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.

We describe a patient who presented with temporal lobe epilepsy and a seizure onset pattern in the right temporo-occipital area. Structural MRI revealed sclerosis in the right hippocampus. A comprehensive presurgical neuropsychological assessment allowed us to disentangle deficits in visual object recognition and visual imagery from well-preserved spatial capacities. Following a right temporal lobectomy, the patient remained seizure free, and 1year postsurgery, the patient's scores on object recognition and imagery were in the normal range. Our findings suggest that visual object recognition and visual imagery are sustained by cortical areas located in proximity to the temporo-occipital ventral pathway and that perceptual and imagery spatial processing is subserved as well by anatomically close mechanisms. Furthermore, the results seem to indicate that nonlesional paroxysmal activity in the posterior temporal lobe can cause chronic dysfunctions of the visual system and that it may be reversible with effective seizure control.

[Case records of Epileptic Disorders. Anatomo-electro-clinical correlations. Case 02-2009] Background. Functional hemispherectomy (FH) is mainly performed in children with catastrophic epilepsies and preexisting severe hemiparesis. It is accepted that mainly patients at young ages with more or less complete loss of their hand function are suitable candidates for FH. However, we feel that FH should be considered also in some adults with devastating unilateral epilepsy syndromes. Patients and methods. We report four cases of adults who underwent FH because of their intractable epilepsy syndromes. Three of the four patients had reduced but not complete loss of hand function. Careful weighing-up of the risk-benefit ratio still led to the decision to perform FH in order to achieve complete seizure relief. Results. All patients had a surgical outcome of Engel class I; one of them suffered from post-operative psychogenic non-epileptic seizures which were treated successfully by psychotherapy. No clinically relevant complications occurred. Conclusion. We wish to point out that FH is a possible surgical option in cases with catastrophic epilepsies in adulthood. Patients and relatives should strongly consider that the likelihood of at least partially, impaired hand function or other surgery-related additional handicaps may be outweighed by the chance of freedom of seizures in a limited number of patients. In all of our cases, patients and relatives agreed that the surgical outcome led to a marked improvement of quality of life and that it had been the right decision to proceed with FH in spite of the expected negative impacts of the operation. Video examples of the preand post-surgical hand function of one sample patient are enclosed. [Published with video sequences]

BACKGROUND:Functional hemispherectomy (FH) is mainly performed in children with catastrophic epilepsies and pre-existing severe hemiparesis. It is accepted that mainly patients at young ages with more or less complete loss of their hand function are suitable candidates for FH. However, we feel that FH should be considered also in some adults with devastating unilateral epilepsy syndromes.PATIENTS AND METHODS:We report four cases of adults who underwent FH because of their intractable epilepsy syndromes. Three of the four patients had reduced but not complete loss of hand function. Careful weighing-up of the risk-benefit ratio still led to the decision to perform FH in order to achieve complete seizure relief.RESULTS:All patients had a surgical outcome of Engel class I; one of them suffered from post-operative psychogenic non-epileptic seizures which were treated successfully by psychotherapy. No clinically relevant complications occurred.CONCLUSION:We wish to point out that FH is a possible surgical option in cases with catastrophic epilepsies in adulthood. Patients and relatives should strongly consider that the likelihood of at least partially, impaired hand function or other surgery-related additional handicaps may be outweighed by the chance of freedom of seizures in a limited number of patients. In all of our cases, patients and relatives agreed that the surgical outcome led to a marked improvement of quality of life and that it had been the right decision to proceed with FH in spite of the expected negative impacts of the operation. Video examples of the pre- and post-surgical hand function of one sample patient are enclosed.

Lacosamid (Vimpat®) wurde im September 2008 zur Zusatztherapie bei Patienten mit fokalen Epilepsien ab 16 Jahren zugelassen. Unmittelbar nach der Zulassung haben wir 35 Patienten mit schwer therapieresistenten Epilepsien auf Lacosamid einzustellen begonnen. In allen Fällen strebten wir eine Erhaltungsdosis von 300 mg an. Elf Patienten haben diese noch nicht erreicht. In vier Fällen haben wir inzwischen höhere Dosen als 300 mg erreicht, die höchste Dosis beträgt derzeit 500 mg. Die ersten Erfahrungen zeigen eine zufrieden stellende Verträglichkeit und keine wesentlichen Eindosierungsprobleme. Bislang hat erst eine Patientin die Therapie wegen einer Zunahme von Anfällen bei einer Tagesdosis von 200 mg verworfen, wobei ein ursächlicher Zusammenhang angesichts der hohen Spontanfluktuation der Anfälle nicht eindeutig konstatiert werden kann. Die häufigste Störwirkung war Schwindel, der ab einer Tagesdosis von 300 mg beobachtet wurde und in fast allen Fällen durch eine Verteilung auf drei Tagesdosen kupiert werden konnte. Angaben zur Wirksamkeit wären generell verfrüht, es wurden aber Eindosierungseffekte beobachtet: Über einen Beobachtungszeitraum von vier bis fünf Wochen nach Erreichen einer Erhaltungsdosis von 300 mg wurde bei drei Patienten Anfallsfreiheit erzielt, bei fünf weiteren Patienten besteht bislang eine Anfallsreduktion um mindestens 50%.

Lacosamide (Vimpat((R))) was labelled for add-on treatment of patients with localizationrelated epilepsies from age 16 years on in September 2008. Immediately after labelling we started addon lacosamide in 35 adult patients with difficult-to-treat locali-zation-related epilepsies. In all patients the target maintenance daily dosage was 300 mg, which is not yet reached in eleven cases. In four cases we increased the dose beyond 300 mg, the highest upto-date dose is 500 mg. First experiences show a satisfying tolerability without relevant titration problems. Only one patient discontinued lacosamide at a dosage of 200 mg because of an increase of seizures. However, due to the high spontaneous fluctuation rate of seizures in this patient a clear causal relationship cannot be claimed. Corresponding with the pivotal clinical trials prior to labelling dizziness was the leading adverse event and mainly occurred at a dose of 300 mg. In almost every case it could be prevented by t.i.d. dosing. The observation period is too short to judge the efficacy of lacosamide. Promising effects during titration were apparent in three seizure-free patients and five responders with a reduction of the baseline seizure frequency by at least 50% during a maintenance period of four to five weeks.