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 clinical evaluation of gelastic and dacrystic seizures: a multicenter study / Uma avaliação clínica das crises gelásticas e dacrísticas: um estudo multicêntrico

Abstract 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.

Resumo 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.

Surround inhibition in patients with juvenile myoclonic epilepsy.

OBJECTIVE: In healthy subjects, there is a reduction in the amplitudes of somatosensory-evoked potentials (SEPs) after the simultaneous stimulation of two nerves compared to the sum of separate stimulations. This reduction is due to the inhibition of one area in the cortex after stimulation of the neighboring area, which results from the surround inhibition (SI) phenomenon. In this study, we aimed to investigate whether there was a decrease in SI of SEP in patients with juvenile myoclonic epilepsy (JME). METHODS: We included 17 patients with JME and 18 healthy subjects. Groups were similar in terms of age and gender. We recorded SEPs after stimulating (i) median nerve (mSEP), (ii) ulnar nerve (uSEP), (iii) median and ulnar nerves simultaneously (muSEP) at wrist. The arithmetic sum (aSEP) of amplitudes of mSEP and uSEP was compared with the amplitudes of muSEP. We also calculated SI%. RESULTS: The amplitudes of SEPs were significantly higher in the JME group than in the healthy subjects (mSEP, p = 0.005; uSEP, p = 0.032; muSEP, p = 0.014). In healthy subjects and the JME group, the amplitude of muSEP was significantly lower than the aSEP (p = 0.014; p = 0.001, respectively). However, SI% was significantly higher in the JME group (p = 0.010). SIGNIFICANCE: Although the SI is maintained in JME patients, the higher SI% indicates an impairment relative to healthy subjects.

Does Valproic Acid/Na Valproate Suppress Auditory Startle Reflex in Patients With Epilepsy?

BACKGROUND AND OBJECTIVE: Auditory startle response (ASR) was normal in juvenile myoclonic epilepsy whereas it was suppressed in progressive myoclonic epilepsy. However, both groups were using valproic acid/Na valproate (VPA) in different doses. Therefore, we aimed to analyze whether VPA has an impact on ASR in a cohort of epilepsy. For this purpose, we included patients with epilepsy and analyzed ASR in patients who were using VPA. PATIENTS AND METHOD: We included 51 consecutive patients who had epilepsy and were using VPA between January 2014 and January 2016. Two control groups of 37 epilepsy patients using other antiepileptic drugs (AEDs) and of 25 healthy subjects were also constituted. All participants underwent investigations of ASR and startle response to somatosensory inputs (SSS) under similar conditions. RESULTS: An analysis of patients using VPA, not using VPA and healthy subjects revealed significantly longer latency and lower probability of orbicularis oculi (O.oc) and sternocleidomastoid responses after auditory stimulation, decreased total ASR probability and longer latency of O.oc response after somatosensory stimulation in patient groups compared with healthy subjects. Multivariate analysis showed type of AED had a role in the generation of abnormalities. VPA, carbamazepine, and multiple AED use caused suppression of ASR. Total ASR probability was decreased or O.oc latency got longer with longer duration of VPA use whereas serum VPA level at the time of investigation did not correlate with total ASR probability. DISCUSSION: Both ASR and SSS are suppressed by the effect of VPA, especially in patients using for a long period and in patients using other AEDs with VPA. Given the fact that VPA leads to long-standing synaptic changes of dopaminergic transmission, abnormalities of this network may be the more likely cause.

Anti-Neuronal Autoantibodies in Both Drug Responsive and Resistant Focal Seizures with Unknown Cause.

BACKGROUND: and Objective Autoimmunity is an emerging field of research in the etiology of different neurological disorders including epilepsy. We aimed to investigate the presence of neuronal autoantibodies in focal epilepsy with unknown cause and their clinical correlates in both drug-responsive and resistant patients. METHOD: Between 2009 and 2010 94 patients were prospectively enrolled, had their antibodies tested and clinically followed." An additional 50 age- and gender-matched controls were also tested for antibodies. Age at examination, gender, age at onset, seizure frequency, risk factors, seizure precipitants, and type of seizures were noted. Plasma obtained from patients was frozen at -80°C and analysed for autoantibodies against VGKC-complex, VGCC, GAD, LGI1, CASPR2, NMDA, AMPA and GABAB receptors with immunocytochemistry and radioimmunoassay as required. RESULTS: Thirteen (13.8%) patients, but none of the controls, had antibodies (p=0.003). Antibodies were directed against the uncharacterized components of VGKC-complex in 5 patients (5.3%), GAD in 4 patients (4.2%), NMDA-R in 1 patient (1%), AMPA-R in 1 patient (1%) and both GAD and VGKC-complex in 2 patients (2.1%). Prognosis of epilepsy, in subsequent follow-up, did not correlate to general presence of anti-neuronal antibodies with slightly more patients with antibodies epilepsy control than without (76.9% vs. 69.1%, not-statistically significant. Three patients with suspected active autoimmunity and epilepsy who were treated, showed a response to treatment with a reduction in the seizure frequency. Although most clinical features were identical between seropositive and seronegative patient groups, seropositive patients were more likely to have inflammatory/autoimmune disorders in their medical history. DISCUSSION: In keeping with previous studies, we have shown anti-neuronal antibodies in a proportion of focal epilepsy patients. Although autoimmunity might merely occur as a bystander effect in many chronic neurological disorders, association of anti-neuronal antibodies with good response to immunotherapy and coexisting autoimmune disorders suggests that anti-neuronal autoimmunity might participate in seizure formation at least in a subgroup of focal epilepsy patients. CONCLUSION: Immunity may play a role in some patients with unknown etiology regardless of prognosis and immunmodulatuar treatment may be helpful in seropositive group.

Orienting reaction may help recognition of patients with psychogenic nonepileptic seizures.

OBJECTIVE: Psychogenic nonepileptic seizures (PNES) are abrupt, paroxysmal changes in behavior or consciousness that may phenomenologically resemble epileptic seizures. Given the known association between anxiety and PNES, we hypothesized that in these subjects there may be evidence that the nervous system is hypersensitive to external stimuli. We aimed to test our hypothesis by means of the auditory startle reaction (ASR). By investigating ASR, we also had the opportunity to test presence of orienting reaction, which is generally defined as the second phase of response after the auditory stimulus, with longer latency. METHODS: We included 22 patients diagnosed as PNES and 25 age- and gender-matched healthy subjects. Clinical assessments and ASR recordings were performed. Electrophysiological findings were compared between patients with PNES and healthy subjects, including the presence of an orienting reaction. Orienting reaction was defined as a late response with latency between 100-1000ms. RESULTS: The mean ages of patients with PNES and healthy subjects were 34.9±12.3 years and 33.3±10.9 years, respectively (P=0.709). All patients were diagnosed as having conversion disorder. Additionally, 19 patients had depressive disorder and four had anxiety. The recruitment pattern of muscles and probability were similar between patients with PNES and healthy subjects. Orienting reaction was solely observed in patients with PNES (n=13, 59.1% of the patients vs. no healthy subject). The sequence and contribution of muscles in the orienting reaction changed almost in all patients. The duration of these responses was long, sometimes more than 200ms. CONCLUSION: PNES is associated with orienting reaction. This provides a possible electrophysiological marker of altered nervous system function in patients with PNES and may also reflect the distorted emotional processing in these patients.

Startle Response in Progressive Myoclonic Epilepsy.

Cortical reflex myoclonus is a typical feature of progressive myoclonic epilepsy (PME) in which it is accompanied by other types of mostly drug-resistant seizures and progressive neurological signs. Although PME is characterized by cortical hyperexcitability, studies have demonstrated atrophy and degenerative changes in the brainstem in various types of PME. Thus, we have questioned whether any stimuli may trigger a hyperactive response of brainstem reticular formation in PME and investigated the startle reflex in individuals with PME. We recorded the auditory startle response (ASR) and the startle response to somatosensory inputs (SSS) in patients with PME, and compared the results with healthy volunteers and patients with other types of drug-resistant epilepsy. All patients were using antiepileptic drugs (AEDs), 12 were on multiple AEDs. The probability of ASR was significantly lower and mean onset latency was longer in patients with PME compared with other groups. SSS responses over all muscles were low in both the PME and drug-resistant epilepsy groups; however, the differences were not statistically significant. The presence of a response over the biceps brachii muscle was zero in the PME group and showed a borderline difference compared with the other groups. Decreased probability and prolonged latencies of ASR in PME indicate inhibition of reflex circuit. A trend for decreased responses of SSS suggests hypoactive SSS in both PME and other epilepsy groups. Hypoactive ASR in PME and hypoactive SSS in both PME and other epilepsies may be attributed to the degeneration of pontine reticular nuclei in PME and functional inhibition by AEDs in both disorders.

Electrophysiological findings in Rasmussen's syndrome.

Rasmussen syndrome is a rare, inflammatory and probably autoimmune disease presenting with epilepsia partialis continua which is generally in the form of myoclonic jerks and involves the upper extremities with or without head involvement. We sought to demonstrate the electrophysiological features in patients with Rasmussen syndrome. We performed continuous electrophysiological recordings of involuntary movement, as well as recordings of startle responses and long latency reflex in three patients with a diagnosis of Rasmussen syndrome. Positive and negative myoclonus were recorded. Startle responses were found to be suppressed. However, long latency reflexes were high in amplitude and one patient even had a C reflex. Stimulus-sensitive positive and negative cortical myoclonus are typical in epilepsia partialis continua of Rasmussen syndrome and degeneration of brainstem and reticulospinal pathways may develop in Rasmussen syndrome.

The role of sleep electroencephalography in patients with new onset epilepsy.

PURPOSE: An increased propensity for seizures is associated with different stages of the sleep-wake cycle. In this study, we prospectively analyzed patients with new-onset epilepsy and investigated the clinical correlates of the yield obtained from sleep electroencephalography (EEG) recordings in patients with a normal wakefulness EEG. METHODS: All patients admitted to our epilepsy unit due to unprovoked epileptic seizures and not yet treated with antiepileptic drugs were recruited consecutively for the last three years. All had a routine EEG at wakefulness (WEEG), and those with no epileptiform activity had a video-EEG recording during sleep (SEEG). RESULTS: We investigated a total of 241 patients; 129 patients (53.5%) had both wakefulness and sleep EEG recordings. The patients with abnormal WEEG were older than those with normal WEEG (p = 0.005). Abnormal WEEG was detected in only 31.2% of patients with focal seizures, but in 77.3% of patients with generalized seizures (p < 0.001). WEEG was abnormal in 44.0% of patients with diurnal seizures, but in 27.5% of nocturnal seizures (p = 0.007). Abnormal WEEG was present in 75.5% of patients with a presumed genetic origin and in 59.3% of patients with structural etiology (p < 0.001). Sleep EEG detected an abnormality in 41.8% of patients with normal WEEG; of these, 82.8% were focal abnormalities. In contrast, the majority of abnormalities detected in WEEG were generalized (55.8%, p < 0.001). CONCLUSION: Our results showed a greater likelihood of abnormal WEEG in older patients and in those with generalized epilepsy, diurnally precipitating seizures, and epilepsy of presumed genetic origin.

Blink reflex in progressive myoclonic epilepsies.

PURPOSE: Progressive myoclonic epilepsies (PME) include a heterogeneous group of disorders. The brainstem is involved in these disorders, as demonstrated by neuroimaging and autopsy studies. The blink reflex (BR) is characteristically elicited after supraorbital electrical stimulation. The BR has two components, an ipsilateral R1 and bilateral R2 (R2 and R2c). The central generator of the BR is the brainstem. In this study, we aimed to investigate the functional status of the brainstem using the BR in PME cases with different etiological factors. METHODS: We prospectively included 17 patients with a diagnosis of PME (8 male, 47.1%) who were examined between June 2009 and June 2012. For comparison, we included 41 healthy volunteers (18 male 43.9%) who did not have any neurological or systemic diseases. We recorded responses bilaterally over the orbicularis oculi muscles after supraorbital stimulation in all participants. RESULTS: The R1 and R2 components of the BR were obtained in all healthy subjects with normal latencies, whereas abnormalities in the R2 and R2c components were observed at significantly higher rates in the PME patients. The mean latencies of the bilateral R2 and R2c components were significantly prolonged, and the amplitudes were diminished in the PME patients. Disease duration and the use of multiple antiepileptic drugs were related to abnormal R2s. CONCLUSION: The abnormalities of the R2 and R2c components of the BR confirmed the inhibition of the reticular formation. The findings are probably related to disease processes and partially due to the use of multiple antiepileptic drugs.

Surgical and anesthesiological considerations of awake craniotomy: Cerrahpasa experience.

AIM: Awake craniotomy (AC) with electrical cortical stimulation has become popular during the last ten years although the basic principles were introduced almost 50 years ago. The aim of this paper is to share with the readers our experience in 25 patients who underwent AC with electrical stimulation. MATERIAL AND METHODS: Twenty-five patients who underwent AC between 2010 and 2013 are the subjects of this paper. All patients were diagnosed with intraaxial lesions involving the functional area itself or very close to it by preoperative imaging. During surgery, the functional area was demonstrated by cortical electrical stimulation and resection aimed to preserve it in order to avoid an irreversible functional deficit. RESULTS: Total resection was possible in 80% while in 20% subtotal resection had to be performed because of involvement of the functional area itself. The neurological complication rate was found to be 16% (4 patients) and all were transient. No complication regarding anesthesia was noted. CONCLUSION: Awake craniotomy in selected patients is very effective, safe and practical for supratentorial lesions close to the eloquent area. Complications related to the surgery itself are uncommon and general anesthesia is avoided. The hospital stay including the intensive care unit is short which makes it very economical surgical procedure.

Misdiagnosis of menstruation-related recurrent hypersomnia as epilepsy in a patient with generalized epileptic discharges.

Recurrent hypersomnias are very rare with two subtypes as Kleine-Levin syndrome and menstruation-related hypersomnia, which is very rarely encountered worldwide. Here, we report a young girl with menstruation-related recurrent hypersomnia, who was misdiagnosed as epilepsy due to co-existing generalized epileptic discharges. The importance of this comorbidity in terms of differential diagnosis of the attacks is discussed.

Auditory startle response is normal in juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy (JME) is hypothesized to originate from the dysfunction of thalamo-cortical circuit. We aimed to analyze any changes in auditory startle response in JME patients to determine the role of brainstem in JME. The responses of 18 JME patients to auditory simulation were recorded over the unilateral orbicularis oculi, masseter, sternocleidomastoid, and extremity muscles. Results were compared with those of 18 age and gender matched healthy volunteers. Total auditory startle response frequencies were similar between the two groups (31.1 ± 11.1 % vs. 33.7 ± 8.7 %, p = 0.400). Other parameters over each muscle were also similar. There were no impacts of antiepileptic drug use or disease duration. We may conclude that our findings may provide sufficient evidence for the lack of functional changes of the auditory startle response circuit even in longstanding cases of JME.

Re-emergent Tongue Tremor as the Presenting Symptom of Parkinson's Disease.

BACKGROUND: The most common symptom of Parkinson's disease is the unilateral, typically resting tremor in body parts, most commonly in the upper extremities. However, this finding can spread to the other parts of the body like lips, chin, jaw and tongue during the course of the disease. Nevertheless, we have not come across any Parkinson's disease case presenting with tongue tremor in the literature. CASE REPORT: Here, we present a 58 year-old man with Parkinson's disease presenting with tongue tremor, his striking response to the levodopa test and his follow-up data. CONCLUSION: The topography of motor symptoms at onset in Parkinson's disease is presumably determined by the severity of the Lewy body neuronal degeneration in distinct parts of the substantia nigra. Therefore, patterns of somatic symptom progression in Parkinson's disease indicate that involvement of the cranial structures always follows the development of symptoms in the extremities. However, in our case, cranial structures seem to precede the involvement of extremity-related areas.

Contribution of FDG-PET in epilepsy surgery: consistency and postoperative results compared with magnetic resonance imaging and electroencephalography.

AIM: Surgery is a treatment option for medically intractable epilepsy patients. Abnormalities in regional cerebral glucose metabolism, as identified by 18-fluorodeoxyglucose positron emission tomography (FDG-PET) have predictive prognostic value in evaluating the outcome of epilepsy surgery. This study investigated the efficacy of FDG-PET for delineation of the epileptogenic zone (EZ) by comparing its consistency with other diagnostic tools and surgical outcome. MATERIAL AND METHODS: We analyzed the results of 121 consecutive patients evaluated for epilepsy surgery. FDG-PET results were crosschecked with magnetic resonance imaging (MRI) and electroencephalography (EEG) results, as well as postoperative outcome and pathology. RESULTS: FDG-PET findings of 75 patients (62 %) were concordant with MRI (Mc-Nemar-χ2 test p=0.024, Kappa=0.22). Further, the PET findings were consistent with EEG, and was statistically significant, according to Post-hoc test, in temporal epilepsy (TLE) group (χ2=8.21 P=0.04). Both investigations revealed localizing information in 56 (46.2%) patients. Twenty-six (72.2%) MRI-negative patients had hypometabolism on PET. The pathology of the 10 PET-negative patients was 5 cases of mesial temporal sclerosis, 2 cortical dysplasia, 2 gliosis and one tumor. Seven (70%) of these patients' lesions originated from the temporal lobe. FDG-PET had correctly predicted the EZ in 37 (86%) of 43 patients who underwent surgery. CONCLUSION: FDG-PET results may not be strongly associated with EZ but represent an additional tool in delineation of EZ during the noninvasive phase of presurgical evaluation.

Survey on Smoking, Consuming Alcohol, and using Illicit Drugs in Patients with Epilepsy.

INTRODUCTION: Addiction can be defined as the continuous consumption of addictive substances or repetition of certain behaviors despite adverse consequences. Epilepsy is associated with an increased risk of psychiatric disorders. However, to the best of our knowledge, there is no published data regarding addictions in patients with epilepsy. Considering the high incidence of psychopathology, we planned a survey using a self-report questionnaire to study some of the addictive behaviors in patients with epilepsy and in control subjects. METHODS: Patients from our outpatient epilepsy clinic (n=106) and control subjects (n=96) aged between 18 and 65 years took the 20-question questionnaire that screened for smoking, consuming alcohol, or using other illicit drugs. RESULTS: Fifty-three percent of patients with epilepsy were male (n=57) and in the control group, 52% were male (n=50) (p=.062). The mean age was 32.66±2.23 years for patients with epilepsy and 35.70±0.59 years for the control group (p=.810). Mean duration of epilepsy was found to be 14.33±11.26 (1-46) years. Majority of patients with epilepsy (84%) had focal epilepsy. Alcohol intake was found to be significantly lower in patients with epilepsy (p=.0001). There was no difference regarding smoking (p=.530) or using illicit drugs between the groups (p=.262). Smoking cigarettes was lower in new onset epilepsies (<5 years) compared with epilepsies of longer duration (p=.031). CONCLUSION: Recent studies connote to some common substrates in the pathophysiology of epilepsy and addiction. Therefore, we wanted to evaluate some addictive behaviors in patients with epilepsy. Although this study did not show significant differences other than low frequency of alcohol use in patients with epilepsy and low rate of smoking in patients with epilepsy duration of <5 year, further studies among homogeneous epilepsy subgroups with larger scale along with their neuropsychological profiles may still be required.

Magnetic resonance imaging findings in probable Creutzfeld-Jacob disease: comparison with electroencephalography and cerebrospinal fluid characteristics.

BACKGROUND: Creutzfeld-Jacob disease (CJD) is a rare, progressive disease that has a vast clinical manifestation range. Cranial magnetic resonance imaging (MRI), electroencephalography (EEG), and measurement of 14-3-3 in cerebrospinal fluid (CSF) may offer a pragmatic approach in the diagnosis of CJD as an alternative to histopathological confirmation. PURPOSE: To present the symptoms and signs of the CJD patients in regard to radiological and neurophysiological findings. MATERIAL AND METHODS: We collected all cases with the diagnosis of probable CJD admitted to our neurology department between June 2010 and June 2014. The medical records and laboratory data, clinical features, results of MRI (including diffusion weighted images), EEG and CSF evaluations, and other laboratory data to exclude other possible diagnoses were recorded. None of the patients underwent biopsy or autopsy for histological diagnosis. RESULTS: Of 20 patients, 11 (55%) were men and nine (45%) were women. The mean age at disease onset was 60.0 ± 9.5 years (age range, 47-80 years). All patients without exception had characteristic abnormalities in DWI and/or FLAIR on admission, about 4 months after the initial symptom. Periodic complexes on EEGs characteristic for CJD were detected only in 10 patients (50%) on admission and in 13 patients (65%) during disease course. Out of 14 patients who underwent CSF examination, 11 (78.5%) were positive for 14-3-3 protein. CONCLUSION: Although the definite diagnosis of CJD is made histopathologically, we aimed to discuss the value of magnetic resonance imaging in the diagnosis of CJD in respect to EEG findings and protein 14-3-3 levels in CSF.

Low-grade temporal gliomas: surgical strategy and long-term seizure outcome.

Low-grade gliomas (LGGs) are generally located in temporal lobe and cause medically-intractable seizure so that surgical treatment becomes inevitable. This study includes a retrospective analysis of our patients with temporal LGGs retrieved from our epilepsy surgery data base and tries to present appropriate surgical approach and long-term seizure and anti-epileptic drug (AED) outcomes. Fifty-three patients including children and adults underwent surgery on temporal lobe LGGs and 35 patients were reached to report seizure and AED outcomes. On the non-dominant temporal lobe, anterior temporal resection with hippocampectomy whether mesial structure are involved or not is the appropriate approach. On the dominant temporal lobe mesial structures should be respected. However, total resection of the tumor should be the goal of surgery. Mean follow-up period was 8.3 years and favorable seizure outcome was found to be 91.4%. Surgery decreased AED usage and mean number of AED significantly decreased. Children also benefited from surgery as adults. Surgical treatment of tumor-related epilepsy from temporal lobe controls seizures, and total removal should be the main goal of surgery as neuropsychological testing permit.

End of the barbexaclone era: an experience of treatment withdrawal.

Barbexaclone is a salt compound of phenobarbital and propylhexedrine (a drug with indirect sympathomimetic properties). Due to the presence of the psychostimulating agent, propylhexedrine, this drug has less of a sedative effect and is well tolerated, compared to phenobarbital. Barbexaclone was widely used in Turkey until 2009 when its production ended, however, it gave rise to an epidemic for which we were not prepared. Since then, no standardised management protocol has been developed and each patient has been evaluated individually, thereby creating tailor-made solutions based on the extent of each patient's supply of remaining drug (from a few tablets to a stock which might last for six months). The rate of seizure freedom was 37.7% under barbexaclone treatment and dropped to 32.2% in the follow-up period after discontinuation of the drug. In the majority of cases, a new antiepileptic drug was added and this was commonly levetiracetam, a more expensive drug. In this article, we share our experiences of a general problem: the withdrawal of an antiepileptic drug from the market. Although there was prior notification regarding barbexaclone withdrawal, it was not possible to contact all patients since such a database is not available in Turkey. Although no conclusions regarding the efficacy of the drug or comparison of efficacy with other antiepileptic drugs is provided, it is nonetheless noteworthy to share these experiences since some patients had lost seizure control for reasons that could not be explained.