Ictal kissing in a patient with right frontal lobe epilepsy

When performing pre‐surgical evaluation of patients with refractory epilepsy, the analysis of seizure semiology is one of the key elements used to generate a hypothesis about the location of the epileptogenic zone. Ictal kissing is a very rarely observed ictal automatism described in patients with temporal lobe epilepsy. We present a 62‐year‐old man who was referred to our epilepsy centre for comprehensive evaluation. During prolonged video‐EEG monitoring, six focal‐onset hyperkinetic seizures were registered. In five seizures, the patient repeatedly produced sonorous kisses “into the air”. Initial ictal EEG pattern consisted of rhythmic theta or alpha activity at the right fronto‐polar and fronto‐medial electrodes. MRI depicted focal cortical dysplasia located in the right prefrontal medial cortex. This case suggests that ictal kissing can also occur in the setting of right frontal lobe epilepsy; we therefore believe that this observation expands the anatomo‐clinical correlation for this rare ictal automatism. [Published with video sequences].     

Adult absence semiology misinterpreted as mesial temporal lobe epilepsy

Correct diagnosis of seizure type and epilepsy syndrome is the foundation for appropriate antiepileptic drug selection. Inappropriate medication choices occur in the treatment of generalized epilepsy and may aggravate some seizure types, including absence seizures, potentially leading to pseudo‐drug resistance. Fortunately, a correct diagnosis of absence seizures is usually not difficult, though rarely demonstrates electroclinical overlap with focal seizures. EEG can be especially misleading when secondary bilateral synchronous discharges occur in patients with focal seizures. However, the semiology of focal seizures associated with mesial temporal lobe epilepsy has a characteristic and consistent semiology that is the mark of this common epilepsy syndrome in adulthood. We recently encountered a 53‐year‐old female with refractory seizures and a semiology strongly suggesting mesial temporal lobe epilepsy. Instead of focal seizures, prolonged absence seizures were validated by video‐EEG monitoring and she became seizure‐free after a change to broad‐spectrum antiepileptic drugs. This case further expands our understanding of the complexity of semiology in electroclinical classification and the spectrum that may occur in adult absence seizures. It serves to underscore the need for ictal EEG recordings and the importance of concordance with the clinical course during the pre‐surgical evaluation of patients with lesions and drug‐resistant epilepsy. [Published with video sequences]     

Hyperkinetic seizures in patients with temporal lobe epilepsy: clinical features and outcome after temporal lobe resection

Purpose: Temporal lobe epilepsy (TLE) is usually associated with automatisms. Hyperkinetic seizures are supposed to be unusual. Because we witnessed several patients with TLE and ictal hyperkinetic symptoms, we retrospectively assessed the number, clinical findings, and seizure outcome in such patients who had undergone temporal lobe resection. Methods: We reviewed medical history, video–electroencephalography (EEG) recording and neuroimaging of adult patients who underwent epilepsy surgery for TLE at the Kork Epilepsy Center over the last 20 years with a minimum postoperative follow‐up of 12 months. Key Findings: Among 294 patients who were resected exclusively in the temporal region, we identified 17 (6%) who presented with hyperkinetic semiology such as violent vocalization, complex movements of the proximal segments of the limbs, rotation of the trunk, pelvic thrusting, or early tonic or dystonic posturing. Most of the patients had a preceding aura. Ictal EEG activity was located in the corresponding temporal region, usually with a wide distribution over temporal electrodes with fast spread to unilateral frontal electrodes and to the contralateral side. Neuroimaging revealed extended lesions in the temporal lobe involving mesial and neocortical structures. Most of the patients underwent classical anterior temporal lobe resection including amygdalo‐hippocampectomy. Fourteen patients (82%) became completely seizure‐free (Engel class Ia). Histopathology showed mainly focal cortical dysplasia plus hippocampal sclerosis. Significance: Hyperkinetic seizure semiology may occasionally occur in patients with TLE and is, therefore, no contradiction to the hypothesis of TLE if scalp EEG patterns and neuroimaging findings correspond. The postoperative seizure outcome is favorable in such patients and not different from outcome data in classical TLE.     

Gelastic seizures: Incidence,clinical and EEG features in adult patients undergoing video‐EEG telemetry

This study aimed to determine clinical features of adult patients with gelastic seizures recorded on video –electroencephalography (EEG) over a 5‐year period. We screened video‐EEG telemetry reports for the occurrence of the term “gelastic” seizures, and assessed the semiology, EEG features, and duration of those seizures. Gelastic seizures were identified in 19 (0.8%) of 2,446 admissions. The presumed epileptogenic zone was in the hypothalamus in one third of the cases, temporal lobe epilepsy was diagnosed in another third, and the remainder of the cases presenting with gelastic seizures were classified as frontal, parietal lobe epilepsy or remained undetermined or were multifocal. Gelastic seizures were embedded in a semiology, with part of the seizure showing features of automotor seizures. A small proportion of patients underwent epilepsy surgery. Outcome of epilepsy surgery was related to the underlying pathology; two patients with hippocampal sclerosis had good outcomes following temporal lobe resection and one of four patients with hypothalamic hamartomas undergoing gamma knife surgery had a good outcome.     

Frontal lobe epileptic seizures are accompanied by elevated pitch during verbal communication

The objective of our study was to assess alterations in speech as a possible localizing sign in frontal lobe epilepsy. Ictal speech was analyzed in 18 patients with frontal lobe epilepsy (FLE) during seizures and in the interictal period. Matched identical words were analyzed regarding alterations in fundamental frequency (?o) as an approximation of pitch. In patients with FLE, ?o of ictal utterances was significantly higher than ?o in interictal recordings (p = 0.016). Ictal ?o increases occurred in both FLE of right and left seizure origin. In contrast, a matched temporal lobe epilepsy (TLE) group showed less pronounced increases in ?o, and only in patients with right‐sided seizure foci. This study for the first time shows significant voice alterations in ictal speech in a cohort of patients with FLE. This may contribute to the localization of the epileptic focus. Increases in ?o were interestingly found in frontal lobe seizures with origin in either hemisphere, suggesting a bilateral involvement to the planning of speech production, in contrast to a more right‐sided lateralization of pitch perception in prosodic processing.     

Parahippocampal epilepsy with subtle dysplasia: A cause of “imaging negative” partial epilepsy

Purpose: Lesion‐negative refractory partial epilepsy is a major challenge in the assessment of patients for potential surgery. Finding a potential epileptogenic lesion simplifies assessment and is associated with good outcome. Here we describe imaging features of subtle parahippocampal dysplasia in five cases that were initially assessed as having imaging‐negative frontal or temporal lobe epilepsy. Methods: We analyzed the clinical and imaging features of five patients with seizures from the parahippocampal region. Results: Five patients had subtle but distinctive magnetic resonance imaging (MRI) abnormalities in the parahippocampal gyrus. This was a unilateral signal abnormality in the parahippocampal white matter extending into gray matter on heavily T₁‐ and T₂‐weighted images with relative preservation of the gray–white matter boundary on T₁‐weighted volume sequences. Only one of these patients had typical electroclinical unilateral temporal lobe epilepsy (TLE); one mimicked frontal lobe epilepsy, two showed bitemporal seizures, and one had unlocalized partial seizures. All have had surgery; four are seizure‐free (one has occasional auras only, follow‐up 6 months to 10 years), and one has a >50% seizure reduction. Histopathologic evaluation suggested dysplastic features in the surgical specimens in all. Discussion: In patients with lesion‐negative partial epilepsy with frontal or temporal semiology, or in cases with apparent bitemporal seizures, subtle parahippocampal abnormalities should be carefully excluded. Recognizing the MRI findings of an abnormal parahippocampal gyrus can lead to successful surgery without invasive monitoring, despite apparently incongruent electroclinical features.     

Complex Partial Seizures of Temporal Lobe Origin in Children of Different Age Groups

Summary: The semiology of complex partial seizures(CPS) of temporal lobe origin in adults is well known and is important in establishing seizure localization in patients considered for epilepsy surgery. In contrast, the behavioral features of temporal lobe seizures (TLS) in children described in the literature have not been consistent. In the present study, we investigated children with TLS to compare their attacks to TLS occurring in adults. The study was based on video recordings of 29 children with TLS aged 18 months to 16 years. Children were included, if they became seizure-free after temporal lobectomy (except 4 children with a marked reduction in seizure frequency and 1 with isolated auras), and if clear unitemporal seizure onset in ictal EEG-recordings, unilateral radiological lesions, and corresponding histopathological findings were detected. Children aged >6 years had TLS with features similar to those of adults. In younger children, typical semiology included symmetric motor phenomena of the limbs, postures similar to frontal lobe seizures in adults, and head nodding as in infantile spasms. We concluded that the clinical features of TLS in younger children can be misleading and should therefore be considered with caution in selecting patients for surgical procedures on the temporal lobe.     

Autosomal dominant lateral temporal lobe epilepsy associated with a novel reelin mutation

Aims. Reelin mutations are responsible for a minority of families with autosomal dominant lateral temporal lobe epilepsy. Here, we report a novel nuclear family with distinct clinical and neuroradiological findings. Methods. We studied the proband and her mother by means of EEG, video‐EEG, 3T MRI, FDG‐PET and genetic testing. Results. Both patients had a focal drug‐resistant epilepsy with onset at the age of 16 and focal seizures with typical auditory features combined with fear, followed by loss of contact or evolving to bilateral tonic‐clonic seizures. The proband's ictal EEG showed clear left temporal seizure onset, and cerebral MRI revealed subtle left temporal changes (mild hypotrophy, slight blurring of the white and grey matter and hyperintensity) with corresponding left temporal mesial focal hypometabolism on FDG‐PET. Genetic testing identified a missense variant, c.6631C>T (p.Arg2211Cys), in reelin repeat #5 in both patients, which markedly affected the secretion of the protein. Conclusion. The data from this family support previous findings indicating that reelin mutations are a cause of autosomal dominant lateral temporal lobe epilepsy which has a clinical spectrum that may also encompass drug‐resistant epilepsy associated with mild MRI temporal changes.     

GAD65‐Ab encephalitis and subtle focal status epilepticus

Aims. To delineate common epilepsy features associated with the presence of glutamic acid decarboxylase autoantibodies (GAD65‐Ab). Methods. Three consecutive cases of GAD65‐Ab encephalitis patients, followed in our neurological department, were investigated with regards to clinical semiology and EEG. Results. These patients presented new‐onset subtle ictal clinical features. Patients 1 and 2 described prolonged and transitory feelings of “déjà vu ‐ déjà vécu” and a “dreamy state”. Patient 3 was admitted for subsequent transient aphasia events followed by paroxysmal behavioural disturbances. Epileptic origin of the symptoms was confirmed using either a standard EEG (observation of temporal status epilepticus in one case) or a prolonged EEG (focal epileptiform activity during an asymptomatic period for two patients). All patients suffered from clinical focal status epilepticus. Patients 1 and 2 presented with temporo‐mesial seizures in agreement with the definition for limbic encephalitis, whereas Patient 3 presented with neocortical (lateral temporal and frontal lobe) seizures arguing for a non‐limbic encephalitis. A high level of GAD65‐Ab was found in cerebral spinal fluid, confirming a diagnosis of epilepsy associated with GAD65‐Ab encephalitis. Conclusion. Encephalitis seems to be a frequent neurological syndrome associated with GAD65‐Ab disorders. Epilepsy may be more frequent and severe than currently suggested, as ictal semiology may be subtle for these outpatients in whom standard EEG is commonly falsely reassuring. Subtle focal status epilepticus is a particular semiology of the GAD65‐Ab encephalitis spectrum.     

Familial partial epilepsy with variable foci: A new family with suggestion of linkage to chromosome 22q12

Familial partial epilepsy with variable foci (FPEVF) is an autosomal dominant form of partial epilepsy characterized by the presence of epileptic seizures originating from different cerebral lobes in different members of the same family. Linkage to chromosomes 22q12 and 2q36 has been reported, although only six families have been published. We studied a new FPEVF family including nine affected individuals. The phenotype in this family was similar to that previously described and consisted of nocturnal and daytime seizures with semiology suggesting a frontal lobe origin. A video‐EEG (electroencephalography) recording of the proband’s seizures is presented and revealed hyperkinetic seizures of frontal lobe origin preceded by left frontal spikes. We excluded linkage to chromosome 2q36 and found a suggestion of linkage to chromosome 22q12 with a lod score of 2.64 (θ = 0) for marker D22S689.     

Clinical features of seizures associated with parahippocampal/inferior temporal lesions compared to those with hippocampal sclerosis

Temporal lobe epilepsy (TLE) is not a unitary electroclinical imaging syndrome. We asked if seizures arising from the parahippocampal‐inferior temporal (PIT) region differ from those associated with hippocampal sclerosis (HS). The electroclinical features of 22 patients with HS and 14 patients with lesions in the PIT region who underwent epilepsy surgery and were seizure free for at least 2 years postoperatively were analyzed retrospectively. Patients with PIT lesions had a higher frequency of hypermotor and bilateral features and a lower frequency of behavioral arrest at the onset of seizure compared to cases with HS, suggesting that TLE originating in the PIT area can mimic frontal lobe epilepsy or contralateral mesial TLE.     

Drug‐resistant parietal lobe epilepsy: clinical manifestations and surgery outcome

Aim. We reviewed a large surgical cohort to investigate the clinical manifestations, EEG and neuroimaging findings, and postoperative seizure outcome in patients with drug‐resistant parietal lobe epilepsy (PLE). Methods. All drug‐resistant PLE patients, who were investigated for epilepsy surgery at Jefferson Comprehensive Epilepsy Center between 1986 and 2015, were identified. Demographic data, seizure data, EEG recordings, brain MRI, pathological findings, and postsurgical seizure outcome were reviewed. Results. In total, 18 patients (11 males and seven females) were identified. Sixteen patients (88%) had tonic‐clonic seizures, 12 (66%) had focal seizures with impaired awareness, and 13 (72%) described auras. Among 15 patients who had brain MRI, 14 patients (93%) had parietal lobe lesions. Only three of 15 patients (20%) who had interictal scalp EEG recordings showed parietal interictal spikes. Of 12 patients with available ictal surface EEG recordings, only three patients (25%) had parietal ictal EEG onset. After a mean follow‐up duration of 8.6 years, 14 patients (77.7%) showed a favourable postoperative seizure outcome. Conclusion. In patients with PLE, semiology and EEG may be misleading and brain MRI is the most valuable tool to localize the epileptogenic zone. Postsurgical seizure outcome was favourable in our patients with drug‐resistant parietal lobe epilepsy.     

Somatosensory auras in refractory temporal lobe epilepsy

PURPOSE: To determine the prevalence, manifestations, lateralizing value, and surgical prognostic value of somatosensory auras (SSAs) in patients with refractory temporal lobe epilepsy (TLE). METHODS: Eighty-one consecutive patients undergoing temporal lobectomy for refractory complex-partial seizures were screened for SSAs. The characteristics of the somatosensory phenomena, occurrence of other aura types, seizure semiology, findings of EEG and imaging studies, temporal lobe neuropathology, and postoperative seizure outcome were determined in each patient with SSAs. RESULTS: Nine (11%) of 81 patients with refractory temporal lobe seizures reported distinct SSAs as part of their habitual seizures. The most common manifestation of SSAs was tingling (eight of nine, 89%), but sensory loss (one of nine, 11%) and pain (one of nine, 11%) also were reported. Five patients had unilateral somatosensory symptoms, and four patients had bilateral somatosensory symptoms. Seizure origin was in the contralateral temporal lobe in four (80%) of five patients with unilateral SSAs, including all patients with unilateral SSAs affecting a limb. Partial temporal lobe resection produced complete seizure remission in all nine (100%) patients 1 year after surgery and in seven (78%) of nine patients 2 years after surgery. CONCLUSIONS: SSAs occur more frequently than previously appreciated in patients with refractory temporal lobe seizures and usually manifest as either unilateral or bilateral tingling. In patients with temporal lobe seizures, unilateral SSAs involving a limb suggest a seizure origin in the contralateral temporal lobe. The surgical outcome of TLE patients with SSAs is favorable. Thus the presence of SSAs should not serve as a deterrent to temporal lobe resection in patients with clearly defined TLE.     

Ictal asystole mimicking seizure deterioration in temporal lobe epilepsy

We report on a patient with temporal lobe epilepsy, secondary to a left lateral temporal cavernoma, in whom the change in seizure semiology suggested recurrence of secondary generalized seizures. Anticonvulsive medication previously controlled secondary generalized seizures over a period of years but focal seizures continued at a lower rate. Continuous video‐EEG monitoring revealed ictal asystole associated with myoclonic syncope and falls during focal seizures arising from the left temporal lobe. After implantation of a cardiac pacemaker, no more falls occurred during the focal seizures. In conclusion, recurrence of seizure‐associated falls is typically attributed to recurrence of secondary generalized seizures, however, ictal asystole should be considered in selected epilepsy patients as a differential diagnosis of falls. [Published with video sequence]     

Frontal lobe epilepsy

About one-quarter of patients with refractory focal epilepsies have frontal lobe epilepsy (FLE). The typical seizure semiology for FLE includes unilateral clonic, tonic asymmetric or hypermotor seizures. Interictal electroencephalograms (EEG) usually reveal interictal epileptiform discharges and rhythmical midline theta, which has localizing value. The usefulness of ictal EEG recordings is limited by frequent muscle artifacts in motor seizures and because a large portion of the frontal lobe cortex is “hidden” to scalp electrodes. Ictal single photon emission CT and positron emission tomography are able to localize FLE in about one-third of patients only. A pre-surgical evaluation should include, whenever possible, a subclassification of FLE as dorsolateral frontal, mesial frontal or basal frontal lobe epilepsy to allow a minimal cortical resection. A review of the typical findings of seizure semiology, interictal and ictal EEG regarding the different FLE subtypes is given. Etiology, medical treatment and surgery are also discussed.     

Hyperkinetic motor seizures: a common semiology generated by two different cortical seizure origins

We report a 37‐year‐old, right‐handed patient with drug‐resistant focal epilepsy whose seizures were characterized by explosive hyperkinetic behaviour. Video‐SEEG revealed bifocal organization of epilepsy with two distinct cortical origins of seizures: the right temporal pole and left temporal lateral and perisylvian cortex. Irrespective of the cortical pattern of seizure onset, the hyperkinetic semiology was extremely similar. This supports a major role for “final common pathway” subcortical circuits in the genesis of the hyperkinetic semiology in this patient.     

Stereotactic bilateral transfrontal minimal radiofrequency thermocoagulation of the amygdalohippocampal complex for bilateral medial temporal lobe epilepsy: a retrospective study of 12 patients

Background. Some patients with temporal lobe epilepsy have bilateral discharges and a few have bilateral medial temporal sclerosis. Stereotactic bilateral radiofrequency thermocoagulation (RFTC) of the amygdalohippocampal complex can terminate seizures or reduce seizure severity in patients with bilateral medial temporal lobe epilepsy (BMTLE). Aim. To explore the safety and efficacy of bilateral transfrontal minimal RFTC of the amygdalohippocampal complex for the treatment of BMTLE. Methods. A total of 12 BMTLE patients were treated with bilateral transfrontal minimal RFTC of the amygdalohippocampal complex under limited coagulations. The volumes of coagulated lesions were less than 0.6 cm³ Clinical outcomes were evaluated using Engel's classification, the Liverpool Seizure Severity Scale (LSSS) 2.0, Wechsler Adult Intelligence Scale‐Revised (WAIS‐R), and Wechsler Memory Scale‐Revised (WMS‐R). Quality of life (QOL) was evaluated using the 36‐item Short Form Health Survey (SF‐36). Results. Of the 12 patients, five (42%) were assessed as Engel Class I during 12–62 months of follow‐up. LSSS scores declined sharply compared with the baseline of patients not in the seizure‐free category. Functions of memory and intelligence declined transiently without statistical significance (p>0.05) immediately after surgery, but improved significantly (p<0.05) six months later. The qualities of life improved except vitality. Conclusion. Bilateral transfrontal minimal RFTC of the amygdalohippocampal complex may terminate seizures or reduce seizure severity in patients with BMTLE. Under limited coagulations, neuropsychological function was not affected but improved along with seizure control.     

Can ACTH therapy improve the long‐term outcome of drug‐resistant frontal lobe epilepsy?

Frontal lobe epilepsy is a common focal epilepsy in children and is often difficult to treat. Adrenocorticotropic hormone (ACTH) or steroids have been used for patients with several forms of medically intractable epilepsy. We evaluated the short, medium, and long‐term evolution of patients with frontal lobe epilepsy and secondary bilateral synchrony on the EEG, who received ACTH treatment. Patients were recruited for an add‐on trial during clinical practice, and data was retrospectively analysed. The study group comprised 6 patients treated with ACTH. The effects of ACTH were assessed in the short term (at the end of a 6‐week period of ACTH treatment), medium term (at 6 months after the end of treatment), and long term (at 12 months after the end of treatment). At short‐term follow‐up, ACTH treatment was effective for all types of seizures in 5 of 6 patients and ineffective in 1 patient. All patients who were seizure‐free at the end of ACTH treatment maintained an excellent outcome, remaining seizure‐free at the end of follow‐up. Our study demonstrates that ACTH may represent an effective treatment for frontal lobe epilepsy with secondary bilateral synchrony. Further double‐blind prospective studies are required to confirm our initial findings.     

Semiology of Temporal Lobe Seizures: Value in Lateralizing the Seizure Focus

Summary: Purpose: To determine the lateralizing value of the clinical manifestations of seizures in patients with temporal lobe epilepsy (TLE), we made a retrospective videotape analysis of complex partial seizures (CPS) in 55 patients who underwent temporal lobectomy and were seizure-free postopera-tively for >2 years. Methods: Blinded to clinical details, we reviewed videotapes from video-EEG telemetry monitoring with attention paid to seizure semiology. Results: Useful lateralizing features included unilateral clonic activity (with the seizure focus contralateral in all patients), unilateral dystonic or tonic posturing (with the seizure focus contralateral in 90 and 86%, respectively), unilateral automatisms (with the seizure focus ipsilateral in 80%), and ictal speech preservation (with the seizure focus contralateral to the language-dominant hemisphere in 80%). Versive head rotation occurring ≤10 s before seizures secondarily generalized consistently predicted a contralateral focus. Seizure manifestations less predictive but suggestive of lateralization included ictal speech arrest and postictal speech status, with predictive values of 67%. Seizure manifestations not providing reliable lateralizing information included eye deviation, type of aura, and versive head movements occurring at times other than immediately before seizures secondarily generalized. Conclusions: In TLE, several clinical seizure manifestations are useful in lateralizing the seizure focus, although some provide no reliable information. Therefore, ictal semiology can assist in the evaluation of patients for seizure surgery, providing additional information in the lateralization of TLE.     

Epileptic seizure semiology in different age groups

Aims. Seizure semiology provides information about the eloquent cortex involved during a seizure and helps to generate a hypothesis regarding the localization of the epileptogenic zone (EZ), a prerequisite for surgical management of epilepsy. We aimed to study the seizure semiology among all different age groups to better characterize semiological changes that occur with age. Methods. We performed a retrospective review of video‐EEG data in paediatric and adult patients admitted to the Epilepsy Monitoring Unit over a three‐year period. Authors independently reviewed and classified the seizure semiology while blinded to clinical, EEG, and neuroimaging data. A total of 270 patients were included in the study. Results. The most frequent EZ in patients who were one month to three years old was undetermined. Focal epilepsy became more frequent in patients older than 10 years. Among patients with focal epilepsy, a posterior quadrant EZ was most frequent in children younger than three years old, a temporal EZ between three and six years old, and a frontal EZ between six and 10 years old. The temporal lobe was the most frequent location for focal EZ in patients older than 18 years. Auras, automotor seizures, and generalized tonic‐clonic seizures were extremely infrequent in patients younger than 10 years old. The youngest patient with auras was 5.7 years old. The youngest patient with automotor seizures was 3.7 years old. We identified only three patients with generalized tonic‐clonic seizures who were younger than 10 years (aged six months, 6.6 years, and nine years, respectively). Patients younger than three years exhibited mostly generalized simple motor seizures and hypomotor seizures. Generalized epileptic spasms, generalized tonic seizures, and generalized clonic seizures were infrequent in patients older than 10 years. Conclusion. Seizure semiology and electroencephalographic changes most likely reflect the maturation of cortical functions.