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Purpose The aim of the current study was to systematically review the literature to compare the efficacy of levetiracetam (LEV) with that of carbamazepine (CBZ) to control seizures and reduce the burden of interictal epileptiform discharges in children with rolandic epilepsy (RE) and also to compare their tolerability. Methods We searched the electronic database PubMed on January 9, 2019 for original articles that included the following English-language search terms in the title: “Rolandic epilepsy” OR “benign childhood epilepsy with centrotemporal spikes” since 2000. We concentrated our review on three main areas: 1. Neuropsychological impairments in children with RE; 2. Influence of epileptic activity on cognitive performance in RE; 3. Effects of antiepileptic drug (AED) therapies in RE. Results The primary search yielded 308 papers. We reviewed the results and removed duplicate articles and all nonoriginal, non-English papers. Finally, after carefully reviewing the full texts, we included 44 original articles to achieve the aims of this review. Conclusion Physicians taking care of children with RE should be aware of the risks for cognitive dysfunctions in these patients and screen their patients for any subtle dysfunction that may affect their academic performance and achievement. If and when the physician decides to prescribe an AED for their patients with RE, LEV is probably a better option compared with CBZ to prescribe for these children.

Objective The Middle Eastern country of Qatar opened its first epilepsy monitoring unit (EMU) in late 2015. This study compared demographic and clinical characteristics of patients with confirmed epilepsy to those of patients with confirmed psychogenic nonepileptic seizures (PNES). Methods Data were collected via retrospective chart review on 113 patients admitted for evaluation to the Qatar national health system EMU between November 2015 and May 2017. Results Seventy-one patients had a confirmed diagnosis (20 had PNES, 46 had epilepsy, 5 had both PNES and epilepsy). Evaluation in 33 patients was inconclusive, and 9 had other medical conditions. Patients with PNES were significantly more likely to be primary Arabic speakers (p = 0.003), and this difference was not explained by education or employment status. The most common referral request in patients with PNES was for recurrent/refractory seizures (p = 0.011), and there was a trend for patients with PNES to have more frequent seizures compared with patients with epilepsy (daily to several per week versus several times a month or less, p = 0.051). Depression was identified in 47% of patients with epilepsy and 65% of patients with PNES, and patients with PNES had higher mean depression scores on the PHQ-9 than patients with epilepsy (p = 0.014). Patients with PNES experienced significantly more fatigue (p = 0.021). Seventy percent of patients with PNES and 50% of patients with epilepsy reported sleep problems. Conclusions The characteristics of patients with epilepsy and PNES at the EMU in Qatar were generally similar to those found worldwide. Patients with PNES more often suffered from frequent depression, sleep problems, and fatigue than those with epilepsy, but these were significant concerns for both groups.

Purpose The aim of this retrospective study was to scrutinize factors that are associated with a delay in making the diagnosis of psychogenic nonepileptic seizures (PNES). Methods In this study, patients with PNES, who were investigated at Shiraz Comprehensive Epilepsy Center, Iran, from 2008 to 2019, were studied. We categorized the patients into the following: 1. those with a definite diagnosis of PNES in less than a year since the onset of their attacks; 2. those with a definite diagnosis of PNES later than 10 years since the onset of their attacks. Results During the study period, 330 patients were recorded. In 98 patients (30%), the diagnosis of PNES was made in less than a year since their seizure onset. In 67 patients (20%), the diagnosis of PNES was made later than 10 years since their seizure onset. Taking antiepileptic drugs (AEDs) (odds ratio (OR) = 6) and a history of ictal injury (OR = 3.6) had a positive association, and age at the onset (OR = 0.8) had an inverse association with a delay in receiving a definite diagnosis of PNES (p = 0.0001). Conclusion Some demographic variables (i.e., early age at the onset of seizures), patients' clinical variables (i.e., severe seizure manifestations such as ictal injury), and finally, some physician-related variables (i.e., prescribing AEDs) have significant associations with a delay in making a definite diagnosis of PNES.

We applied the Two-Step cluster analysis on a large cohort of patients with functional seizures (FS). We studied whether the background risk factors differed between the patient clusters.All patients with a diagnosis of FS were studied at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Shiraz, Iran, from 2008 until 2021. The Two-Step cluster analysis was applied considering the age at onset, sex, and seizure semiology. We also studied whether the background risk factors (e.g., a history of sexual abuse, physical abuse, etc.) differed between these patient clusters.Three-hundred and fifty four patients were studied. The Two-Step cluster analysis was applied to the 230 patients who reported any associated risk factors; there were three clusters of patients. The most prominent feature of cluster 1 included akinetic seizures. The most prominent features of cluster 2 included motor seizures and no ictal injury. The most prominent features of cluster 3 included motor seizures with ictal injury. Compared with patients in cluster 3, a history of sexual abuse was more often reported by patients in cluster 1 (OR: 3.26, 95%CI: 1.12-9.47; p = 0.03). Compared with patients in cluster 3, a history of physical abuse was less often reported by patients in cluster 2 (OR: 0.45, 95%CI: 0.22-0.90; p = 0.026).The Two-Step cluster analysis could identify three distinct clusters of patients based on their demographic and clinical characteristics. These clusters had correlations with the associated risk factors in patients with FS.

Objective To characterize the effective connectivity (EC) between the emotion and motor brain regions in patients with psychogenic nonepileptic seizures (PNES), based on resting-state spectral dynamic causal modeling (spDCM). Methods Twenty-three patients with PNES and twenty-five healthy control (HC) subjects underwent resting-state fMRI scanning. The coupling parameters indicating the causal interactions between eight brain regions associated with emotion, executive control, and motion were estimated for both groups, using resting-state fMRI spDCM. Results Compared to the HC subjects, in patients with PNES: (i) the left insula (INS) and left and right inferior frontal gyri (IFG) are more inhibited by the amygdala (AMYG), anterior cingulate cortex (ACC), and precentral gyrus (PCG); (ii) the left AMYG has greater inhibitory effects on the INS, IFG, dorsolateral prefrontal cortex (DLPFC), PCG, and supplementary motor area (SMA); (iii) the left ACC has more inhibitory effects on the INS and IFG; (iv) the right ACC is more inhibited by the INS and IFG, and has a less inhibitory effect on the SMA and PCG; and (v) the left caudate (CAU) had increased inhibitory effects on the AMYG and IFG and a more excitatory effect on the SMA. Conclusion Our results suggest that in patients with PNES, the emotion-processing regions have inhibitory effects on the executive control areas and motor regions. Our findings may provide further insight into the influence of emotional arousal on functional movements and the underlying mechanisms of involuntary movements during functional seizures. Furthermore, they may suggest that emotion regulation through cognitive behavioral psychotherapies can be a potentially effective treatment modality.

The history of epilepsy and its treatment goes back to ancient times when it included medicinal herbs, lifestyle modifications, and even surgery. Trepanation is considered the oldest surgical procedure for the treatment of epilepsy. The first series of temporal lobectomies for the treatment of drug-resistant epilepsy were reported by Penfield and Flanigin (1950). During the years since then, neuroimaging and other technologies have had remarkable and revolutionary progress. This progress has resulted in tremendous advancements in understanding the underlying causes and pathophysiology of epilepsies. With the help of these technologies and advancements, we may now offer surgery as a safer therapeutic option to more patients who are suffering from drug-resistant temporal lobe epilepsy. However, the degree of improvement in surgery outcome has not been proportionate to the technological progress.

Objective We performed a follow-up study of patients with COVID-19 presenting with seizures. Methods All consecutive patients with seizures, who were referred to Namazee Hospital, Shiraz, Iran, with a diagnosis of COVID-19, from 10 August 2020 until 20 October 2020 were included in this longitudinal study. The clinical data were collected by the admitting physician. In a follow-up phone call to the discharged patients (after eight weeks or more), we inquired their seizure outcome. Results In total, 32 patients were studied; 28 patients were followed. Twelve patients (37.5%) presented with a single tonic-clonic seizure and nine (28.1%) had convulsive status epilepticus; one patient had functional (psychogenic) seizures. Ten patients (31.3%) had pre-existing epilepsy, eight others (25%) had pre-existing CNS problems (without epilepsy), one person (3.1%) had pre-existing functional seizures, and 13 individuals (40.1%) neither had epilepsy nor had other CNS problems. Eight patients (28.6%) reported experiencing seizure(s) after being discharged from the hospital; six of these had pre-existing epilepsy and one had pre-existing functional seizures. One patient, who had a newly developed ischemic brain infarction, reported experiencing recurrent seizures. Conclusion Seizures in patients with COVID-19 are either acute symptomatic (in about two-thirds) or an exacerbation of a pre-existing epilepsy/functional seizures (in about one-third). A thorough investigation of the underlying etiology of seizures in patients with COVID-19 is necessary. Seizure outcome in patients, who are hospitalized with COVID-19 and seizures, is generally good.

Objectives We investigated the rate of driving in patients with seizures [i.e., epilepsy or functional seizures (FS)]. We also investigated the factors that may be associated with NOT driving a motor vehicle. Methods This was a retrospective study of an electronic database of patients with seizures that has been built prospectively over more than a decade. All patients, 20 years of age or older, with a diagnosis of idiopathic generalized epilepsy (IGE), focal epilepsy, or FS were studied at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Shiraz, Iran, from 2008 until 2020. Results One thousand four hundred and ninety-two patients were studied (918 patients with focal epilepsy, 338 people with IGE, and 236 individuals with FS). In total, 387 patients (25.9%) reported driving a motor vehicle. Driving rate in patients with IGE was 26.9%, in people with focal epilepsy was 27.0%, and in individuals with FS was 20.3%; the difference was not significant (p = 0.10). Female sex and a younger age at the onset of seizures were significantly associated with not driving a motor vehicle. Being married and having any education were significantly inversely associated with not driving a motor vehicle. Conclusion Most patients with seizures (either people with epilepsy or those with FS alike) do not drive a motor vehicle in their routine daily lives. Demographic factors (e.g., sex, marital status, and education) have significant associations with not driving a motor vehicle.

The human, as a biological system, is an open system embedded within larger systems —including the family, culture, and socio-political environment. In this context, a patient with functional seizures (FS) is embedded in relationships, educational/professional institutions, culture, and society. Both connection to these broader systems and the quality of these connections, as well as the soundness of each system in and of itself, influence the health and well-being of patients in positive or negative ways. The social aspects of life are important determinants of health and quality of life across the lifespan. The current narrative review brings out several overarching themes in patients with FS. Sections on attachment, marriage, social networking, and stigma highlight the central roles of supportive and affirmative relationships across the lifespan. The section on education underscores the importance of keeping children and youth with FS connected within their school environments, as well as managing any barriers – learning difficulties, school response to FS events, stigma, etc.—that can diminish this connection. Finally, the sections on employment and driving highlight the value of being an active participant in one’s society. In summary, FS impacts patients across most social aspects of life domains regardless of age — factors that are important when developing biopsychosocial formulations. This review concludes that the multidisciplinary management of FS requires careful assessment of social aspects of life in patients which can then be targeted for treatment, to improve their quality of life, facilitating recovery, and reducing the risk of relapse.

Purpose To investigate the opinions of physicians on the use of complementary and alternative medicine (CAM) in patients with epilepsy (PWE) worldwide. Methods Online survey addressed to neurologists and psychiatrists from different countries. Results Totally, 1112 physicians from 25 countries (different world region: Europe, North America, South America, Middle-East, Africa, Former Soviet Union Republics) participated; 804 (72.3%) believed that CAM might be helpful in PWE. The most commonly endorsed CAM included meditation (41%) and yoga (39%). Female sex, psychiatry specialization, and working in North and South America were associated with the belief that CAM is helpful in PWE. Two-hundred and forty five out of 1098 participants (22.3%) used/prescribed CAM to PWE; among them, 174 (71%) people perceived CAM to be less effective and 114 (46.5%) people found CAM to be safer than conventional antiseizure medications (ASMs). The most common reasons to prescribe CAM for PWE were: to satisfy the patient (49.9%), dissatisfaction with the efficacy (35.6%), and dissatisfaction with the adverse effects (31.2%) of conventional therapies. Conclusion Although the evidence supporting the use of CAM for the treatment of epilepsy is extremely sparse, most physicians worldwide believe that it could be integrated with the use of conventional ASMs, at least in some patients. High-quality controlled trials are warranted to provide robust evidence on the usefulness of CAM options in PWE.

Objective The aim of the current study was to compare the risk and also the types of ictal injuries in three groups of people with seizures [i.e., IGE vs. TLE vs. FS]. Methods This was a retrospective study. All patients with an electro-clinical diagnosis of IGE, TLE, or FS were recruited at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Shiraz, Iran, from 2008 until 2020. Age, sex, age at seizure onset, seizure type(s), and occurrence of ictal injury at any time since the onset of the seizures and its characteristics were registered routinely for all patients at the time of the first visit. Results One thousand and one hundred seventy-four patients were studied (481 patients with IGE, 402 people with TLE, and 291 persons with FS). While the groups differed in their demographic and clinical characteristics, the rates of ictal injury did not differ significantly between the groups. Tongue injury was more frequently reported by patients with TLE compared with that by people with IGE or FS. Other types/locations of ictal injury were more or less reported by all three groups of the patients. Conclusion Ictal injuries may happen with more or less similar rates among people with epilepsy (IGE and TLE) and those with FS. Ictal injury (rate, type, or location) should not be used as a marker for any specific diagnosis among people with seizures.

Objective The aim of the current study was to investigate the rates of contracting COVID-19 in various populations to provide evidence on the susceptibility of patients with epilepsy (PWE) to contracting symptomatic COVID-19. Methods We surveyed a random sample of three groups of people: patients with epilepsy, people with psychiatric problems, and a group of the general population. The survey included four general questions (age, sex, education, and medical/psychiatric problem) and four COVID-19 specific questions (contracting COVID-19, relatives with COVID-19, wearing a face mask, and frequent hand washings). Results Three hundred and fifty -eight people were surveyed (108 healthy individuals, 154 patients with epilepsy, and 96 patients with psychiatric problems). Thirty-eight (11%) people had a history of COVID-19 contraction. The only factor that had a significant association with COVID-19 contraction was a relative with COVID-19 (Odds Ratio: 5.82; 95% Confidence Interval: 2.85–11.86; p = 0.0001). Having epilepsy did not increase the risk of COVID-19 contraction. Conclusion Symptomatic COVID-19 does not seem to be more likely in PWE. The single most important factor associated with contracting COVID-19 is a close relative with this infection. Isolation of people with SARS-CoV-2 infection and observation of their close contacts may reduce the risk of secondary infections.

Objectives We investigated the clinical features associated with olfactory auras in patients with drug-resistant epilepsy and also hypothesized that this type of aura may predict worse postsurgical outcome in patients with drug-resistant temporal lobe epilepsy (TLE). Methods In this retrospective analysis, data from all patients with drug-resistant epilepsy who underwent epilepsy surgery were reviewed. Patients were prospectively registered in a database from 1986 through 2016. We assessed outcome in the first 5 years after surgery to produce a Kaplan–Meier estimate of seizure recurrence. Post-surgical outcome was classified into two groups; 1) seizure-free, with or without auras; or 2) relapse of complex partial or secondarily generalized seizures. We also investigated the clinical features of patients with TLE and olfactory auras compared with those without olfactory auras. Results We studied 1186 patients. Thirty-seven patients (3.1%) reported olfactory auras with their seizures. Thirty-two patients had temporal lobe surgery. Intracranial video-EEG recording was performed in four patients. Three patients with lateral temporal neocortical seizures reported olfactory auras with their seizures; two of them were seizure-free after surgery. There were no significant clinical differences between patients with TLE and olfactory auras compared with those without. Seizure outcome after surgery was not significantly different between these two groups (p = 0.3; Cox–Mantel test). Conclusion The rarity of olfactory auras makes it difficult to propose new diagnostic and treatment strategies. A multicenter approach, which can enroll more patients, is needed to devise better therapies for patients with drug-resistant epilepsy and this symptom.

Purpose We analyzed a series of patients with ictal pain to estimate its occurrence and characterize the underlying etiologies. Methods We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004–2015) for the occurrence of the term “pain” in the text body. All the extracted reports were reviewed, and patients with at least one documented episode of ictal pain in the epilepsy monitoring unit (EMU) were included in the study. Results During the study period, 5133 patients were investigated in our EMU. Forty-six patients (0.9%) had at least one documented episode of ictal pain. Twenty-four patients (0.5%) had psychogenic nonepileptic seizures (PNES), 10 patients (0.2%) had epilepsy, 11 patients (0.2%) had migraine, and one woman had a cardiac problem. Pain location was in the upper or lower extremities (with or without other locations) in 80% of the patients with epilepsy, 33% of the patients with PNES (p = 0.01), and none of the patients with migraine. Conclusion Ictal pain is a rare finding among patients evaluated in EMUs. Psychogenic nonepileptic seizures are the most common cause, but ictal pain is not specific for this diagnosis. Location of the ictal pain in a limb may help differentiate an epileptic cause from others.

Purpose The aim of the current post hoc study was to investigate factors associated with delay in diagnosis of adult patients with psychogenic nonepileptic seizures (PNES). Methods We retrospectively investigated all patients with PNES admitted to the epilepsy-monitoring unit at the Jefferson Comprehensive Epilepsy Center from 2012 through 2016. We identified the median time to diagnosis of PNES and divided the patients into two groups. We studied factors associated with delay in diagnosis of PNES. Results In all, 49 patients (39 women and 10 men) were studied. Mean age at the time of admission was 40 ± 16 years and at the onset of the seizures was 34 ± 16 years. Disease duration was 5.6 ± 8.2 years. The median for time to diagnosis was 3 years. Patients with early diagnosis (before 3 years after seizure onset) (21 patients) and patients with late diagnosis (delay of 3 years or more from onset) (28 patients) were compared. Only history of head trauma had significant association with the delay in diagnosis: 2 of 19 patients (7%) with an early diagnosis and 11 of 28 patients (39%) with a late diagnosis reported head trauma (P = 0.02). Conclusion Delay in diagnosis of PNES is common, and some factors (e.g., history of head trauma) may contribute to this delay. It is important that physicians involved in the management of seizures appreciate the importance of making an early and definitive diagnosis of PNES.

Purpose To describe a series of patients with ictal coughing to estimate its occurrence and characterize the clinical features and differential diagnoses. Methods We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 7-year period (2010–2016) for the occurrence of the term “cough” in the text body. All the extracted reports were reviewed and patients with at least one documented ictal coughing at the epilepsy monitoring unit (EMU) were included in the study. Results During the study period, 2487 patients were investigated at our EMU. Seven patients (0.28%) had at least one documented seizure accompanied by coughing. Four patients (0.16%) had epilepsy and three patients (0.12%) had psychogenic nonepileptic seizures (PNES). All patients with epilepsy had focal onset epilepsy; ictal coughing was not associated with any particular localization or lateralization. Conclusion We add to the literature on ictal coughing by providing additional information on its differential diagnosis and clinical features. Ictal coughing is a rare finding among patients evaluated at the EMUs. The differential diagnoses for ictal coughing include epilepsy and PNES. Epileptic ictal coughing is a rare semiological finding in patients with epilepsy and when present, it is invariably associated with focal epilepsy.

Purpose To explore various social aspects of life (i.e., employment, education, and driving) in a large sample of patients with functional seizures (FS) living in seven countries from four continents. Methods In this retrospective study, we investigated adult patients with FS, who were admitted to the epilepsy monitoring units at centers in Iran, Qatar, USA, France, Georgia, Egypt, and United Arab Emirates (UAE). We studied the social aspects of life in the whole cohort. Then, we compared the social aspects of life between different world regions. Results Four hundred and forty patients were included (241 from Iran, 56 from Qatar, 52 from France, 41 from the USA, 19 from UAE, 18 from Egypt, and 13 from Georgia). One hundred and twenty six people (30%) had college education, 142 (33%) were employed, and 101 (28%) drove a motor vehicle in their routine daily lives. People with FS and college education were more likely to report a history of sexual abuse compared with those with a lower education. Patients with no loss of responsiveness with their FS were more likely to be employed. Male patients and patients without aura were more likely to drive a motor vehicle in their routine daily lives. None of the social characteristics of the patients with FS showed significant differences among the two large culturally different groups (Muslim nations vs. Christian nations). Conclusion It appears that patients with FS across cultures have significant problems in their social aspects of life.

Education is a human right that plays a key role in social and economic development. Children having active epilepsy may not be properly schooled in ordinary school structures. Students with epilepsy (SWE) are often faced with academic barriers. The lack of information about the current status of special education for SWE obliged the researchers to try to describe the existing reality of the special education practices in the Middle East and North Africa (MENA) region. We disseminated a simple self-administered questionnaire via email to main healthcare professionals involved in epilepsy care in the MENA region, and we also did a literature search on the PubMed, Google Scholar, and Embase engines. We ranked the countries in terms of their educational system. The 1st group contains the most advanced educational system, including specialized schools for SWE. The 2nd group contains an educational system with specialized but less structured schools. The 3rd group includes countries using integrated classes or classes mixing many children with cognitive disabilities. The 4th group includes countries with ordinary classes for SWE, and finally, the 5th group with no schooling for children with epilepsy (CWE). This study indicates that 60% of the countries in the MENA region belong to the 3rd group; there is only one country in each of the first two groups. This work highlights the need to develop structured environments for schooling for SWE in the MENA region.

PURPOSE: The purpose of the study was to review the literature on the terminologies for psychogenic nonepileptic seizures (PNES) and make a proposal on the terminology of this condition. This proposal reflects the authors' own opinions.METHODS: We systematically searched MEDLINE (accessed from PubMed) and EMBASE from inception to October 10, 2019 for articles written in English with a main focus on PNES (with or without discussion of other functional neurological disorders) and which either proposed or discussed the accuracy or appropriateness of PNES terminologies.RESULTS: The search strategy reported above yielded 757 articles; 30 articles were eventually included, which were generally of low quality. "Functional seizures" (FS) appeared to be an acceptable terminology to name this condition from the perspective of patients. In addition, FS is a term that is relatively popular with clinicians.CONCLUSION: From the available evidence, FS meets more of the criteria proposed for an acceptable label than other popular terms in the field. While the term FS is neutral with regard to etiology and pathology (particularly regarding whether psychological or not), other terms such as "dissociative", "conversion", or "psychogenic" seizures are not. In addition, FS can potentially facilitate multidisciplinary (physical and psychological) management more than other terms. Adopting a universally accepted terminology to describe this disorder could standardize our approach to the illness and facilitate communication between healthcare professionals, patients, their families, carers, and the wider public.

Objectives The objectives of this study were 1) to review the literature about “job/employment and psychogenic nonepileptic seizures (PNES)” and 2) to gather the views of neurologists and psychiatrists on the issue of job consultation in patients with PNES. Methods Phase one involved a systematic literature review; phase two involved the collection of the views of neurologists and psychiatrists, practicing in Fars province, Iran, on the issue of job consultation in patients with PNES. A questionnaire was designed for the purpose of this study. Results No studies were identified by the systematic review process on the issue of job consultation in patients with PNES. Of the 87 health-care professionals approached, 63 responded to the survey (response rate: 72%). Respondents included 31 neurologists and 32 psychiatrists. In response to the question “Should all patients with PNES be counseled to pursue any job or profession they would like?”, 17 (55%) neurologists and 19 (59%) psychiatrists answered “no” (p = 0.8). Conclusion While it is possible that some patients with PNES are more likely than other people in the general public to be involved in job-related difficulties (performing duties related to any job, accidents, etc.), there is currently no evidence supporting or refuting this proposition. However, in this study, the majority of the health-care professionals, who often deal with these patients, believed that not all patients with PNES should be counseled to pursue any job or profession they would like (i.e., there should be some restrictions).

We critically review the existing literature about the epidemiology (i.e., diagnosis, occurrence, age, gender, comorbidity with epilepsy, associated factors, prognosis, mortality, and cost) of psychogenic nonepileptic seizures (PNES) and provide suggestions for future research. Psychogenic nonepileptic seizures are commonly diagnosed at epilepsy centers. The diagnosis of PNES relies on a multidisciplinary evaluation and is usually based on different combinations of data. Recording a seizure, while under video-EEG monitoring, is the most reliable diagnostic test. However, not all patients present with seizures while under video-EEG monitoring. Furthermore, not all epileptic seizures produce visible changes in the scalp EEG. The incidence of PNES was estimated to be 1.4–4.9/100,000/year in three previous studies, and the prevalence was calculated to be between 2 to 33 per 100,000 in one study, making it a significant neuropsychiatric condition. However, there remains a scarcity of data about the epidemiology of PNES, and extant studies that assessed the epidemiological characteristics of PNES have significant limitations. For example, inconsistencies with regard to the age of patients studied and lack of standardization of the diagnostic criteria are some of the significant limitations among studies. In conclusion, PNES merit further epidemiological and pathophysiological investigation. A more precise definition and clear guidance on standards for the diagnosis might influence the direction of future research. Well-designed prospective population-based studies to clarify the epidemiology of PNES in various parts of the world, including an evaluation of the predisposing, precipitating, and perpetuating factors in cross-cultural comparisons is required.

Purpose We classified patients with psychogenic nonepileptic seizures (PNESs) according to a newly proposed classification system. Then, we investigated the demographic and clinical differences between various classes of the patients. Methods We retrospectively investigated all patients with PNESs admitted to the Jefferson Comprehensive Epilepsy Center from 2012 through 2016. We classified the patients into four distinct classes: patients with generalized motor seizures, patients with akinetic seizures, patients with focal motor seizures, and patients with seizures with subjective symptoms. All patients were interviewed by a neuropsychologist and were administered psychological assessment measures, including questions about PNES risk factors. For the statistical analyses, we compared patients who had generalized motor seizures with patients who had nonmotor seizures. Results Sixty-three patients were studied. Thirty-five (55.6%) patients had generalized motor seizures, 14 (22.2%) had seizures with subjective symptoms, 12 (19%) had akinetic seizures, and two (3.2%) patients had focal motor seizures. Patients with generalized motor seizures (35 patients) demonstrated a trend for later age at onset (p = 0.06), more frequently had a history of substance abuse (p = 0.001), and more often had loss of responsiveness with their seizures (p = 0.04) compared with patients who had nonmotor seizures (26 patients). Conclusion The recently proposed PNES classification system is useful and practical. This proposed classification of PNESs may address proper diagnosis and provide standardization across future studies. This may also potentially shed light on the etiologic understanding and management of various classes of patients affected with PNESs.

Purpose Ictal verbal help-seeking has never been systematically studied before. In this study, we evaluated a series of patients with ictal verbal help-seeking to characterize its frequency and underlying etiology. Methods We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004–2015) for the occurrence of the term “help” in the text body. All the extracted reports were reviewed and patients with at least one episode of documented ictal verbal help-seeking in epilepsy monitoring unit (EMU) were studied. For each patient, the data were reviewed from the electronic medical records, EMU report, and neuroimaging records. Results During the study period, 5133 patients were investigated in our EMU. Twelve patients (0.23%) had at least one episode of documented ictal verbal help-seeking. Nine patients (six women and three men) had epilepsy and three patients (two women and one man) had psychogenic nonepileptic seizures (PNES). Seven out of nine patients with epilepsy had temporal lobe epilepsy; six patients had right temporal lobe epilepsy. Conclusion Ictal verbal help-seeking is a rare finding among patients evaluated in epilepsy monitoring units. Ictal verbal help-seeking may suggest that seizures arise in or propagate to the right temporal lobe.

The purpose of this study was to describe a series of patients with ictal crying to estimate its occurrence and characterize the clinical features and the underlying etiology.We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004-2015) for the occurrence of the terms "cry" or "sob" or "weep" in the text body. All the extracted reports were reviewed, and patients with at least one episode of documented ictal crying at the epilepsy monitoring unit (EMU) were included in the study.During the study period, 5133 patients were investigated at our EMU. Thirty-two patients (0.6%) had at least one documented seizure accompanied by crying. Twenty-seven patients (26 women and one man) had psychogenic nonepileptic seizures (PNES), and five patients (0.1%) had epilepsy. Among patients with epileptic ictal crying, four patients had focal epilepsy (two had definite, and two had probable frontal lobe epilepsy), while one patient had Lennox-Gastaut syndrome.Ictal crying is a rare finding among patients evaluated at the EMUs. The most common underlying etiology for ictal crying is PNES. However, ictal crying is not a specific sign for PNES. Epileptic ictal crying is often a rare type of partial seizure in patients with focal epilepsy. Dacrystic seizures do not provide clinical value in predicting localization of the epileptogenic zone.

Purpose Previous studies from a few countries have reported semiological differences in younger children compared with adolescents or adults with psychogenic nonepileptic seizures (PNESs). This study tested the hypothesis that semiological, demographic, and historical risk factors vary with different ages of PNES onset in a large cohort from different countries. Methods In this retrospective study, we investigated patients consecutively referred for PNES, who were admitted to epilepsy monitoring units in Iran, Brazil, Venezuela, Canada, Argentina, and USA. Age, gender, age at seizure onset, seizure semiology, and factors predisposing to PNES (abuse, stressors) were documented according to routine diagnostic practices at each center. Participants were grouped according to their age at onset (i.e., childhood, adolescence, or adulthood). Results A total of 448 patients were studied. Female predominance was associated with adolescent- (85/122, 70%) and adult-onset (190/270, 70%) but not in childhood-onset PNES (28/56, 50%) (p = 0.011). Event frequency in the month preceding the diagnosis was higher in the childhood- [ x ¯ = 50, standard deviation (sd) = 82, p = 0.025] versus adolescent- ( x ¯ = 24, sd = 36) or adult-onset groups ( x ¯ = 29, sd = 61). Significant between-group differences were observed for generalized body movements (p = 0.0001) and ictal injury (p = 0.027), suggesting more severe ictal presentations in adult-onset PNES compared with younger ages. Adult-onset patients were also more likely to be taking an unnecessary antiepileptic medication (p = 0.010). Conclusion While PNES may present at any age, there appear to be notable differences across the lifespan with respect to some of the clinical characteristics. Further international and cross-cultural studies may reveal other interesting characteristics of PNES.

Purpose A semiological classification of psychogenic nonepileptic seizures (PNES) may help address proper diagnosis and management and also provide standardization for future studies. The aim of the current paper was to systematically review the literature on the proposed classification systems for the semiology of PNES and to provide a new proposal based on the best available evidence. Methods I searched the electronic database PubMed on May 16, 2019 for articles that included the following search terms: “psychogenic” AND “semiology” or “classification” and also “nonepileptic” AND “semiology” or “classification” and also “pseudoseizure” AND “semiology” or “classification” since 1940. I applied the same methodology using the electronic database Scopus, though I limited the search to the title, abstract, and keywords. Results I could identify 15 classification systems through this search strategy using the electronic database PubMed. Searching the Scopus did not yield any additional relevant papers. Conclusion I proposed a new semiological classification system for PNES based on this systematic review. This includes three major classes of motor seizures, nonmotor seizures, and mixed semiology. A universally accepted and appropriate semiological classification system for PNES may lead to better standardization of future studies and may also help in better understanding of the pathophysiological basis of this condition.

Objective The present study aimed to determine if adjunctive use of verapamil, as a P-glycoprotein (P-gp) inhibitor, is efficacious in decreasing seizure frequency in patients with refractory temporal lobe epilepsy. Materials and methods This was an open-label pilot study. Adult patients with refractory temporal lobe epilepsy were studied. Baseline seizure type and seizure count were determined. Patients were divided randomly into two groups. Group A received verapamil 120 mg/day (n = 13), and group B received 240 mg/day (n = 6). All patients were followed for eight weeks. The proportion of responders, which consist of patients with more than 50% reduction in seizure frequency from baseline, was tabulated. Results Nineteen patients were studied. Seven patients (36.84%) reached the responder rate. Three patients (50%) in group B were among the responders; two of these patients achieved seizure freedom. Four patients (30.7%) in group A responded favorably to verapamil. Conclusion Developing new means of improving the effectiveness of existing antiepileptic drugs is a desirable way of tackling the dilemma of medically refractory epilepsy. Hypothetically, P-gp inhibitors (e.g., verapamil) might be used to counteract the removal of AEDs from the epileptogenic tissue. Such a strategy was adopted in this non-placebo-controlled, open-label, pilot study. We observed a significant achievement in seizure control associated with adjunctive use of verapamil in patients with refractory temporal lobe epilepsy.

Corpus callosotomy is a palliative surgical procedure that is suitable for some patients with intractable seizures who are not candidates for focal resective surgery. The rationale for this procedure is based on the hypothesis that the corpus callosum is a critical pathway for interhemispheric spread of epileptic activity. Efficacy and relatively low permanent morbidity in corpus callosotomy for medically intractable epilepsy have been demonstrated by more than six decades of experience. Callosotomy best ameliorates drop attacks (tonic and atonic seizures), though tonic-clonic, absence, and frontal lobe complex partial seizures often respond as well. In addition to seizure reduction, behavior and quality of life may improve. Hence, callosotomy is justified as a therapy for appropriate patients with intractable epilepsy.

Objective A person’s health locus of control orientation is one of several factors that determine which health-related behaviors a person will perform. The aim of this study was to determine the health locus of control in patients with epilepsy and its relationship to anxiety, depression, and seizure control. Methods Adults aged 18 and older who had had epilepsy for at least 1 year were recruited in either the inpatient epilepsy monitoring unit or the outpatient epilepsy clinic at Thomas Jefferson University in 2006. Patients anonymously filled out a questionnaire, which elicited data on age, sex, education, and seizure control. The Hospital Anxiety and Depression (HAD) scale was used to evaluate anxiety level and depression, and Form C of the Multidimensional Health Locus of Control (MHLC) scales was used to evaluate the health locus of control. Statistical analyses were performed using regression analyses to determine potentially significant associations. Results Two hundred patients with a mean age of 40.3 ± 16 participated. Patients had low mean scores on the Internal, medium mean scores on the Chance, and high mean scores on the Powerful Others MHLC subscales. Patients with epilepsy with higher Internal MHLC scores more frequently had controlled seizures. Patients with higher Powerful Others MHLC scores had higher scores on the Anxiety subscale of the HAD scale. Conclusions Patients with epilepsy in our study had weak perceptions of internal and strong perceptions of external health locus of control. This probably means patients with epilepsy might adapt less effectively to their illness and have lower levels of engagement in beneficial health behaviors and active coping strategies.

Objectives It has been reported that a common belief of the families of the patients with epilepsy in Iran is that foods provoke seizures. Our aim in the present study was to ascertain whether a culturally different population of patients with epilepsy in the United States believe that foods precipitate seizures. Methods Adults aged 18 and older with epilepsy were recruited in either the inpatient epilepsy monitoring unit or the outpatient epilepsy clinic at Thomas Jefferson University from September to December 2006. Patients completed a questionnaire asking their age, sex, education, seizure control, and beliefs about the relationship between foods and seizures. Results One hundred ninety-three patients participated, with a mean age of 40.3 ± 16. Only 11 (5.7%) patients reported foods as a precipitating factor for seizures. The difference between the results of the Iranian study, in which 55.2% of the families of children with epilepsy reported a relationship between specific foods and seizures, and the present results is significant (P = 0.0001). Conclusion The perception of foods as a seizure precipitant differs greatly between a Middle Eastern country (Iran) and a Western country (United States). This discordance suggests that cultural factors may play a large role in the perception of probable precipitating factors related to seizures. Some commonly reported seizure precipitants may represent cultural beliefs, and this raises a question as to whether foods truly precipitate seizures.

Purpose Status epilepticus (SE) is a medical emergency with significant associated mortality and morbidity. This study was conducted to determine the etiology and outcome of SE in children. Methods All patients from 1 month to 15 years old with SE admitted to Nemazee Hospital, Shiraz, Iran, the main pediatric hospital in the area, between 1999 and 2004, were studied in a retrospective cross-sectional study. Outcomes were rated according to the Glasgow Outcome Score (GOS). Results In all, 135 patients (76 boys, 59 girls) were studied. The most common cause of SE was fever. Etiology of SE did not differ significantly between various age groups and also between boys and girls ( P = 0.736 and P = 0.156, respectively). Fourteen patients died in hospital and 81 were discharged in good health. Outcome did not differ significantly between the age groups ( P = 0.695) or between boys and girls ( P = 0.386). When classified as good (GOS = 5) and bad (GOS = 1–4), outcome was significantly correlated with etiology of SE ( P = 0.017). Prolonged febrile seizures had the best, and symptomatic SE the worst, outcome. Discussion In this study, even in children above 3 years of age, fever was the most common cause of SE. This is noteworthy and heralds the need to pay more attention to febrile seizures, especially in children at risk for their occurrence. Outcome was not related to age and sex, but etiology of SE significantly influenced outcome.

Background . Risk factors for epilepsy are conditions associated with an increased frequency of epilepsy, and those for childhood epilepsy are different from those for epilepsy occurring later in life. In the present matched case–control study, we attempted to identify some possible risk factors for childhood epilepsy. Methods . All children with epilepsy who attended the clinic of Motahary in Shiraz, Iran during a 6-month period were included in the study. Neurologically normal children, matched for age and sex, visiting the same clinic were considered as controls. The data on patients and controls were obtained from answers to a questionnaire obtained through personal interviews. Details on the patient, family history, parental smoking, breast versus formula feeding, and parental age at the time of childbirth were included. Medical records were then reviewed. Results . In total, 142 patients and 138 controls participated. Positive family history of epilepsy increased the risk of developing epilepsy by 3.34-fold. A higher risk of epilepsy was observed in patients living in rural areas (OR = 2.44). Conclusions . Positive family history of epilepsy and residence in rural areas were the two major risk factors associated with epilepsy in this study; neonatal jaundice, parental age, and passive smoke exposure added no risk. Breast-feeding was not shown to have a protective effect against development of epilepsy.

Purpose The aim of the current study was to clarify the impact of co-existing epileptic seizures (either in patients themselves or in their family members) on the demographic and clinical characteristics and manifestations of psychogenic non-epileptic seizures (PNES). Methods In this prospective study, all patients with a definite diagnosis of PNES were recruited at the epilepsy center at Shiraz University of Medical Sciences from September 2008 through May 2012. We subdivided the patients into three groups: those with PNES but without either epilepsy or a family history of epilepsy (group 1); those with PNES and concomitant epilepsy but without a family history of epilepsy (group 2); and those with PNES and a family history of epilepsy but without concomitant epilepsy (group 3). We studied the demographic and clinical findings and associated risk factors among these three groups. Results One hundred and eighty-eight patients had confirmed diagnosis of PNES at our center, of whom 32 patients (17%) had confirmed epilepsy. In total, 176 patients met our inclusion criteria and were studied: 103 patients in group 1, 19 patients in group 2, and 54 patients in group 3. Demographic characteristics, seizure characteristics, and semiology in these three groups of patients with PNES were similar. They reported similar associated factors for PNES. Conclusion Epilepsy is relatively common among patients with PNES. The demographic and clinical characteristics of patients with PNES are similar in those with or without co-existing epilepsy (in themselves or among their family members).

Purpose Almost 1000 years ago, Avicenna, the great Iranian scientist, recommended that patients with epilepsy should avoid some foods. In the present study we attempted to identify beliefs and experiences of families with epileptic children with respect to the need for specific dietary restrictions in these patients. Methods A cross-sectional study was carried out using a questionnaire at the outpatient clinic of Motahary in Shiraz, Iran, in 2004. Patients with unprovoked seizures and abnormal electroencephalograms who were under follow-up for at least 2 months were included in the study. Results In total 125 families participated. Of these, 55.2% believed there was a relationship between specific foods and the occurrence of seizures. Personal experience with seizures after consumption of specific foods was reported by 31.2% of the families. Dairy products, sour foods, fruits, and vegetables were the most common foods reported to be responsible. Conclusion It was clearly observed that parents desire to know if their children with epilepsy require dietary restrictions and, hence, try to collect information from different and sometimes unreliable sources. It would be invaluable to consider the experience of families caring for epileptic children when evaluating the relationship between consumption of specific foods and seizures in future studies. This relationship may be due to a food–disease or food–drug interaction.

Familial hemiplegic migraine (FHM) is a clinically and genetically heterogeneous disease most commonly linked to CACNA1A gene mutation. Epilepsy rarely occurs in FHM and is seen predominantly with specific CACNA1A gene mutations. Here we report a sporadic case of FHM1 linked to S218L CACNA1A gene mutation with the triad of prolonged hemiplegic migraine, cerebellar symptoms, and epileptic seizures. Epilepsy in this syndrome follows the pattern of isolated unprovoked seizures occurring only during childhood and hemiplegic migraine-provoked seizures occurring during adulthood. Clinical and electrographic status epilepticus can occur during prolonged migraine attacks. We suggest that patients with seizures, ataxia, and hemiplegic migraine be genetically tested for FHM. Patients with prolonged hemiplegic migraine attacks and confusion should be tested with continuous EEG recording to ascertain whether electrographic status is occurring, as intensive antiepileptic treatment not only resolves status but immediately stops hemiplegic migraine and improves associated neurological deficits.

Objective Distinguishing epileptic generalized tonic–clonic seizures (GTCS) from either psychogenic nonepileptic seizures (PNES) or vasovagal syncope (VVS) is important. In this study, we investigated the use of postictal serum creatine phosphokinase (CPK) concentrations in distinguishing between these events. Methods Patients admitted to the Neurology Ward at Namazi Hospital in Shiraz, Iran, with an attack of transient loss of consciousness and abnormal movements witnessed by a neurologist were studied. We categorized the patients into three groups: 20 patients with GTCS, 22 with VVS, and 20 with PNES. A group of 20 normal healthy individuals were included in the study as the control group. Serum CPK concentration was measured 12–15 h after the attack in all patients and at one time in the control group. A P value less than 5% was considered significant. Results There were no significant differences between the four groups with respect to age and sex. Mean CPK concentrations statistically significantly differed between the four groups, with higher levels in patients with GTCS ( P = 0.0001). Serum CPK concentration had a sensitivity of 75% and specificity of 86% for the diagnosis of GTCS. CPK concentration was above 160 mg/dl in 75% of patients with GTCS, 15% of patients with PNES, 13.6% of patients with VVS, and 15% of the control group ( P = 0.0001). The PNES, VVS, and control groups did not statistically significantly differ with respect to CPK concentrations. Conclusion In patients with a recent loss of consciousness and abnormal movements, serum CPK concentration is a useful, practical, and relatively accurate parameter to assist in the differentiation of epileptic seizures from either VVS or PNES.