The case report describes a 65-year-old man with arterial hypertension and a metallic aortic valve who presented to the emergency room for a loss of consciousness event and memory impairment. The electroencephalographic recording showed right temporal epileptiform activity followed by a 9 s asystole with quick consciousness recovery. The patient was diagnosed with right temporal epilepsy with asystole and was prescribed levetiracetam to prevent new events. A pacemaker was indicated in the follow-up for the long duration of the asystole, preventing major morbidity. Ictal asystole (IA) is a rare phenomenon of epilepsy that leads to syncope. It is observed in focal epilepsy, especially in left temporal epilepsy. Underlying cardiac pathology may facilitate IA, especially when the onset of the epilepsy is new. Knowledge of focal temporal semiology is key, concerning our case report, the memory impairment points to temporal pathology, and ictal vomiting in the non-dominant hemisphere. Anti-seizures drugs must be initiated in all patients, and there is a recommendation to avoid those with negative inotropic and arrhythmogenic effects (such as phenytoin, carbamazepine, and lacosamide). There is a discussion about pacemaker indication, however, it is highly recommended in non-controlled epilepsy and in ictal asystoles that last for more than 6 s to reduce morbidity.

暂无摘要。

Treatment decisions in epilepsy critically depend on information on the course of the disease, its severity and options for specific local interventions. We here report a patient with pharmaco-resistant non-lesional temporal lobe epilepsy with evidence for predominant right temporal epileptogenesis. While seizure frequency had been grossly underestimated for many years, ultralong-term monitoring with a subcutaneous EEG device revealed actual seizure frequency (66 over 11 months vs four patient-documented seizures), providing objective data on treatment efficacy and additional supportive lateralizing information that played a decisive role for the choice of surgical treatment, which had been rejected by the patient prior to this information.

OBJECTIVE: Patients with Temporal Lobe Epilepsy (TLE) and Alzheimer\'s Disease (ad) demonstrate increased β-amyloid and tau deposition, relative to patients with either TLE or ad. Presentation of ad may be atypical and more rapid in patients with both disorders. We present four serial neuropsychological evaluations (NPE) of a 68-year-old, right-handed woman with history of epilepsy (which became treatment refractory), neurosurgical resection, and incidental neuropathological findings of early-onset ad.
METHODS: History included generalized tonic-clonic seizure in adolescence and numerous complex partial seizures, managed for ~20 years on valproic acid. At age 63, she reported memory changes. Neurophysiological work-up revealed right frontotemporal seizure foci, and neuroimaging studies revealed amyloid angiopathy. She underwent right anterior temporal lobectomy and amygdalohippocampectomy. Afterwards, seizure control and perceived memory function improved. Post-surgical pathological analysis detected neurofibrillary tangles, neuritic plaques, angiopathy, anoxic changes, and gliosis. Later, she and family described memory decline and behavioral disinhibition. Follow-up CSF analysis identified p-tau/β-amyloid ratio consistent with ad.
RESULTS: Presurgical NPE was largely intact. Post-surgical NPE revealed stable/improved executive functioning (EF), visuospatial skills (VS), and memory, with variability in language. Follow-up NPE 10 months later identified general stability with some variability (e.g., source memory difficulties). Follow-up NPE 16 months after that revealed declines in several domains, with sparing of VS and language.
CONCLUSIONS: We present the cognitive trajectory of an individual with refractory TLE and early-onset ad, complicated by cognitive/neuropsychiatric sequelae of neurosurgical resection. This case highlights the potential benefits of serial re-evaluation in assessing for cognitive change suggestive of ad in aging populations with refractory TLE.

Temporal lobe epilepsy is the most common focal epilepsy syndrome and has a broad spectrum of presentations. Nevertheless, isolated vestibular symptoms without other symptoms typical of temporal lobe seizures are relatively rare. Here, we report one female patient who suffered from chronic refractory vertigo and had inappropriate pharmacotherapy for several years. Eventually, epileptic vertigo and dizziness (ictal vertigo) were accurately diagnosed by detailed history taking and serial examinations assisted by sphenoid electroencephalography. Awareness of this unique syndrome is important in the diagnosis of patients with epileptic vertigo and dizziness.

In patients with treatment-refractory temporal lobe epilepsy (TLE), a single stereotactic laser interstitial thermotherapy (LITT) procedure is sometimes insufficient to ablate epileptogenic tissue, particularly the medial structures often implicated in TLE. In patients with seizure recurrence after initial ablation, the extent to which a second ablation may achieve improved seizure outcomes is uncertain. The objective of this study was to investigate the feasibility and potential efficacy of repeat LITT amygdalohippocampotomy as a worthwhile strategy for intractable temporal lobe epilepsy by quantifying changes to targeted mesial temporal lobe structures and seizure outcomes.
Patients who underwent two LITT procedures for drug-resistant mesial TLE at our institution were included in the study. Lesion volumes for both procedures were calculated by comparing post-ablation intraoperative sequences to preoperative anatomy. Clinical outcomes after the initial procedure and repeat procedure were classified according to Engel scores.
Five consecutive patients were included in this retrospective case series: 3 with right- and 2 with left-sided TLE. The median interval between LITT procedures was 294 days (range: 227-1918). After the first LITT, 3 patients experienced class III outcomes, 1 experienced a class IV, and 1 experienced a class IB outcome. All patients achieved increased seizure freedom after a second procedure, with class I outcomes (3 IA, 2 IB).
Repeat LITT may be sufficient to achieve satisfactory seizure outcomes in some individuals who might otherwise be considered for more aggressive resection or palliative neuromodulation. A larger study to establish the potential value of repeat LITT amygdalohippocampotomy vs. other re-operation strategies for persistent, intractable temporal lobe epilepsy is worth pursuing.

暂无摘要。

In the evaluation of drug-resistant epilepsy (DRF), a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature.
We present two cases from our series of patients monitored by videoEEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS. A bibliographic review is also carried out to try to establish a localizing and/or lateralizing value of this sign.
The sign of the cross is a rare ictal automatism that occurs in patients with temporal lobe epilepsy. The hand used to make the sign of the cross is the dominant one, regardless of the origin of the crises.
La señal de la cruz: un automatismo muy poco frecuente en la epilepsia del lóbulo temporal. Descripción de dos casos.
Introducción. En la evaluación de la epilepsia farmacorresistente, el análisis detallado de la semiología es fundamental para establecer una hipótesis diagnóstica de la localización de la zona epileptógena. La señal de la cruz es un automatismo manual complejo muy infrecuente descrito por primera vez en 2008 y poco referido en la bibliografía. Caso clínico. Presentamos dos casos con presencia de la señal de la cruz de nuestra serie de pacientes monitorizados mediante videoelectroencefalograma, uno de ellos estudiado también con electrodos profundos, en los que describimos la localización de la descarga en el momento de realizar la señal de la cruz. Se realiza también una revisión bibliográfica para intentar establecer un valor localizador y/o lateralizador de este signo. Conclusión. La señal de la cruz es un raro automatismo ictal que ocurre en pacientes con epilepsia del lóbulo temporal. La mano empleada para la señal de la cruz es la dominante, independientemente del origen de las crisis.

The N-methyl-d-aspartate receptor (NMDAR) is an enigmatic macromolecule that has garnered a good deal of attention on account of its involvement in the cellular processes that underlie learning and memory, following its discovery in the mid twentieth century (Baudry and Davis, 1991). Yet, despite advances in knowledge about its function, there remains much more to be uncovered regarding the receptor\'s biophysical properties, subunit composition, and role in CNS physiology and pathophysiology. The motivation for this review stems from the need for synthesizing new information gathered about these receptors that sheds light on their role in synaptic plasticity and their dichotomous relationship with the amino acid d-serine through which they influence the pathogenesis of neurodegenerative diseases like temporal lobe epilepsy (TLE), the most common type of adult epilepsies (Beesley et al., 2020a). This review will outline pertinent ideas relating structure and function of t-NMDARs (GluN3 subunit-containing triheteromeric NMDARs) for which d-serine might serve as an inverse co-agonist. We will explore how tracing d-serine\'s origins blends glutamate-receptor biology with glial biology to help provide fresh perspectives on how neurodegeneration might interlink with neuroinflammation to initiate and perpetuate the disease state. Taken together, we envisage the review to deepen our understanding of endogenous d-serine\'s new role in the brain while also recognizing its therapeutic potential in the treatment of TLE that is oftentimes refractory to medications.

Cerebrovascular disease (CVD) is a major contributor to epilepsy; however, patients with epilepsy also have a significantly increased risk of stroke. The way in which epilepsy contributes to the increased risk of stroke is still uncertain and is ill-characterized in neuropathological studies. A neuropathological characterization of cerebral small vessel disease (cSVD) in patients with chronic epilepsy was performed.
Thirty-three patients with refractory epilepsy and hippocampal sclerosis (HS) submitted to epilepsy surgery from a reference center were selected between 2010 and 2020 and compared with 19 autopsy controls. Five randomly selected arterioles from each patient were analyzed using a previously validated scale for cSVD. The presence of CVD disease imaging markers in pre-surgical brain magnetic resonance imaging (MRI) was studied.
There were no differences in age (43.8 vs. 41.6 years; p = 0.547) or gender distribution (female gender 60.6% vs. male gender 52.6%; p = 0.575) between groups. Most CVD findings in brain MRI were mild. Patients had a mean time between the epilepsy onset and surgery of 26 ± 14.7 years and were medicated with a median number of three antiseizure medication (ASMs) [IQR 2-3]. Patients had higher median scores in arteriolosclerosis (3 vs. 1; p < 0.0001), microhemorrhages (4 vs. 1; p < 0.0001) and total score value (12 vs. 8.9; p = 0.031) in comparison with controls. No correlation was found between age, number of years until surgery, number of ASMs or cumulative defined daily dosage of ASM.
The present study provides evidence supporting the increased burden of cSVD in the neuropathological samples of patients with chronic epilepsy.