcomes, affecting the lives of people with epilepsy (PWE). Obtaining basic social rights can be difficult compared to the general population. The aim of our study was to evaluate the perceived stigma among PWE and social attitude towards the disease and to compare the social measures with the general population in Turkey. METHOD A self-completed questionnaire consisting of demographic details and items about attitudes and perceived stigmatization was developed. Participants consisted of patients with various types of seizures who were randomly chosen from the epilepsy outpatient clinic. They were requested to complete the questionnaire. RESULTS Questionnaires were obtained from 330 PWE. One hundred forty individuals (43.3%) out of 323 reported feeling stigmatized. The marriage and total fertility rates were below the national rates of Turkey. Keeping their epilepsy as a secret from society was prevalent. Although the education rate was not below the national rate, unemployment rate was high, and the average monthly wage was significantly lower than that of the general population. The majority thought that their families were protective towards them. Only 2 of the 330 participants were living alone. CONCLUSION The present study supports the perception of stigma associated with epilepsy and its negative impact on the lives of PWE in Turkey. Clearly, more research is needed to understand the reasons for stigma and how to decrease its impact.

Eye closure sensitivity in juvenile myoclonic epilepsy and its effect on prognosis.

PURPOSE To investigate eye closure sensitivity (ECS) in the EEGs of patients diagnosed with juvenile myoclonic epilepsy (JME) and its relationship to prognosis. METHODS We included 76 JME patients with a minimum follow-up of one year and evaluated a total of 254 EEGs to obtain evidence of ECS. The patients were grouped according to their response to treatment, and these subgroups were compared in relation to ECS and other clinical and EEG features. RESULTS There were 12 patients (15.8%) with poor prognosis who showed resistance to appropriate anti-epileptic drug treatment, 15 (19.7%) patients with pseudo-resistance, and 49 (64.5%) patients with good prognosis. The EEGs of only four of the patients displayed pure ECS (5.3%), and only one of these exhibited poor prognosis. Furthermore, 11 patients (14.5%) had both ECS and photosensitivity, and two of these patients exhibited poor prognosis. Thus, neither pure ECS nor ECS with photosensitivity correlated with poor prognosis. A family history of epilepsy and focal findings on the EEG was correlated with poorer prognosis. CONCLUSIONS ECS is a rare EEG finding in JME and does not appear to be a marker for poor prognosis.

Long-term outcomes in patients with West syndrome: An outpatient clinical study

PURPOSE Nearly half of all patients with seizure onset in the first year of life suffer from West syndrome (WS). The prognosis of epilepsy and psychosocial outcomes in children with WS are variable. This study was performed to examine the factors influencing the outcome of this patient population. METHODS A total of 109 patients with WS followed up regularly for at least 3 years were included in the study. Relevant clinical, laboratory, and imaging data were collected. RESULTS The male/female ratio was 65/44 (59.6%/40.4%). The mean age at onset of infantile spasm (IS) was 6 ± 6 (1-36) months. With regard to neuro-developmental and social conditions during the final evaluation, 29.4% of the patients were socially dependent on caregivers, 61.8% needed assistance, and 8.8% were normal. Among the patients, 5.9% were free of epilepsy and antiepileptic drugs (AED) for at least 2 years, 49.0% had no seizures with AEDs, and 45.1% had uncontrollable seizures. Parameters with significant negative effects on the long-term outcomes included symptomatic etiology, presence of developmental retardation before the onset of IS, persistence of active epilepsy, and male gender. CONCLUSION In this study, 37 (33.9%) patients had severe consequences as a result of WS. The majority of the rest could cope with daily life with varying degrees of assistance. Eight percent of the patients had a normal development. These results draw attention to the two-thirds of patients with WS who have the chance of an acceptable quality of life (QoL) with early diagnosis and therapeutic measures.

Teratogenicity of Antiepileptic Drugs

Objective Antiepileptic drugs (AED) have chronic teratogenic effects, the most common of which are congenital heart disease, cleft lip/palate, urogenital and neural tube defects. The aim of our study is to examine teratogenic effects of AED and the correlation between these malformations and AED in single or multiple pregnancies. Methods This is a retrospective study of malformations in children born to mothers currently followed up by our outpatient clinics who used or discontinued AED during their pregnancy. Their children were then investigated using echocardiography, urinary ultrasound, cranial magnetic resonance image, and examined by geneticists and pediatric dentists. Results One hundred and seventeen children were included in the study. Ninety one of these children were exposed to AED during pregnancy. The most commonly used AED were valproic acid and carbamazepine in monotherapy. The percentage of major anomaly was 6.8% in all children. Dysmorphic features and dental anomalies were observed more in children exposed especially to valproic acid. There were 26 mothers with two and four mothers with three pregnancies from the same fathers. No correlation was found between the distribution of malformations in recurring pregnancies and AED usage. Conclusion Our study has the highest number of dysmorphism examined in literature, found in all the children exposed to valproic acid, which may account for the higher rate of facial dysmorphism and dental anomalies. On lower doses of valproic acid, major malformations are not seen, although the risk increases with polytherapy. Our data also indicate possible effects of genetic and environmental factors on malformations.

Misdiagnosis in JME: Still a problem after 17 years?

PURPOSE Juvenile myoclonic epilepsy (JME) is one of the most common and recognizable idiopathic generalized epilepsy with its characteristic clinical and EEG features. We think despite the well defined diagnostic criteria, and increasing awareness, misdiagnosis in JME may still be a problem. The present study aims to determine misdiagnosis in JME and to compare the results with our previous study reported in 1998. METHODS Two hundred JME patients examined at epilepsy outpatient clinics of Bakirkoy Prof. Dr. Mazhar Osman Training and Research Hospital for Psychiatric, Neurologic and Neurosurgical Diseases between the years 2014-2015 were enrolled. Medical records of all patients were evaluated retrospectively; demographical, clinical and electrophysiological data and causes of misdiagnosis were collected from chart reviews. RESULTS Of 200 JME patients, 49 were misdiagnosed at first medical evaluation. The most common presenting seizure types were generalized tonic clonic seizure and myoclonia in misdiagnosed patients and correctly diagnosed patients, respectively. EEG revealed generalized spike wave and polyspike-wave discharges in 52% of the misdiagnosed patients. Unfortunately the physician was a neurologist in 87.8% of cases with misdiagnoses. Nearly half of 49 misdiagnosed patients were prescribed an inappropriate antiepileptic drug, and the other half were prescribed none. CONCLUSIONS Comparing our new results with the ones in 1998, misdiagnosis rate was less and time to put a correct diagnosis was shorter. However, proper diagnosis at first sight is still a problem among neurologists even the typical EEG changes are present.

Effects of Oxcarbazepine and Levetiracetam on Calcium, Ionized Calcium, and 25-OH Vitamin-D3 Levels in Patients with Epilepsy

Objective The primary objective of the present study was to further elucidate the effects of oxcarbazepine (OXC) and levetiracetam (LEV) monotherapies on the bone health status of patients with epilepsy. Methods This study included 48 patients who attended our epilepsy outpatient clinic, had a diagnosis of epilepsy, and were undergoing either OXC or LEV monotherapy and 42 healthy control subjects. The demographic and clinical features of the patients, including gender, age, onset of disease, daily drug dosage, and duration of disease, were noted. Additionally, the calcium, ionized calcium, and 25-OH vitamin-D3 levels of the participants were prospectively evaluated. Results The 25-OH vitamin-D3, calcium, and ionized calcium levels of the patients taking OXC were significantly lower than those of the control group. These levels did not significantly differ between the patients taking LEV and the control group, but there was a significant negative relationship between daily drug dose and ionized calcium levels in the LEV patients. Conclusion In the present study, anti-epileptic drugs altered the calcium, ionized calcium, and 25-OH vitamin-D3 levels of epilepsy patients and resulted in bone loss, abnormal mineralization, and fractures. These findings suggest that the calcium, ionized calcium, and 25-OH vitamin-D3 levels of patients with epilepsy should be regularly assessed.

A rare cause of cerebral venous thrombosis: cryptococcal meningoencephalitis

Cryptococcal meningoencephalitis (CM) is a serious central nervous system infection caused by Cryptococcus neoformans, seen mostly in immunocompromised hosts and less in immunocompetent patients. The vast majority of cryptococcosis cases are seen as human immunodeficiency virus infections with advanced immunosuppression. Meningitis and meningoencephalitis are the most common clinical manifestations. Nevertheless, immunocompetent patients with CM are rarely reported. Cerebral venous sinus thrombosis is a rare complication of CM. Here, we report an immunocompetent patient with CM from a non-endemic area, who presented with an acute onset and atypical symptoms associated with cerebral venous thrombosis.

Long-Term Clinical and Electroencephalography (EEG) Consequences of Idiopathic Partial Epilepsies

Background Idiopathic partial epilepsies of childhood (IPE) affect a considerable proportion of children. Three main electroclinical syndromes of IPE are the Benign Childhood Epilepsy with Centro-temporal Spikes (BECTS), Panayiotopoulos Syndrome (PS), and Childhood Epilepsy with Occipital Paroxysms (CEOP). In this study we investigated the long-term prognosis of patients with IPE and discussed the semiological and electroencephalography (EEG) data in terms of syndromic characteristics. Material/Methods This study included a group of consecutive patients with IPE who had been followed since 1990. Demographic and clinical variables were investigated. Patients were divided into 3 groups – A: Cases suitable for a single IPE (BECTS, PS and CEOP); B: cases with intermediate characteristics within IPEs; and C: cases with both IPE and IGE characteristics. Long-term data regarding the individual seizure types and EEG findings were re-evaluated. Results A total of 61 patients were included in the study. Mean follow-up duration was 7.8±4.50 years. The mean age at onset of seizures was 7.7 years. There were 40 patients in group A 40, 14 in group B, and 7 in group C. Seizure and EEG characteristics were also explored independently from the syndromic approach. Incidence of autonomic seizures is considerably high at 2–5 years and incidence of oromotor seizures is high at age 9–11 years. The EEG is most abnormal at 6–8 years. The vast majority (86%) of epileptic activity (EA) with parietooccipital is present at 2–5 years, whereas EA with fronto-temporal or multiple sites become more abundant between ages 6 and 11. Conclusions Results of the present study provide support for the age-related characteristics of the seizures and EEGs in IPE syndromes. Acknowledgement of those phenomena may improve the management of IPEs and give a better estimate of the future consequences.

Behavioral characteristics and cognitive development among school age children born to women with epilepsy

Abstract Introduction: Greater risks of congenital malformation as well as cognitive and behavioral development in later childhood occur as a result of in utero exposure to antiepileptic drugs (AEDs). We examined the effects of AEDs on behavioral characteristics and cognitive development among school age children born to women with epilepsy. Methods: Children aged 6–15 years and born to women with epilepsy were enrolled in the study. Information was collected on the women’s demographics and the details of their usage/non-usage of AEDs during pregnancy. The Wechsler Intelligence Scale for the Children-Revised (WICS-R) test was administered to the children. The Alexander IQ test and the Conner Parent Rating Scale (CPRS) were administered to the mothers. Comparisons were made between children who had been exposed and not exposed to AEDs in utero and if exposed, according to exposure to AED monotherapy or polytherapy. The mother’s education level and IQ score and data from the same parent’s siblings were evaluated with respect to consanguinity. Results: Forty-one children born to 28 women with epilepsy were enrolled. Seven mothers had multiple pregnancies. Twenty-three pregnancies (56%) were exposed to monotherapy and five (12·1%) to polytherapy. The remaining 13 (31·7%) were not exposed to AEDs. Maternal education level was a significant major factor in child IQ development (P < 0·05). The performance IQ-coding scale results were lower in children exposed to polytherapy than in children exposed to monotherapy in utero (P < 0·05). Although it is difficult to assert with confidence, the sibling assessment indicated a negative effect of valproate on IQ. Conclusion: It is important that the AED dosage be reduced to a minimum to maintain seizure control for healthy cognitive and behavioral development of a child.

Panayiotopoulos syndrome and symptomatic occipital lobe epilepsy of childhood: a clinical and EEG study

AIM Panayiotopoulos syndrome (PS) is an age-related seizure susceptibility syndrome that affects the central autonomic system. Although the majority of the few ictal recordings obtained so far suggest an occipital origin, semiological and interictal EEG data appear to favour more extensive involvement. In this study, the characteristics (including those based on semiology and EEG) of children with Panayiotopoulos syndrome (n=24) and those with lesion-related, symptomatic occipital lobe epilepsy (SOLE) (n=23) were compared. METHODS Detailed semiological information and EEG parameters including the localisation, distribution, density (n/sec), reactivity, and morphological characteristics of spike-wave foci and their relationship with different states of vigilance were compared between the two groups. RESULTS The age at seizure onset was significantly younger in patients with symptomatic occipital lobe epilepsy than in those with PS (mean age at onset: 3.4 versus 5.6 years, respectively; p=0.044). Autonomic seizures (p=0.001) and ictal syncope (p=0.055) were more frequent in PS than in symptomatic occipital lobe epilepsy (87.5% and 37.5% versus 43.5% and 13%, respectively). The interictal spike-wave activity increased significantly during non-rapid eye movement (non-REM) sleep in both groups. The spike waves in non-REM seen in PS tended to spread mainly to central and centro-temporal regions. CONCLUSIONS The results indicate that although common features do exist, Panayiotopoulos syndrome differs from symptomatic occipital lobe epilepsy and has a unique low epileptogenic threshold related to particular brain circuits.