Outcome after corticoamygdalohippocampectomy in patients with refractory temporal lobe epilepsy and mesial temporal sclerosis without preoperative ictal recording.

PURPOSE: We report on the surgical outcome obtained in patients with refractory temporal lobe epilepsy with mesial temporal sclerosis (MTS) who were evaluated preoperatively without ictal recording and were submitted to corticoamygdalohippocampectomy. METHODS: Two hundred twelve patients with refractory temporal lobe epilepsy were evaluated by means of clinical history, neurological examination, interictal electroencephalography (EEG), magnetic resonance imaging (MRI), and neuropsychological testing. MRI disclosed unilateral MTS in all patients. All patients were submitted to corticoamygdalohippocampectomy at the side determined by MRI. RESULTS: Interictal EEG showed unilateral temporal lobe spiking in 176 patients; in 36 patients, bilateral discharges were found. Mean follow-up time was 2.7 years. One hundred ninety-four patients (92%) were classified as Engel's class I. Eighteen patients (8%) were rated as Engel's class II. Thirty-two out of 36 patients, in whom bilateral discharges were found, were in Engel's class I. Sixty percent of the patients had an improvement in memory function related to the nonoperated temporal lobe. Fifty-nine percent of the patients had a 10-point increase in general IQ postoperatively. Verbal memory decline was noted in three patients. Pathological examination showed MTS in all patients. CONCLUSIONS: It is possible to adequately select good surgical candidates for temporal lobe resection using MRI and interictal EEG alone. In patients with MRI-defined MTS, we should expect a 90% postoperative remission rate. Cognitive decline was very rarely seen in this patient population. The finding of MTS on MRI is the single most important prognostic factor for good outcome after temporal lobe surgery.

Outcome after extended callosal section in patients with primary idiopathic generalized epilepsy.

PURPOSE: We report the outcome of patients with refractory idiopathic generalized epilepsy (IGE) who were submitted to extended one-stage callosal section. METHODS: Eleven patients with IGE who were submitted to extended one-stage callosal section were studied. Preoperative workup included history and neurologic examination, interictal, and ictal electroencephalography (EEG) recording, high resolution 1.5T magnetic resonance imaging (MRI) and intelligence quotient (IQ) testing. All patients were submitted to extended one-stage microsurgical callosal section, leaving only the splenium intact. RESULTS: Preoperative ictal patterns included repetitive spike and wave or polyspike and wave discharges or fast epileptic recruiting rhythm. MRI showed no focal lesions. Preoperatively, mean general IQ was 85. Postoperatively, at least a 75% reduction in the frequency of generalized tonic-clonic seizures was noted in all patients. In three patients absences disappeared completely, and the others had at least 90% reduction in seizure frequency. Only one patient had myoclonic seizures preoperatively, and these seizures disappeared after callosal section. After surgery, mean general IQ was 89. A very clear increase in attention level was noted in all patients. Postoperative interictal EEG recordings showed rupture of bilateral synchrony in all patients. DISCUSSION: This article reports on a large and homogeneous series of patients with refractory IGE submitted for callosal section. There was a marked decrease in generalized seizure frequency and increase in the attention level in this patient population. Our results suggest that corticocortical interaction might have a role in IGE pathogenesis. Callosotomy is a safe, effective, and underused palliative procedure in these well-selected patients with refractory IGE.

Outcome after hemispherectomy in hemiplegic adult patients with refractory epilepsy associated with early middle cerebral artery infarcts.

PURPOSE: To study the outcome after hemispherectomy (HP) in a homogeneous adult patient population with refractory hemispheric epilepsy. METHODS: Fourteen adult patients submitted to HP were studied. Patients had to be at least 18 years old, and have refractory epilepsy, clearly focal lateralized seizures and unilateral porencephalus consistent with early middle cerebral artery infarct on magnetic resonance imaging (MRI). All patients were submitted to functional hemispherectomy. We analyzed age of seizure onset, age by the time of surgery, gender, seizure type and frequency, interictal and ictal electroencephalography (EEG) findings, MRI and IQ scores preoperatively; seizure frequency, drug regimen, and IQ outcome were studied postoperatively. RESULTS: Mean follow-up was 64 months. All patients had frequent daily seizures preoperatively. All patients had unilateral simple partial motor seizures (SPS); 11 patients had secondarily generalized tonic-clonic (GTC) seizures and five patients had complex partial seizures (CPS), preoperatively. All patients had hemiplegia and hemianopsia. Twelve patients had unilateral EEG findings, and in two epileptic discharges were seen exclusively over the apparently normal hemisphere. Twelve patients were seizure-free after surgery and two patients had at least 90% improvement in seizure frequency. Pre- and postoperative mean general IQ was 84 and 88, respectively. Five of the twelve Engel I patients were receiving no drugs at last follow-up. There was no mortality or major morbidity. CONCLUSIONS: Our results suggest that well-selected adult patients might also get good results after HP. Although good results were obtained in our adult series, the same procedure yielded a much more striking result if performed earlier in life.

Surgical outcome in patients with refractory epilepsy associated to MRI-defined unilateral mesial temporal sclerosis.

INTRODUCTION: Several pre-operative work-up protocols have been used while selecting epileptic patients for surgery among different centers. The relative value of the different available pre-operative tests is still under discussion. OBJECTIVE: We report on the surgical outcome obtained in patients with refractory temporal lobe epilepsy associated to mesial temporal sclerosis (MTS) and who were evaluated pre-operatively by interictal EEG and MRI alone. METHOD: Forty one patients with refractory unilateral temporal lobe epilepsy were evaluated using interictal EEG and MRI. MRI disclosed unilateral MTS in all patients. All patients had at least 4 interictal EEG recordings. All patients were submitted to cortico-amygdalo-hippocampectomy at the side determined by MRI. RESULTS: Interictal EEG showed unilateral epileptiform discharges compatible with MRI findings in 37 patients; in four out of the 41 patients, bilateral discharges were found. Mean follow-up time was 4.3+/-1.1 years. Thirty-nine patients (95.1%) were classified as Engels Class I (70.6% Engel I-A). Two patients (4.9%) were rated as Engel's Class II. All patients in whom bilateral discharges were found were in Engels Class I. Pathological examination showed MTS in all patients. CONCLUSION: It is possible to adequately select good surgical candidates for temporal lobe resection using MRI and interictal EEG alone. In patients with MRI-defined MTS we should expect a postoperative remission rate higher then 90%. The finding of MTS on MRI is the most important good prognostic factor after temporal lobe resection.

Intraoperative neurophysiological responses in epileptic patients submitted to hippocampal and thalamic deep brain stimulation.

PURPOSE: Deep brain stimulation (DBS) has been used in an increasing frequency for treatment of refractory epilepsy. Acute deep brain macrostimulation intraoperative findings were sparsely published in the literature. We report on our intraoperative macrostimulation findings during thalamic and hippocampal DBS implantation. METHODS: Eighteen patients were studied. All patients underwent routine pre-operative evaluation that included clinical history, neurological examination, interictal and ictal EEG, high resolution 1.5T MRI and neuropsychological testing. Six patients with temporal lobe epilepsy were submitted to hippocampal DBS (Hip-DBS); 6 patients with focal epilepsy were submitted to anterior thalamic nucleus DBS (AN-DBS) and 6 patients with generalized epilepsy were submitted to centro-median thalamic nucleus DBS (CM-DBS). Age ranged from 9 to 40 years (11 males). All patients were submitted to bilateral quadripolar DBS electrode implantation in a single procedure, under general anesthesia, and intraoperative scalp EEG monitoring. Final electrode's position was checked postoperatively using volumetric CT scanning. Bipolar stimulation using the more proximal and distal electrodes was performed. Final standard stimulation parameters were 6Hz, 4V, 300µs (low frequency range: LF) or 130Hz, 4V, 300µs (high frequency range: HF). KEY FINDINGS: Bilateral recruiting response (RR) was obtained after unilateral stimulation in all patients submitted to AN and CM-DBS using LF stimulation. RR was widespread but prevailed over the fronto-temporal region bilaterally, and over the stimulated hemisphere. HF stimulation led to background slowing and a DC shift. The mean voltage for the appearance of RR was 4V (CM) and 3V (AN). CM and AN-DBS did not alter inter-ictal spiking frequency or morphology. RR obtained after LF Hip-DBS was restricted to the stimulated temporal lobe and no contralateral activation was noted. HF stimulation yielded no visually recognizable EEG modification. Mean intensity for initial appearance of RR was 3V. In 5 of the 6 patients submitted to Hip-DBS, an increase in inter-ictal spiking was noted unilaterally immediately after electrode insertion. Intraoperative LF stimulation did not modify temporal lobe spiking; on the other hand, HF was effective in abolishing inter-ictal spiking in 4 of the 6 patients studied. There was no immediate morbidity or mortality in this series. SIGNIFICANCE: Macrostimulation might be used to confirm that the hardware was working properly. There was no typical RR derived from each studied thalamic nuclei after LF stimulation. On the other hand, absence of such RRs was highly suggestive of hardware malfunction or inadequate targeting. Thalamic-DBS (Th-DBS) RR was always bilateral after unilateral stimulation, although they somehow prevailed over the stimulated hemisphere. Contrary to Th-DBS, Hip-DBS gave rise to localized RR over the ipsolateral temporal neocortex, and absence of this response might very likely be related to inadequate targeting or hardware failure. Increased spiking was seen over temporal neocortex during hippocampal electrode insertion; this might point to the more epileptogenic hippocampal region in each individual patient. We did not notice any intraoperative response difference among patients with temporal lobe epilepsy with or without MTS. The relationship between these intraoperative findings and seizure outcome is not yet clear and should be further evaluated.

Speech preservation using a non-linear paradigm for determination of the extent of neocortical resection in patients with mesial temporal sclerosis submitted to cortico-amygdalo-hippocampectomy (CAH).

RATIONALE: The rationale for using a non-linear (proportional) paradigm for determining the extent of the neocortex to be removed in temporal lobe resection was based on anatomical and intra-operative cortical mapping findings. We present our results regarding speech preservation in patients submitted to CAH using the central artery as an anatomical landmark for determining the posterior border of neocortical resection. METHODS: Two hundred and fifty consecutive right-handed patients with left unilateral mesial sclerosis were studied. All patients were submitted to CAH under general anesthesia and without intraoperative electrocorticography. The posterior border of the lateral neocortical resection was defined by a line perpendicular to the temporal axis at the level of the central artery. RESULTS: Seven patients had transient (1-3 weeks; mean=9 days) receptive speech disturbance. There was no permanent speech deficit. Imaging documented edema or contusion at the posterior temporal cortical border in all patients who had transient speech deficits. The mean extent of cortical resection was 3.9 cm in adults and 3.1cm in kids. DISCUSSION: This is the first report in the literature discussing the use of a non-linear paradigm to determine the extent of lateral neocortical removal in this patient population. We found no permanent speech disturbances in this series. The non-linear approach used in this series proved to be safe and effective to avoid post-operative speech disorders. It was able to compensate for different brain and head sizes, and allowed smaller neocortical removal when compared to traditional linear approaches.

Outcome after cortico-amygdalo-hippocampectomy in patients with temporal lobe epilepsy and normal MRI.

RATIONALE: We describe seizure and neuropsychological outcome obtained after CAH in patients with TLE and normal MRI evaluated in the modern imaging era. METHODS: Forty-five adult consecutive patients with TLE and normal MRI were studied. All patients had neuropsychological testing, interictal and ictal EEG recordings and MRI. They were divided into two groups: Group 1 (n=18), included patients in whom non-invasive neurophysiological evaluation was lateralizing and Group 2 (n=27) included patients with non-lateralizing neurophysiological data who were submitted to invasive recordings. RESULTS: Seventy-seven percent of the Group 1 patients were rated as Engel I; 11% were rated as Engel II and 11% as Engel III. In Group 2, there were 57% of patients seizure-free, 26% in Engel II and 14% in Engel III. Pre-operatively, mean general IQ was 82 and 78 in Groups 1 and 2, respectively; post-operatively, mean general IQ was respectively 86 and 71. Some degree of verbal memory decline was noted in all patients submitted to dominant temporal lobe resection in both Groups 1 and 2. At last follow-up visit, 22% of Group 1 and 11% of Group 2 patients were receiving no antiepileptic drugs (AED). CONCLUSIONS: Our data showed that patients with TLE and normal MRI could get good surgical results after CAH although 60% of them would need invasive recordings and their results regarding seizure control and cognition were worse than those obtained in patients with MRI defined temporal lobe lesions. Caution should be taken in offering dominant temporal lobe resection to this subset of patients.

Centro-median stimulation yields additional seizure frequency and attention improvement in patients previously submitted to callosotomy.

RATIONALE: Deep brain stimulation (DBS) has been increasingly used in the treatment of refractory epilepsy over the last decade. We report on the outcome after thalamic centro-median (CM) DBS in patients with generalized epilepsy who had been previously treated with extended callosal section. METHODS: Four consecutive patients with generalized epilepsy who were previously submitted to callosal section and had at least 1 year of follow-up after deep brain implantation were studied. Age ranged from 19 to 44 years. All patients were submitted to bilateral CM thalamic DBS. Post-operative CT scans documented the electrode position in all patients. All patients had pre- and post-stimulation prolonged interictal scalp EEG recordings, including spike counts. Attention level was evaluated by means of the SNAP-IV questionnaire. The pre-implantation anti-epileptic drug regimen was maintained post-operatively in all patients. RESULTS: Post-operative CT documented that all electrodes were correctly located. There was no morbidity or mortality. Seizure frequency reduction ranging from 65 to 95% and increased attention level was seen in all patients. Interictal spiking frequency was reduced from 25 to 95%, but their morphology remained the same. There was re-synchronization of interictal discharges during slow-wave sleep in 2 patients. CONCLUSION: All patients benefit from the procedure. The CM seems to play a role in modulating the epileptic discharges and attention in these patients. On the other hand, it is not the generator of the epileptic abnormality and appeared not to be involved in non-REM sleep-related interictal spiking modulation.

Outcome after cortico-amygdalo-hippocampectomy in patients with severe bilateral mesial temporal sclerosis submitted to invasive recording.

INTRODUCTION: Although some degree of bilateral hippocampal involvement might be frequent in patients with temporal lobe epilepsy, severe bilateral mesial temporal sclerosis (MTS) is very rare. We present our experience while treating patients with severe bilateral MTS submitted to invasive recordings. METHODS: Nine adult patients were studied. All patients had simple and complex partial seizures. All patients had bilateral independent interictal temporal lobe spiking and non-lateralizing video-EEG findings. MRI showed severe bilateral MTS and no other brain lesion. All patients had severe verbal and non-verbal memory deficits. All patients were submitted to invasive recordings after bilateral subdural grids implantation. Cortico-amygdalo-hippocampectomy (CAH) was performed in all patients on the side suggested by invasive recording. Follow-up time ranged from 5 to 10 years. RESULTS: Invasive video-EEG showed exclusively unilateral seizure onset in seven patients; in two patients, seizures originating from both temporal lobes were found (80% of them originated from one side). Five patients were submitted to left and four to right CAH. Seven patients were rendered seizure-free after surgery (Engel I); the other two were rated as Engel II. There was no additional memory decline. There was no surgical morbidity or mortality. Pathological examination showed MTS in all patients. DISCUSSION: Good surgical outcome (77% seizure-free patients) could be obtained even in this apparently unsuitable group of patients. All patients benefit from the procedure. We did not see any cognitive decline in our patients with severe bilateral MTS. Patients with severe bilateral MTS would need invasive recordings despite any findings during surface video-EEG.

Extended, one-stage callosal section for treatment of refractory secondarily generalized epilepsy in patients with Lennox-Gastaut and Lennox-like syndromes.

PURPOSE: We report on the efficacy and safety of extended one-stage callosal section performed in a large and homogeneous series of patients. METHODS: Seventy-six patients with Lennox-Gastaut (n = 28) and Lennox-like (n = 48) syndrome were studied (42 male patients; mean age, 11.2 years). All patients had multiple seizure types per day, including atonic, tonic-clonic, atypical absence, myoclonic, and tonic seizures. All of them were developmentally impaired. The EEG showed intense secondary bilateral synchrony in all of them. All patients were submitted to an extended, one-stage, callosal section, leaving only the splenium intact. Mean follow-up time was 4.7 years. RESULTS: Worthwhile improvement (>50%) was noted in 69 of 76 patients; 52 patients had a > or =90% reduction in seizure frequency. Seven patients were seizure free after surgery. The seizure patterns most responsive to surgery were atonic (92%), atypical absence (82%), and tonic-clonic (57%) seizures. All patients had some degree of a transient acute postoperative disconnection syndrome. A consistent increase in attention level was observed postoperatively. CONCLUSIONS: We report one of the larger homogeneous series of patients submitted to callosotomy and are the first to report on the effectiveness and safety of performing extended callosal section in a single stage in this patient group. Extended callosal section should be considered a good palliative surgical option for suitable candidates. The increase in attention level was as useful as seizure control in improving quality of life of these patients.

Surgical outcome in patients with refractory epilepsy associated to MRI-defined unilateral mesial temporal sclerosis

INTRODUÇÃO: Protocolos diferentes têm sido utilizados para a investigação pré-operatória de pacientes epilépticos nos diferentes centros. No entanto, o valor relativo de cada teste disponível ainda é controverso na literatura.OBJETIVO: Relatamos os resultados cirúrgicos de pacientes com epilepsia refratária do lobo temporal associada a esclerose hipocampal (EH), cuja investigação pré-operatória consistiu exclusivamente de estudo de eletrencefalograma (EEG) inter-crítico e ressonância magnética (RM). MÉTODO: Foram estudados 41 pacientes com epilepsia refratária do lobo temporal, avaliados pré-operatoriamente somente por meio de EEG interictal e RM encefálica. Foram incluídos somente pacientes em quem a RM mostrava apenas EH unilateral. Todos pacientes possuíam ao menos quatro EEG inter-ictais. Todos os pacientes foram submetidos a córtico-amígdalo-hipocampectomia no lado demonstrado pela RM.RESULTADOS: A análise do EEG interictal revelou atividade epileptiforme unilateral, compatível com os achados da RM em 37 pacientes. Nos outros quatro pacientes, o EEG evidenciou comprometimento bilateral. O tempo médio de seguimento pós-operatório dos pacientes foi 4,3±1,1 anos. No período pós-operatório, 95,1% dos pacientes encontravam-se em classe I de Engel (70,6% em Engel I-A) e 4.9% em classe II de Engel. Todos os pacientes com achados bilaterais ao EEG estavam em classe I de Engel. O estudo anátomo-patológico das amostras cirúrgicas revelou EH em todos os pacientes. CONCLUSÃO: É possível selecionar bons candidatos à ressecção temporal utilizando-se apenas dados de RM e EEG inter-ictal. Nos pacientes com EH unilateral na RM, espera-se um índice de remissão pós-operatória das crises superior a 90%. O achado de EH na RM é, isoladamente, o maior fator indicativo de bom prognóstico após ressecção temporal.