Ictal Coprolalia: Three Cases with Nondominat Frontal Lobe Involvement and Review of the Literature.

Objective: Coprolalia is defined as the involuntary use of obscene, socially unacceptable, and derogatory words. Ictal coprolalia is a rare presentation of epilepsy. This study aimed to determine the localizing and lateralizing value and frequency of ictal coprolalia in epilepsy patients. Methods: Medical files, discharge summaries, and electroencephalography (EEG) reports of 2238 patients were reviewed retrospectively. We identified patients who suffered from ictal coprolalia. Electroencephalography reports, neuroimaging [brain magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG-PET), single-photon emission computerized tomography (SPECT)] records, F-18 FDG fused on MRI images, and ictal SPECT fused on MRI images were evaluated. Also, original and review articles were identified through a systematic search of Pubmed, Scopus, and Clarivate Analytics. Results: Ictal coprolalia was detected in 3 male (0.15%) patients. In all patients, ictal semiology was extratemporal-frontal type, and potential/proven epileptic focus was non-dominant hemisphere frontal lobe. Topectomy was done in one of the patients, including the suspected dysplastic area plus the area where the electroencephalographic ictal and interictal changes occur, on the left frontal lobe, and the patient had an Engel's classification class IIA. The data depending on the published cases showed that ictal coprolalia was dominant in the male gender and the responsible epileptic area tended to be located in the non-dominant hemisphere frontotemporal region. Conclusion: The rate of ictal coprolalia in the Turkish population is lower compared to other series. Our results are consistent with previous studies in which reported that male preponderance for ictal coprolalia and involvement of non-dominant frontal lobe.

Ictal Asystole During Focal Seizures Due to Left Occipital Glioneuronal Tumor: A Report of Case Treated With Cardiac Neuromodulation.

Ictal asystole (IA) is a rare but potentially life-threatening complication of focal epilepsy. The sudden onset of loss of consciousness and drop attacks in a patient with chronic epilepsy should suggest the possibility of this complication. Once the diagnosis is established, rapid management should be considered, especially in high-risk cases. The approach does not differ between temporal and extratemporal lobe epilepsies. Strategies can be aimed at preventing the emergence of cortical epileptic activity from the beginning (surgery, antiseizure therapy), neutralizing negative chronotropic effects on the heart (cardiac neuromodulation), or restarting the heart rhythm with a pacemaker. Pacemaker implantation is not a completely complication-free treatment, and living with a device that requires care and follow-up throughout life makes alternative treatment methods more valid for young patients with many years to live or cases that could benefit from surgery. In this article, we present a patient with a left occipital glioneuronal tumor and drug-resistant occipital lobe epilepsy. IA was documented by long-term video EEG monitoring (VEM). During about 2 years of follow-up after a cardiac neuromodulation procedure, there were no drop attacks or asystole with seizures, confirmed by long-term VEM.

Ictal crying in epileptic seizures and psychogenic nonepileptic seizures: What are the hints to differentiate them?

OBJECTIVES: Ictal crying (IC) is a quite rare semiological manifestation of epileptic seizures (ESs) and it has been mostly reported in psychogenic nonepileptic seizures (PNESs). However, labeling IC as a pathognomonic sign of PNES can be harmful. We first aimed to investigate IC frequency in ES and PNES and highlight the differences of IC between ES and PNES. Secondly, we aimed to analyze etiology, detailed semiology, treatment options, and outcome of patients with IC in ES in more detail. METHODS: We retrospectively screened all video-EEG monitoring unit reports from Hacettepe University Hospitals' Epilepsy Center over a 20-year period (1996-2017) for the diagnosis of IC. We included the patients with IC who had at least one documented seizure. Patients who had IC with both facial expression and vocalization compatible with crying with or without weeping and subjective feeling of sadness, were included in the study. We classified patients with IC as ES and PNES. Demographic, historical, clinical, neuroimaging, electrophysiological parameters, video-EEG data, treatment options, and prognosis of all patients were recorded. Demographic, clinical, and video-EEG data were compared between ES and PNES. RESULTS: During the study period, 1983 patients were investigated. Six patients (all female) with ES and 37 patients (33 female) with PNES were identified. When we compared patients with PNES and ES with IC, the number of ASMs taken and duration of disease were significantly higher in patients with ES than PNES. Longer duration of seizure, longer duration of crying component, late onset of crying component in seizure, early responsiveness after seizure, not occurring during sleep, accompanied by eye closure and weeping, were found significantly higher in patients with PNES. Besides, if we analyze ES group in more detail, all had medical treatment refractory focal epilepsy and two of them whose IC was seen as an early semiological manifestation of their seizures had good outcome after nondominant anterior temporal lobectomy (ATL)+amygdalohippocampectomy (AH). However, three patients had various cortical lesions apart from temporal lobe on MRI and one patient had focal epilepsy with frontal lobe semiology with negative MRI. CONCLUSION: Although the most common etiology for IC is PNES and it is rarely seen in ES, it can be harmful to label ictal crying as a pathognomonic sign for PNES. We proposed that there are some semiological differences in terms of IC between PNES and ES. These differences may help to distinguish IC in PNES and ES in daily practice. Moreover, it can be speculated that nondominant temporal lobe involvement may be associated with IC in ES.

Electroclinical Presentations of Fixation-off Sensitivity in Adults With Symptomatic Epilepsy.

PURPOSE: Fixation-off sensitivity (FOS) is a discharge pattern on EEG that occurs owing to the loss of central vision or fixation. Knowledge regarding the relationship between FOS and symptomatic epilepsy is limited. Therefore, we aimed to evaluate the electroclinical features of FOS in adult patients with symptomatic epilepsy. METHODS: Outpatient video-EEG records of the Hacettepe University Faculty of Medicine were reviewed from 2009 to 2019. Patients aged >18 years with symptomatic epilepsy with a FOS pattern were included. Demographic, clinical, EEG, and neuroimaging data were retrospectively evaluated from an electronic database and patient files. RESULTS: Eight patients (50% female) were included in this study; seven (87%) had refractory epilepsy. Prominent risk factors were family history of epilepsy in five patients and prenatal/natal insult in four patients. Notable MRI signs included cortical developmental malformation, posterior gliosis, and frontoparietal porencephalic cyst. The FOS pattern was generalized with posterior emphasis in two patients and lateralized or localized in six patients: frontocentroparietal ( n = 1) and temporoparietooccipital ( n = 5). Fixation-off sensitivity discharges were found to be increased by hyperventilation and decreased by drowsiness and sleep in 50% of patients. Fixation-off sensitivity disappeared in one patient with good seizure control. CONCLUSIONS: In this study, the disappearance of FOS in an epileptic patient with a structural lesion and detection of FOS activity related to a frontoparietal porencephalic cyst were remarkable. Family history of epilepsy was also substantially high. Our results indicate that the underlying mechanism of FOS is much more complicated than previously thought.

Assessment of glymphatic function in narcolepsy using DTI-ALPS index.

INTRODUCTION: Sleep is a modulator of glymphatic activity which is altered in various sleep disorders. Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness (EDS), rapid onset of rapid eye movement (REM) sleep, cataplexy, disturbed night sleep with fragmentation. It is categorized into two types, type 1 (NT1) and type 2 (NT2) depending on the presence of cataplexy and/or absence of orexin. We sought for alterations in glymphatic activity in narcoleptic patients using diffusion tensor imaging (DTI) along perivascular space (ALPS) index on magnetic resonance imaging (MRI). MATERIAL AND METHODS: Adult patients diagnosed with NT1 or NT2 who had polysomnography (PSG) and MRI with DTI were included in the study. Sleep recording included Epworth Sleepiness Scale (ESS) score, sleep latency during multiple sleep latency test (MSLT), sleep efficiency during night PSG, wake after sleep onset (WASO), REM sleep latency during PSG, percentage of non-REM (NREM), REM sleep and wakefulness during night PSG. DTI-ALPS index was calculated for each patient and age-sex matched healthy control(HC)s. RESULTS: The study group was composed of 25 patients [F/M = 15/10, median age = 34 (29.5-44.5)], 14 with NT1 and 11 with NT2 disease. ESS, WASO and percentage of wakefulness were significantly higher in NT1 patients (p < 0.05). Mean DTI-ALPS was not significantly different neither between narcoleptic patients and HCs, nor between NT1 and NT2 patients (all, p > 0.05). However, DTI-ALPS was negatively correlated with WASO (r = -0.745, p = 0.013) and percentage of wakefulness (r = -0.837, p = 0.005) in NT1 patients. DTI-ALPS correlated negatively with percentage of N1 sleep (r = -0.781, p = 0.005) but positively with REM percentage (r = 0.618, p = 0.043) in NT2 patients. CONCLUSION: In this study, DTI-ALPS was not significantly different in narcoleptic patients than the HCs. However, the glymphatic index as assessed by DTI-ALPS correlated with PSG parameters; negatively with WASO, percentage of wakefulness in NT1, percentage of N1 sleep in NT2, and positively with REM sleep in NT2. A tendency for a reduction in DTI-ALPS in NT1 patients compared to both NT2 patients and HCs was also found. These findings might show the first evidence of an alteration of glymphatic activity, especially in NT1 patients, thus warrant further prospective studies in larger size of narcoleptic patient cohorts.

Changes of oxygen saturation in patients with pure temporal lobe epilepsy.

PURPOSE: Ictal hypoxemia is accepted as one of the mechanisms underlying sudden unexpected death in epilepsy (SUDEP). Although ictal hypoxemia is more common in generalized seizures, it also occurs in focal seizures with or without generalization. In this study, we aimed to show the relationship between clinical and electroencephalographic findings of seizures in patients with temporal lobe epilepsy (TLE) with periictal oxygen saturation. METHODS: The data of 55 adult patients who were hospitalized in the Video EEG Monitoring Unit (VEMU) and operated on for drug-resistant TLE between January 2017 and December 2020 were examined. Forty-five seizures from 21 patients with ictal peripheral arterial saturation information and that were seizure-free for at least a year during the follow-up were included in the study. RESULTS: The median patient age was 28 (IQR 25-39.5) years (women: 9, men: 12). Age at epilepsy onset was negatively correlated with saturation at seizure onset. Moreover, the age at VEMU admission was also negatively correlated with saturation at seizure onset and the lowest levels of saturation. The saturation at the end of the seizures and the lowest saturation measured in the periictal period with generalization of EEG were significantly lower than those without generalization. The onset of ictal EEG with the rhythmic theta pattern was significantly associated with the lowest level of saturation (<90%), postictal generalized electroencephalographic suppression (PGES), and the presence of generalization. CONCLUSION: According to the study, rhythmic ictal theta activity, older age, nocturnal seizure, and generalization in ictal EEG might increase the potential risk of SUDEP. Further studies including a greater number of subjects and different epilepsy syndromes may provide more comprehensive information about potential biomarkers for SUDEP.

Relation between orexin A and epileptic seizures.

INTRODUCTION: One of the unknown mechanisms in epilepsy pathogenesis is the involvement of the hypothalamic neuropeptide orexin. Although the relationship between orexin and sleep has been revealed, its effect in epilepsy has not been fully clarified. In this study, we aimed to show the relationship between orexin A and the seizures that occur during sleep and wakefulness. MATERIAL AND METHODS: This study included 40 patients with drug-resistant focal epilepsy and 37 healthy controls. Night basal orexin (NBO) and morning basal orexin (MBO) levels were measured using enzyme-linked immunosorbent assay in patients and controls. Serum samples were collected from patients after epileptic seizures during sleep and wakefulness. RESULTS: In both patients and controls, MBO levels (median: 1039 pg/mL, interquartile ranges [IQR] (899-1078)) were higher than NBO levels (median 989 pq/mL, IQR (893-1078) (p = 0.02). Basal orexin levels were lower in patients than in controls (p < 0.001). However, while the duration of seizures was shortened in awake seizures, the level of orexin increased (p = 0.007). Additionally, orexin levels after nocturnal seizure were higher in patients who had an ictal electroencephalography onset in the left hemisphere or a lesion in the left temporal lobe (p = 0.02; p = 0.01, respectively). There was no relationship between postictal somnolence and orexin levels. Although there was no significant difference, the level of post-seizure orexin increased compared to the basal values, especially in seizures during sleep. DISCUSSION: The increase in serum orexin levels, especially after seizures, suggests that orexin may be associated with the epileptogenic effect. In further studies, determination of orexin from cerebrospinal fluid (CSF) and correlation of CSF and serum orexin levels may provide more useful information regarding the relationship between orexin and epilepsy.

The REM-sleep-related characteristics of narcolepsy: a nation-wide multicenter study in Turkey, the REMCON study.

INTRODUCTION: Narcolepsy type 1 (NT1) is caused by hypocretin deficiency, the pathophysiology of narcolepsy type 2 (NT2) has not been delineated. Except for the hypocretin deficiency and cataplexy, all clinical and laboratory features used in the diagnosis of NT2 are identical to those used for NT1. The aim of this study was to assess the rapid eye movement (REM) sleep-related characteristics in the patients with narcolepsy; the characteristics of REM sleep in polysomnography (PSG) and multiple sleep latency test (MSLT) recordings, the quantification of REM sleep without atonia (RSWA) and atonia index, and the analysis of rapid eye movements (REMs) during REM sleep. MATERIALS AND METHODS: This study was planned by the Sleep Medicine Study Group of the Turkish Neurology Society, and conducted in 11 centers in eight cities in Turkey. The analysis of RSWA was analyzed by reviewing all REM sleep periods on nocturnal PSG and MSLT recordings per standard criteria. The total duration of the increased muscle tone during REM sleep in the chin and bilateral leg electromyography (EMG) recordings was calculated as RSWA index. The REMs index was also investigated the relation to the RSWA. RESULTS: A total of 274 patients were involved; 147 patients (53.6%) were males and 127 patients (46.4%) were females; the mean age was 29.1 ± 12.0 years. The diagnosis of NT1 was made in 166 patients (60.6%), and 108 patients (39.4%) were diagnosed as having NT2. The mean Epworth sleepiness scale score was significantly higher in patients with NT1 than the patients with NT2 (P = 0.001). The diagnosis of REM sleep behavior disorder (RBD) was made in 19.3% of the patients with NT1 versus in 2.8% of the patients with NT2 (P < 0.001). The percentage of SOREMP in PSG recordings was significantly higher in patients with NT1 (37.1%) than those with NT2 (18.9%, P = 0.001). MSLT showed that the mean sleep latency was shorter in patients with NT1 compared to those with NT2 (P < 0.001). The total duration of REMs on electrooculography recordings was also significantly higher in patients with RSWA in comparison with the patients without RSWA (P = 0.002). Total duration of REMs was significantly and positively correlated with the duration of RSWA on chin-EMG and leg-EMG recordings (P = 0.001). ROC analyses showed an RSWA index of ≥2% for the RSWA on chin-EMG with a sensitivity of 86.7% and a specificity of 71.3% (P < 0.001). The REMs index ≥20% was associated with the presence of RSWA with a sensitivity of 70.0% and a specificity of 57.1% (P = 0.008). CONCLUSIONS: In this nation-wide study, we identified for the first time that the increase in REMs density during REM sleep may be a major correlate of the RSWA. Significant positive correlations were demonstrated between the total duration of REMs on electrooculography recordings and the mean durations of RSWA in both chin and leg EMG recordings. A REMs index of >20% was demonstrated to have a moderate sensitivity and specificity in the diagnosis of RSWA. As observed in chin RSWA index, REMs index also showed a significantly high association with RBD, in comparison to RSWA per standard criteria.

Electroclinical spectrum of generalized paroxysmal fast activity in adults without epileptic encephalopathy.

INTRODUCTION: Generalized paroxysmal fast activity (GPFA) is a rare and underreported EEG pattern known to be related to epileptic encephalopathy. We aimed to investigate the electroclinical spectrum of GPFA along with other atypical EEG features in patients without epileptic encephalopathy in routine EEG practice. METHODS: Outpatient EEG records of Hacettepe University Hospital were retrospectively reviewed between 2010 and 2020. Patients ≥ 18 years old with GPFA without epileptic encephalopathy were included. Electroclinical features of GPFA were analyzed. Atypical EEG features, epileptiform K-complexes and sleep spindles, and generalized polyspike train (GPT) were also investigated in this cohort. RESULTS: Most of the 19 included patients had refractory epilepsy (68%), while 16% were seizure-free. Generalized epilepsy (GE) was present in 58% of patients, and the rest had structural-focal epilepsy (26%), combined generalized and focal epilepsy (11%), or childhood occipital epilepsy (COE) (5%). Atypical EEG features with full atypical morphology were found in 91% of patients with GE. All patients with GPFA provoked by sleep had epileptiform K-complexes. The presence of GPT was not different between the GE and non-GE groups and was higher in patients with GPFA occurring only during sleep (p = 0.017). In two patients, GPFA frequency increased postictally. A transition from fixation-off sensitivity to GPFA occurred in a patient with COE. CONCLUSION: In this study, GPFA had a wide diagnostic range from focal to generalized epilepsy. The association of GPFA with other electroclinical features was of importance mostly for sleep outcomes; this finding might lead to a better understanding of epileptogenesis.

Temporal encephaloceles can be missed in patients with refractory temporal lobe epilepsy.

Temporal encephaloceles (TEs) are one of the cause of refractory temporal lobe epilepsy (TLE). We reviewed the neuroimaging and video-electroencephalography (EEG) records of epilepsy patients who underwent temporal lobectomy in our center to investigate frequency of TEs. We retrospectively reevaluated 294 patients who underwent epilepsy surgery in our tertiary epilepsy centre between January 2010 and March 2019 and included 159 patients (78 females, 49 %; 81 males) who had temporal lobectomy. Preoperatively, TEs were reported in 3 of 159 patients (1 female, 2 males). After reevaluation 4 more patients with TEs (1 female, 3 males) were added. The ratio of TE in patients who underwent temporal lobectomy increased from 1.8 % (n=3) to 4,4 % (n=7). The median ages were 18 (range 16-22) versus 10 years (range 5-17) at habitual seizure onset and the median of epilepsy duration was 5 (range 3-15) versus 175 (range 11-25) years between patients with and without TE. Habitual seizure onset age was significantly higher (p =, 007) in the patients with encephalocele and epilepsy duration was shorter (p =, 003) than patients without encephalocele. The ictal EEG records of all patients TE rhythmic delta activity which is suggested neocortical temporal lobe onset seizures. 4 of 7 patients' PET imaging showed temporal lobe hypometabolism compatible with ipsilateral to the TEs. The three patients underwent anterior temporal lobectomy without amygdalohippocampectomy and others had anterior temporal lobectomy with amygdalohippocampectomy. We suggested that there might be some clues for temporal encephalocele, an easily overlooked cause in patients with nonlesional temporal lobe epilepsy.TLE patients with TE had relatively late onset of epilepsy and rhythmic delta activity on ictal EEG. Also, temporal hypometabolism on PET may be a useful key to suspicion of TE.

Nocturnal Rapid Eye Movement Sleep Without Atonia can Be a Diagnostic Parameter in Differentiating Narcolepsy Type 1 From Type 2.

PURPOSE: We aimed to compare rapid eye movement sleep without atonia (RSWA), tonic RSWA, and phasic RSWA indices during polysomnography as a potential biomarker between narcolepsy type 1 and type 2. METHODS: Medical files, polysomnography, and multiple sleep latency tests of patients with narcolepsy were evaluated retrospectively. A total of three adolescents and 31 adult patients were included. We calculated the total number of rapid eye movement (REM) epochs with tonic and phasic activity in accordance with the American Academy of Sleep Medicine manual scoring rules, version 2.4. We defined tonic RSWA index as the ratio of total number of REM sleep stage epochs with only tonic activity to total REM sleep stage epochs, phasic RSWA index as the ratio of total number of REM sleep stage epochs with only phasic activity to total REM sleep stage epochs, and RSWA index as the ratio of total number of REM stage sleep epochs with RSWA to total REM sleep stage epochs on the polysomnography. RESULTS: Clinically and polysomnographically diagnosed 25 patients with narcolepsy type 1 and 9 patients with narcolepsy type 2 were included. The median age of the subjects was 30 (10, 61) and 36 (18, 64), respectively. Eleven narcolepsy type 1 patients (44%) and 4 narcolepsy type 2 patients (44.44%) were women. The RSWA index of ≥ 3% yielded a sensitivity of 76% and specificity of 88.9% (AUC = 0.77 (0.09), 95% confidence interval = 0.58 to 0.97, p = 0.01), and the tonic RSWA index of ≥ 2.2% yielded a sensitivity of 72% and specificity of 77.8% (area under the curve = 0.74 (0.1), 95% confidence interval = 0.54-0.94, p = 0.03). CONCLUSIONS: As an electrophysiological biomarker, RSWA and tonic RSWA indices can be sensitive and specific polysomnography parameters in distinguishing narcolepsy type 1 from narcolepsy type 2.

Aphasic status epilepticus in a tertiary referral center in Turkey: Clinical features, etiology, and outcome.

OBJECTIVE: Aphasic status epilepticus (ASE), although rare, may often remain underdiagnosed. We aimed to report the patients diagnosed with ASE and describe their clinical presentation, etiology, electrophysiological findings, neuroimaging, treatment options, and outcome. METHODS: We retrospectively analyzed a series of 28 patients diagnosed with ASE between January 2010 and August 2019 in our tertiary referral center. We reviewed medical files, patient charts, and short- and long-term intermittent electroencephalogram (EEG) recordings. Demographical, historical, clinical, neuroimaging, electrophysiological parameters, administered antiseizure medications, and prognosis of all patients were recorded. Furthermore, EEGs were re-evaluated according to Salzburg criteria. RESULTS: Most patients presented with tumors (n = 11) and cerebrovascular disease (CVD) (n = 11), while the rest were diagnosed with hyperglycemia (n = 2), autoimmune encephalitis (n = 1), remote intracranial abscess (n = 1), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) attack (n = 1), or unknown cause (n = 1). Seventy-one percent of patients had prior history of epilepsy. The most common EEG findings were rhythmic delta activity with fluctuation and epileptiform discharges ≤ 2.5 Hz with spatiotemporal evolution (66 %). Magnetic resonance imaging (MRI) and EEG findings indicated dominant hemisphere involvement in all patients. No casualties occurred; 71 % patients exhibited total recovery, while 29 % had mild-moderate sequela aphasia. None of the patients received aggressive treatment for ASE. SIGNIFICANCE: A relatively higher number of ASE patients could be contributed to the literature through this study. ASE should be considered in patients with prolonged unexplained aphasia, especially with pre-existing risk factors, such as prior history of epilepsy, structural lesion, and metabolic disorders accompanied by concordant EEG findings. Although the underlying cause is a determining prognostic factor, this study shows a significant number of patients with complete remission; thus, the prognosis of ASE may be considered more favorable than other types of nonconvulsive status epilepticus.

The coexistence of psychogenic nonepileptic and epileptic seizures in the same patient is more frequent than expected: Is there any clinical feature for defining these patients?

OBJECTIVES: This study aimed to investigate the prevalence of psychogenic nonepileptic seizures (PNES) and PNES-epilepsy coexistence within all video-electroencephalography (EEG) monitoring unit (VEMU) referrals and to identify semiological and electrophysiological features to differentiate patients with PNES-epilepsy coexistence from PNES-only. METHODS: We retrospectively reviewed medical files, VEMU reports, and videos of 1983 adult patients. Demographical, historical, clinical, neuroimaging, and electrophysiological parameters of all patients were recorded. We classified patients into five groups as definite PNES-only, definite PNES-epilepsy coexistence, definite PNES-probable epilepsy coexistence, probable PNES-definite epilepsy coexistence, and probable PNES-only. We defined a "definite" group when we saw the ictal EEG and/or video recording of the seizure. The "probable" term is used when there is strong evidence from the history of a particular seizure type and suggestive interictal EEGs without video recordings. RESULTS: Two hundred and three of 1983 patients (10.23%) had PNES. Sixty-six of patients with PNES (32.51%) had definite PNES-epilepsy coexistence. When probable cases were included, the PNES-epilepsy coexistence ratio was 53.69% within all patients with PNES. The prevalence of PNES-epilepsy coexistence was 3.32% within all our VEMU referrals. Lower high school graduation rate, earlier age of disease onset, history of status epilepticus, febrile convulsion and brain surgery, use of three or more antiepileptic drugs, and abnormal magnetic resonance imaging (MRI) findings supported PNES-epilepsy coexistence (p < 0.05). On the contrary, seizure duration longer than 10 min was in favor of PNES-only (p < 0.05). CONCLUSIONS: The prevalence of PNES-epilepsy coexistence might be more frequent in VEMUs than expected. Some demographic and semiological features and electrophysiological findings might be useful in differentiating patients with PNES-epilepsy coexistence from patients with PNES-only.

Lower limb automatism in surgically treated temporal lobe epilepsy patients: Clinical and electrophysiological features.

Lower limb automatism has not been known well in temporal lobe epilepsy (TLE) patients. This study investigated the distribution of risk factors, EEG features, and pathology types in surgically treated TLE patients. We also made a comparison of this group to surgically treated TLE patients with isolated hand automatism. Twenty TLE patients with lower limb automatism (Group 1) and 20 TLE patients with isolated hand automatisms (Group 2) of similar age/sex distribution were enrolled in our study. Male/female ratio was 14/6 in both groups. Demographical characteristics, risk factors, pathology types and EEG features were compared between two groups. 15 and 8 patients out of Group 1 (75%) and Group 2 (40%) respectively, were undergone right-sided surgery. Ipsilateral lower limb automatism was seen in 80% of patients. The age of epilepsy onset was earlier in patients with lower limb automatism (p = 0.02). There was no significant difference between the groups in terms of the risk factors and other demographical characteristics. Although, EEG features were not different, onset of ictal EEG changes in the first 10 seconds were seen less frequently in Group 1(6 vs 9 patients) (p = 0.31). Hippocampal sclerosis as a pathology type was detected in 11 patients (55%) of Group 1, whereas in 16 patients (80%) of Group 2. TLE patients with lower limb automatism have an earlier age of epilepsy onset and the onset of ictal EEG changed in the first 10 seconds of clinical seizure and pure HS pathology was rarer than in TLE patients with hand automatisms. Further studies are needed to shed more light on the pathophysiology of lower extremity automatisms in TLE patients.

Pharmacoresistant seizures in neurofibromatosis type 1 related to hippocampal sclerosis: Three case presentation and review.

Neurofibromatosis type 1 (NF1) is an autosomal dominantly inherited disorder, with an estimated prevalence of 1 in 3000-4000 people. Seizures occur 4-7% of individuals with NF1, mostly due to associated brain tumors or cortical malformations. Hippocampal sclerosis (HS) in the patients with NF1 has been reported very rarely and only 15 patients were found in review of English literature. We presented here 3 additional patients with NF1 and intractable seizures due to hippocampal sclerosis; in whom one of them underwent epilepsy surgery and he is seizure free for 5 years after right temporal lobectomy.

Heart Rate Variability During Nocturnal Sleep and Daytime Naps in Patients With Narcolepsy Type 1 and Type 2.

PURPOSE: Heart rate variability is a noninvasive measure of autonomic function. The aim of this study was to determine the risk of cardiovascular autonomic dysfunction during sleep in patients with narcolepsy. The effect of daytime napping was also examined in patients with narcolepsy type 1 and type 2. METHODS: Polysomnography recordings and multiple sleep latency tests from 11 patients with narcolepsy type 1 (N1) and 20 patients with narcolepsy type 2 (N2) were included and compared with 12 healthy controls. Heart rate variability values (measured by time- and frequency-domain parameters) were calculated using electrocardiography data from the polysomnography-multiple sleep latency test recordings. RESULTS: As an indicator of increasing sympathovagal imbalance, the low-frequency/high-frequency ratio was higher in all patients than in controls during non-rapid eye movement (NREM)-2 stage sleep (P ≤ 0.01). The root mean square of successive RR interval differences was lower (indicating parasympathetic tone) in N1 patients compared with N2 patients during REM sleep (P ≤ 0.04). In addition, decreased heart rate variability values were observed during sleep-onset REM-negative multiple sleep latency test periods compared with sleep-onset REM-positive periods. CONCLUSIONS: Heart rate variability abnormalities during sleep and the role of these changes on the development of cardiovascular diseases must be investigated in prospective follow-up studies of patients with narcolepsy. Heart rate variability changes during night sleep, daytime napping, and presence of sleep-onset REM periods may affect the life-threatening events.

Differences in diffusion tensor imaging changes between narcolepsy with and without cataplexy.

OBJECTIVE: The distinctive clinical finding of Type 1 narcolepsy compared to Type 2 is the presence of cataplexy. Several neuroimaging studies have also reported abnormalities in narcolepsy patients with or without cataplexy. However, there are conflicting results to differentiate them. In this study, we aimed to clarify the white matter changes in narcolepsy patients both with and without cataplexy and compared them with healthy adults to evaluate microstructural differences in the brain. METHODS: Eleven narcolepsy patients with cataplexy (NC), 12 narcolepsy patients without cataplexy (NOC) and healthy age- and gender-matched controls (N = 16) were studied. Whole-brain diffusion tensor imaging (DTI) was obtained and tract-based spatial statistics were used to localize white matter abnormalities. RESULTS: Compared with the healthy controls, both NC and NOC patients exhibited significant fractional anisotropy (FA) decreases in the bilateral cerebellar hemispheres, bilateral thalami, the corpus callosum and left anterior-medial temporal white matter. Compared with the controls, the NC patients' FA values were also decreased in the midbrain. No significant correlations were found between FA values and clinical-polysomnographic variables. CONCLUSION: This DTI study has demonstrated white matter abnormalities in the midbrain-brainstem regions as a distinctive finding of narcolepsy patients with cataplexy. Involvement of bilateral temporal lobes with greater changes on the left lobe is also a supporting finding of patients with cataplexy. DTI changes in the midbrain-brainstem and bilateral temporal lobes can be signs of different pathological mechanisms in these patients.

Autoimmune Epilepsy and/or Limbic Encephalitis Can Lead to Changes in Sleep Spindles.

INTRODUCTION: Sleep disorders have been described in patients with autoimmune limbic encephalitis (LE). The changes in sleep structure were also reported. Recently sleep spindle abnormalities such as asynchronous or prolonged spindles were observed children with LE. METHODS: We studied the sleep and number of sleep spindles in the continuous electroencephalography-polysomnography (EEG-PSG) recordings of 6 patients with autoimmune epilepsy and/or LE. The longest NREM 2 period was selected. We evaluated the spindle density (spindles per minute), and compared that to the spindle densities of epilepsy patients with bilateral hippocampal sclerosis and healthy controls. RESULTS: We have demonstrated that patients with autoimmune epilepsy and/or LE had reduced slow wave sleep with decreased number of sleep spindles. The mean number of spindles in 60 seconds was 5.86±5.03 in patients with autoimmune epilepsy and/or LE. But spindle density was higher in two control groups (10.6±1.65 and 9.95±0.79). CONCLUSIONS: The sleep abnormalities in LE can result from the disruption of thalamo-limbic circuits, and lead to changes in spindle wave activity. Although density of spindles decreased with acute lesions in thalamo-limbic circuits, the relations with structural lesions or chronicity of disease are not clear. That may be related to functional disruption of neural circuitry.