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Summary: Objective: To assess the long‐term outcome of epilepsy with auditory features (EAF) and to identify the clinical predictors for prognosis. Methods: The study involved consecutive EAF patients with a follow‐up of ≥5 years. Terminal remission (TR) was defined as a period of ≥5 consecutive years of seizure freedom at the last follow‐up. We used Kaplan‐Meier estimate to calculate the cumulative time‐dependent probability of conversion to TR. Log‐rank test and multivariate Cox regression analyses were performed to study the association between time to TR and prognostic determinants. Results: We included 123 EAF patients (male/female = 58/65) with a median follow‐up of 11 years (1626.9 person‐years). Most were sporadic cases (68.3%), whereas 31.7% reported a family history of epilepsy. At last assessment, 42 patients had achieved TR (34.1%). Of the remaining 81 cases with no TR (65.9%), 37% had been in remission for 1‐4 years and 62.9% still had seizures within the past year. The cumulative rates of TR were 26.6%, 35.7%, and 51.6% at 10, 20, and 30 years from inclusion. On multivariate analysis, age at onset > 10 years (hazard ratio [HR] = 3.2, P = .028), auditory aura characterized by distortions only versus simple/complex hallucinations (HR = 2.9, P = .041), and unremarkable scalp electroencephalogram (EEG) versus EEG with focal epileptiform activity (HR = 3.5, P = .041) were associated with TR. Significance: Our data show a wide prognostic spectrum of EAF, ranging from mild forms with spontaneous remission, to severely refractory epilepsy addressed to surgery. The outcome, less favorable than expected from previous studies, appears to be primarily a function of 3 prognostic negative risk factors: age at onset < 10 years, auditory aura characterized by complex auditory hallucinations, and focal epileptiform abnormalities on scalp EEG. These predictors, easy to collect even at the first visit, may inform both clinicians and patients about the long‐term prognosis and aid patient management. [ABSTRACT FROM AUTHOR]

Lateral temporal lobe epilepsy (lTLE) is a rare condition characterized by auditory auras or receptive aphasia, negative MRI, and relatively benign evolution. With the low number of cases in the world, our objective was to analyze a cohort of sporadic cases with lTLE (slTLE), in order to investigate possible cerebral morphological alterations. Forty patients with lTLE (34.93 ± 12.08 years of age) and 38 healthy controls (CTRL, 34.55 ± 9.08 years of age) were enrolled from four tertiary Italian epilepsy centers, which provided brain MRI T1-weighted images following a standard protocol for patients with epilepsy. We performed group comparison by following different approaches: voxel-based morphometry (VBM, SPM8), cortical thickness (CT), and local gyrification index (lGI) (FreeSurfer 5.3). At a more conservative threshold (p < 0.05, FWE correction), no significant differences between groups survived, neither in VBM nor CT/lGI. Multicenter studies have more power than smaller studies in conducting sophisticated evaluations of rare diseases, and further investigations are required to develop a full picture of this rare phenotype. [ABSTRACT FROM AUTHOR]

• Emotional facial recognition is not globally impaired in TLE. • TLE patients show deficits in recognizing facial expressions of fear and disgust. • Localization of epilepsy focus plays a role in recognizing specific expressions. • Sensitivity to emotional intensity varies with epilepsy localization and emotion. Temporal lobe epilepsy (TLE) can induce various difficulties in recognizing emotional facial expressions (EFE), particularly for negative valence emotions. However, these difficulties have not been systematically examined according to the localization of the epileptic focus. For this purpose, we used a forced-choice recognition task in which faces expressing fear, sadness, anger, disgust, surprise, or happiness were presented in different intensity levels from moderate to high intensity. The first objective of our study was to evaluate the impact of emotional intensity on the recognition of different categories of EFE in TLE patients compared to control participants. The second objective was to assess the effect of localization of epileptic focus on the recognition of EFE in patients with medial temporal lobe epilepsy (MTLE) associated or not with hippocampal sclerosis (HS), or lateral temporal lobe epilepsy (LTLE). The results showed that the 272 TLE patients and the 68 control participants were not differently affected by the intensity of EFE. However, we obtained group differences within the clinical population when we took into account the localization of the temporal lobe epileptic focus. As predicted, TLE patients were impaired in recognizing fear and disgust relative to controls. Moreover, the scores of these patients varied according to the localization of the epileptic focus, but not according to the cerebral lateralization of TLE. The facial expression of fear was less well recognized by MTLE patients, with or without HS, and the expression of disgust was less well recognized by LTLE as well as MTLE without HS patients. Moreover, emotional intensity modulated differently the recognition of disgust and surprise of the three patient groups underlying the relevance of using moderate emotional intensity to distinguish the effect of epileptic focus localization. These findings should be taken into account for interpreting the emotional behaviors and deserve to be further investigated before considering TLE surgical treatment or social cognition interventions in TLE patients. [ABSTRACT FROM AUTHOR]

Attention deficit hyperactivity disorder (ADHD) is one of the most common developmental disorders in school-aged children. Symptoms consistent with ADHD have been observed in 8-77 % of children with epilepsy. Researchers have been motivated to search for alternative forms of treatment because 30 % of patients with ADHD cannot be treated by psychostimulants. Several studies support the use of a multimodal treatment approach that includes neurofeedback (NF) for the long-term management of ADHD. These studies have shown that NF provides a sustained effect, even without concurrent treatment with stimulants. We aimed to assess cognitive flexibility in ADHD children with and without temporal lobe epilepsy (TLE), and to evaluate the effects of NF on cognitive flexibility in these groups of children. We prospectively evaluated 69 patients with ADHD aged 9-12 years. The control group was 26 ADHD children without TLE who received no treatment. The first experimental group comprised 18 children with ADHD. The second experimental group comprised 25 age-matched ADHD children with TLE. This group was further divided in two subgroups. One subgroup comprised those with mesial temporal lobe epilepsy (16 patients, 9 with hippocampal sclerosis and 7 with hippocampal atrophy), and the other with lateral temporal lobe epilepsy (9 patients, 5 with temporal lobe dysplasia, 3 with temporal lobe cysts, and 1 with a temporal lobe cavernoma). We treated their ADHD by conducting 30 sessions of EEG NF. Reaction time and error rates on the Trail Making Test Part B were compared before and after treatment, and significant differences were found for all groups of patients except those who had mesial temporal lobe epilepsy with hippocampal atrophy. Our results demonstrate that in most cases, NF can be considered an alternative treatment option for ADHD children even if they have TLE. Additional studies are needed to confirm our results. [ABSTRACT FROM AUTHOR]

The differentiation of combined mesial and lateral temporal onset of seizures (mesio-lateral TLE, MLTLE) from lateral TLE (LTLE) is critical to achieve good surgical outcomes. However, the functional neuroimaging features in LTLE patients based on the ictal onset zone utilizing intracranial EEG (iEEG) in a large series have not been investigated. We enrolled patients diagnosed with MLTLE ( n = 35) and LTLE ( n = 53) based on the site of ictal onset zone from iEEG monitoring. MLTLE is defined when ictal discharges originate from the mesial and lateral temporal cortices independently, whereas seizures of LTLE arise exclusively from the lateral temporal cortex. Compared to patients with LTLE, patients with MLTLE were more likely to have 18F- fluorodeoxyglucose positron emission tomography (FDG-PET) hypometabolism and hyperperfusion on ictal single-photon emission computed tomography (SPECT) restricted to the temporal areas. MLTLE patients had more frequent aura or secondarily generalized seizures than LTLE patients. No significant differences were found in scalp EEG, MRI, and Wada asymmetry between groups. The overall seizure-free rate was good (73.8 %, mean follow-up = 9.7 years), which was not different (Engel class I, 74.3 % in MLTLE vs. 73.6 % in LTLE). Postsurgical memory function was spared in LTLE patients, while visual memory was impaired in MLTLE patients when their mesial temporal structures were sufficiently resected. It suggests that functional neuroimaging (interictal PET and ictal and interictal SPECT) may play a crucial role to differentiate between MLTLE and LTLE. [ABSTRACT FROM AUTHOR]

Objective: We explored changes in heart rate during the peri-ictal period in patients with focal epilepsy, and differences in heart rate changes according to epileptic site and side were assessed., Methods: A total of 198 epileptic seizures in 102 patients with focal epilepsy, who had a definite epileptogenic focus and had undergone surgical treatment, were assessed from 2014 to 2019. Heart rate was measured manually during the peri-ictal period. Change in heart rate and the time it occurred were assessed and compared between different epileptic sites and sides., Results: Heart rate increased in 177 (89.4%) of 198 seizures. In 82 (44.8%) of 183 seizures, the change in heart rate occurred before seizure onset. The median period of heart rate change was seven seconds (interquartile range: 3–11 seconds) in seizures with heart rate change before seizure onset. The number of seizures with heart rate increase before seizure onset was significantly greater for medial temporal lobe epilepsy compared to lateral temporal lobe epilepsy (p=0.019) and extratemporal lobe epilepsy (p=0.002)., Significance: A change in heart rate prior to seizure onset is more likely to occur in patients with medial temporal lobe epilepsy, compared to those with lateral temporal lobe epilepsy and extratemporal lobe epilepsy. Patients with medial temporal lobe epilepsy may likely benefit from seizure warning and detection devices.

Summary: Purpose: Intracranial EEG in patients with lesional lateral temporal lobe epilepsy is rarely reported. Therefore, the number of patients with seizures arising independently from ipsilateral mesial structures or contralateral hemisphere has not been clarified. We analyzed the intracranial EEG of cases with localized lesion in the lateral temporal cortex. Methods: We studied 15 patients who satisfied the following criteria: (1) MRI depicted a lesion less than 4cm in diameter located lateral to the collateral sulcus and at least 3cm posterior to the temporal pole; (2) intracranial EEG with electrodes placed on bilateral temporal lobes captured at least one complex partial seizure; and (3) postoperative follow-up period of 2 years or longer. The mean age of seizure onset was 16.6 years (range, 11–25) and that at surgery was 26.7 years (range, 16–36). Results: A total of 147 complex partial seizures, 51 simple partial seizures, 16 secondarily generalized seizures, and over 80 subclinical seizures were recorded. On the lesional side, many clinical seizures were recorded from the lateral cortex. Independent of the lateral temporal onset seizures, ictal discharges originating from the mesial temporal structures were recorded in 7 of 15 patients (47%). Moreover, onset of ictal discharges from the contralateral temporal lobe was recorded in 7 of 15 patients (47%). Interictal spikes from ipsilateral mesial structures were recorded in all patients. The presence of ipsilateral mesial onset seizures was not associated with hippocampal neuron losses. Conclusion: Intracranial EEG analysis revealed that approximately one-half of the patients with structural lesions in the lateral cortex showed independent epileptogenic areas in ipsilateral mesial structures. Although ictal discharges originating from the contralateral temporal lobe were recorded in a half of these patients, this finding does not constitute a contraindication of resective surgery. Interictal spike is not an indicator of whether mesial structures should be resected. [Copyright &y& Elsevier]

Purpose:Autosomal dominant lateral temporal lobe epilepsy (ADLTLE) is a rare familial epilepsy with onset in adolescence or early adulthood, associated with mutations ofLGI1in most families. We describe the clinical, neuropsychological, and molecular genetic study of a new ADLTLE Italian family.Methods:A four-generation family from Sardinia was studied. Clinical, neuropsychological, and genetic analysis were performed in eight living affected family members.Results:Nine family members had seizures over four generations; four of them had auditory auras and aphasia followed by secondarily generalized tonic–clonic seizures (SGTCs). One individual in addition had visual symptoms, and one family member had only vertigo followed by SGTCs. The side of seizure onset could not be determined in these five patients with focal seizures. The proband had febrile and afebrile tonic–clonic seizures. Two family members had only febrile seizures. Inheritance was autosomal dominant with 59% penetrance. Genetic molecular analysis showed a newLGI1missense mutation causing a Leu154Pro substitution in six affected and one unaffected individuals. Dichotic listening performance was abnormal in four affected individuals compared with controls. Fluency and lexical abilities also were pathological in three patients. These findings showed that in patients, the left temporal lobe was less specialized in the auditory processing function than in controls.Conclusions:In this ADLTLE family, both seizure semiology and neuropsychological findings point to a lateral temporal lobe dysfunction. The newly identifiedLGI1mutation might underlie both the seizure disorder and the neuropsychological deficits. [ABSTRACT FROM AUTHOR]

• Patients with lateral temporal lobe epilepsies (LTLE) exhibit distinct cognitive profiles. • Memory processing and executive functions are affected in LTLE. • Regions that support language and semantic processing are more affected. • Antiepileptic drugs specifically affect cognition. Lateral temporal lobe epilepsies (LTLE) are poorly characterized heterogeneous epilepsies. As the lateral temporal lobe supports distinct functions, we hypothesized that neuropsychological profiles could differ according to the localization of the seizure focus within the lateral temporal lobe. We retrospectively examined the neuropsychological characteristics of 74 consecutive patients with refractory LTLE assessed in the context of a presurgical investigation at the Pitié-Salpêtrière Hospital in Paris between 1998 and 2018. Precise localization of the epileptic focus was correlated with scores on tests of intelligence (Global, Verbal and Performance IQ), working memory, episodic memory (verbal and visual learning and forgetting), executive functions, and language abilities. We demonstrated an impact of the localization of the epileptic focus within the lateral temporal lobe with worse learning and/or executive performances depicted in the infero-basal and pure pole LTLE groups and greater language difficulties in the posterior LTLE group, Antiepileptic drugs had a greater effect than parameters related to the epilepsy itself as the lesion or the disease duration, and finally as in medial TLE, the age, education, and sex influenced some cognitive performances. Our findings show that the lateral temporal neocortex is also part of the neural substrate for memory processing and executive functions and suggest that this involvement could be related to functions devoted to specific subregions of the temporal lobe (i.e., temporal pole, inferior and basal regions) that support language and semantic processing. [ABSTRACT FROM AUTHOR]