Characteristics of headache associated with intractable partial epilepsy

PURPOSE: The association between headache (HA) and epilepsy is well known. However, few previous studies characterized HA types and head sensations (HSens) in large populations of individuals with well-defined forms of epilepsy. METHODS: To analyze the incidence of HA in such a group, we compare HA and non-HA patients to identify special predictive factors for HAs or migraine. We also investigate the pathologically verified group for possible correlations with HAs or migraine. One hundred consecutive patients undergoing presurgical evaluation for pharmacologically intractable partial epilepsy were interviewed. For each HA type, we inquired about lateralization, localization, quality of HA, and results of treatment. RESULTS: Periictal HAs were reported by 47 patients. Of those, 11 had preictal HA (PIHA), and 44 had postictal HA (PostHA). Eight patients had both PIHA and PostHA. Interictal HAs (InterHAs) were reported by 31 patients. Twenty-nine (62%) of 47 patients had frontotemporal HAs. Twenty-five patients had migraine-like HA without aura: 18 (60%) of 30 patients with temporal lobe epilepsy (TLE) and seven (41%) of 17 with extratemporal epilepsy (ETE). No correlation between pathology and presence of HA was found in 59 pathologically verified patients, except in four who had arteriovenous malformations (AVMs): three had and one did not have HAs. Eighteen patients had, in addition, poorly localized and ill-described HSens other than HAs. CONCLUSIONS: We confirm an association between focal epilepsy and HAs, including migraine without aura. This is true for both TLE and ETE. PIHA and even prodromal HA may be related to the epileptic discharge and may have lateralizing value. This association is not recognized by the current International Headache Society (IHS) classification. The presence of HA and migraine is not related to the underlying epileptogenic pathology except in patients with AVMs.     

Intracerebral masses in patients with intractable partial epilepsy

One hundred ninety patients were considered for surgical treatment of uncontrolled partial seizures. Twenty-seven (15%) had intracranial mass lesions detected during preoperative evaluation. Seizures were present for a mean of 10 years. Sixty percent of the patients had prior negative radiologic studies. The lesions included 19 neoplasms and 8 non-neoplastic structural (non-atrophic) lesions. Refractory simple partial seizures and a changing neurologic examination were more commonly associated with neoplasms. Treatment included biopsy and radiation, mass resection, or subtotal lobectomy. Although any form of treatment gave better seizure control, patients undergoing subtotal lobectomy had more than 95% reduction in seizures.     

Cortical resection with electrocorticography for intractable porencephaly-related partial epilepsy

PURPOSE: We evaluated the results of cortical resection of epileptogenic tissue for treatment of intractable porencephaly-related epilepsy. METHODS: We examined clinical features, electrophysiological data, surgical findings, and seizure outcomes after cortical resection in eight patients with intractable epilepsy related to porencephalic cysts. RESULTS: All eight patients had hemiparesis. Five retained motor function in the hemiparetic extremities; six retained visual fields. All had partial seizures, six with secondary generalization. Seven patients had simple and three had complex partial seizures (CPSs); two also had drop attacks. Four patients had multiple seizure types. Long-term scalp video-EEG (LVEEG) localized interictal epileptic abnormalities that anatomically corresponded to the cyst location in three patients. LVEEG recorded ictal-onset zones in five; these anatomically corresponded to the cyst location in three of the five. EEG recorded generalized seizures in two patients, hemispheric in one, and multifocal in two. Intraoperative electrocorticography (ECoG) revealed interictal epileptic areas extending beyond the margins of the cyst in seven patients. We resected ECoG-localized interictal epileptic areas completely in five patients and partially in two. Cortical resection was based on seizure semiology and LVEEG in one patient whose ECoG showed no epileptiform discharges. After a minimum follow-up of 1 year, six patients had excellent seizure outcome (Engel class I), and two had a >90% seizure reduction (Engel class III) without complications. CONCLUSIONS: Cortical resection guided by ECoG allows preservation of motor function and visual field and provides an effective surgical procedure for treatment of intractable epilepsy secondary to porencephaly.     

Gadolinium-DTPA-enhanced magnetic resonance imaging in intractable partial epilepsy

We performed magnetic resonance imaging (MRI), before and after the administration of gadolinium-DTPA (Gd-DTPA), in 23 patients with medically refractory partial epilepsy who subsequently had surgical treatment for their seizure disorder. In most patients, 61%, unenhanced and enhanced MRI studies were negative. In 26% the unenhanced image was positive, and the abnormality enhanced with Gd-DTPA. In 13% the unenhanced MR image was positive, but the abnormality did not enhance with Gd-DTPA. Gd-DTPA does not appear to increase the diagnostic yield of MRI in the presurgical evaluation of patients with partial epilepsy if the unenhanced MRIs are normal.     

Seizure outcome of intractable partial epilepsy in children

The intractable partial epilepsy outcome information is important in determining not only when epilepsy surgery evaluation should begin but also in deciding who would benefit and what is the likelihood of any benefit from surgery. Medical records of 50 children diagnosed with nontumor-related partial seizures, confirmed by video-electroencephalography (video-EEG), had at least one seizure per week and were followed for at least 2 consecutive years after video-EEGs were reviewed. There were 30 patients who continued with antiepileptic drug treatment after video-EEG. The seizure outcome analysis revealed a significant improvement of seizure control in the first year of follow-up but no difference between the first year and the following 3 years. Only 30% had excellent long-term outcome (seizure free or less than one seizure per 6 months). The presence of focal lesions on neuroimaging was the only risk factor of poor outcome. The other 20 patients underwent epilepsy surgery after video-EEG; 60% attained excellent outcome despite the fact that 90% had focal neuroimaging abnormality. Children whose partial epilepsy remained intractable after 1 year of antiepileptic drug treatment should be evaluated for candidacy of epilepsy surgery, particularly those who have focal lesions on neuroimaging.     

Spontaneous long-term remission of intractable partial epilepsy in childhood

Drug-resistant partial epilepsy in children often has a major impact on cognitive development, and early surgical intervention has been advocated to prevent adverse neurobehavioral effects of seizures in such patients. We report a 5-year-old boy who had cryptogenic partial epilepsy of right parietal origin as documented by ictal electroencephalogram (EEG) and glucose metabolism positron emission tomographic (PET) scan. His atonic seizures could not be controlled by multiple antiepilepsy drugs; therefore, cortical resection was scheduled. However, his seizures remitted spontaneously after 1 year of failed medical treatment. The epileptiform abnormality disappeared on the follow-up EEGs, and a glucose PET scan also normalized. This boy has fully retained his cognitive and motor functions and has remained seizure free in the past 4z\x years off medications.     

Magnetic resonance imaging in intractable partial epilepsy: correlative studies

A study was performed evaluating magnetic resonance imaging (MRI) in 35 patients with intractable complex partial seizures in whom computed tomographic (CT) scans showed no focal abnormalities. These results were correlated with positron emission tomography evaluation (PET), the electroencephalographic ictal onset, and findings during pathological examination. Seven patients had structural lesions that were epileptogenic, detected by MRI; the lesions were tuberous sclerosis, astrocytomas, or hamartomas. Three of these 7 patients underwent PET scanning, which was normal in all. Of 18 patients with mesial temporal sclerosis, 10 were shown by PET to have temporal lobe hypometabolism, though all 18 had normal MRI findings. The results indicate that MRI contributes information to that provided by CT and PET, by detecting nonsclerotic epileptogenic lesions of the temporal lobe.     

Evolution of neuropsychological changes after partial callosotomy in intractable epilepsy

Early neuropsychological changes following partial anterior callosotomy were evaluated in 15 patients with epilepsy (10 secondary generalized and 5 Lennox-Gastaut syndromes) by comparing their performances 1 month before surgery and then 15-20 and 90-100 days postoperatively without modifying the anticonvulsant treatment. The following neuropsychological and motor functions were tested: memory, attention, visuo-motor ability, posture, motor dexterity, language, praxis and gnosis. Social behaviour was also investigated. The main results are as follows: (i) most cognitive functions showed no significant variation; (ii) motor organization was still slightly impaired at the second check-up after the surgery; (iii) improvements in social behaviour and posture were frequently observed at the final evaluation. The analysis of individual cases highlights the influence of the extent of commissurotomy, lesions related to the surgical procedure and preoperative brain damage in determining the post-surgical profile. On this basis, the short-term neuropsychological cost of partial callosotomy appears to be low and seems to depend mostly on surgical parameters and brain conditions before the operation.     

Open study of pranlukast add-on therapy in intractable partial epilepsy

Innovative treatments of epileptic seizures are needed to improve the outcome of epilepsy. We studied the effect of pranlukast on seizure outcome in patients with intractable partial epilepsy. An open study was conducted to evaluate the clinical efficacy of 24-week pranlukast add-on therapy in 50 patients with intractable partial seizures. Serum concentrations of matrix metalloproteinase (MMP)-9 were determined using Biotrak Activity Assay System. Cytokines in cerebrospinal fluid (CSF) were measured by the BioPlex (BioRad) system and soluble TNF receptor1 (sTNFR1) in CSF was measured by the ELISA. Surface markers of lymphocytes in CSF were examined by cell-sorter. Seizure-free rate (SFR) was 13.6%, responder rate (RR) was 47.7%, and aggravation rate (AR) was 18.2% at the 13–24 week period after starting pranlukast. In patients with increased serum MMP-9 before pranlukast therapy (baseline), comparison of paired serum levels showed a significant decrease after pranlukast therapy. Baseline CSF levels of IL-1β and IL-6 were elevated in patients compared with disease controls. Of four patients with paired data, three (including a responder to pranlukast) showed decreased pro-inflammatory cytokines (IL-1β, IL-6, and TNFα), and four showed decreased sTNFR1, after pranlukast treatment, and only a responder had markedly decreased frequency of CD8+ T cells in CSF. Pranlukast reduces seizure frequencies probably by pleiotropic effects including normalization of MMP-9 in sera, reduced leakage of pro-inflammatory cytokines into CNS, and inhibition of extravasation of leucocytes from brain capillaries. Further investigations by double-blind control study and animal models are warranted.     

Remission of intractable partial epilepsy following implantation of intracranial electrodes.

Six patients with medically intractable partial epilepsy (IPE) underwent seizure localization with intracranial EEG using intracerebral or subdural electrodes. No surgical resection was performed, but all had seizure remission ranging from 11 months to 15 years. Invasive monitoring may rarely produce remission of IPE, possibly through interruption of seizure propagation pathways.     

The use of phenacemide for intractable partial complex epilepsy in children

Phenacemide monotherapy controlled seizures in four children with intractable partial complex epilepsy. No toxicity occurred. Previous fatalities caused abandonment of this antiepileptic drug, although it is not established that the drug was at fault.     

Multiple subpial transection for intractable partial epilepsy: an international meta-analysis

PURPOSE: Because the number and variety of patients at any single facility is not sufficient for clinical or statistical analysis, data from six major epilepsy centers that performed multiple subpial transections (MSTs) for medically intractable epilepsy were collected. METHODS: A meta-analysis was performed to elucidate the indications and outcome, and to assess the results of the procedure. Overall, 211 patients were represented with data regarding preoperative evaluation, procedures, seizure types and frequencies before and after surgery, postoperative deficits, and demographic information. Fifty-three patients underwent MST without resection. RESULTS: In patients with MST plus resection, excellent outcome (>95% reduction in seizure frequency) was obtained in 87% of patients for generalized seizures, 68% for complex partial seizures, and 68% for simple partial seizures. For the patients who underwent MST without resection, the rate of excellent outcome was only slightly lower, at 71% for generalized, 62% for complex partial, and 63% for simple partial seizures. EEG localization, age at epilepsy onset, duration of epilepsy, and location of MST were not significant predictors of outcome for any kinds of seizures after MST, with or without resection. New neurologic deficits were found in 47 patients overall, comparable in MST with resection (23%) or without (19%). CONCLUSIONS: These preliminary results suggest that MST has efficacy by itself, with minimal neurologic compromise, in cases in which resective surgery cannot be used to treat uncontrolled epilepsy. MST should be investigated as a stand-alone procedure to allow further development of criteria and predictive factors for outcome.     

Clinical outcome after complete or partial cortical resection for intractable epilepsy

This is the first epilepsy surgery series to analyze the definition of "completeness" of resection, based solely on results of chronic scalp and subdural EEG recording. When patients had complete removal of all cortical areas with ictal and interictal epileptiform discharges, the clinical outcome was usually good. When areas with epileptiform discharges were left behind, good outcome was significantly less frequent. This correlation between complete resection and good outcome was independent of the presence or absence of CT-detected structural lesions or sharp waves on post-resection electrocorticography. These results support completeness of resection, defined by prolonged extraoperative EEG, as an important factor in seizure surgery.     

The use of stereotactic radiosurgery to treat intractable childhood partial epilepsy

PURPOSE: Although conventional surgery is presently used to treat seizures of temporolimbic and neocortical origin, deep-seated lesions are often associated with morbidity. Stereotactic radiosurgery is a noninvasive procedure that effectively treats patients with vascular malformations and brain tumors, but its efficacy for epileptogenic foci is limited, especially in children. METHODS: Between 1995 and 1999, four candidates who had medically uncontrolled seizures and localized seizure foci were selected for stereotactic radiosurgery, with a mean age of 9.75 years at the time of surgery (range, 4-17 years). Seizure foci were identified on the basis of ictal and interictal video-EEG. Magnetic resonance (MR) images were obtained before and after surgery. Ictal single-photon emission computed tomography (SPECT) was performed by using stabilized hexamethyl-propyleneamine oxime (HMPAO; 300 microcuries/kg) with early injection after electrographic ictal onset. The clinical features of the patients are given. All radiosurgical procedures were performed with the gamma knife unit with the Leksell stereotactic frame, stereotactic MRI imaging, and the Gamma Plan workstation. Seizure outcome was scored according to Engel's classification. RESULTS: Two patients had hypothalamic hamartoma (HH), and two had neocortical epilepsy. At mean follow-up of 39.2 months (range, 26-69 months), two patients were seizure free, one with a HH and one with a suggestive developmental tumor in the insular cortex by MRI findings. The other patient with HH had 90% reduction of seizures. One patient with a widespread seizure focus that involved the motor strip was unimproved. The two patients with HH also exhibited markedly improved neurobehavioral status after surgery. There were no significant complications of radiosurgical therapy. CONCLUSIONS: Our findings suggest that gamma knife surgery is a potentially valuable treatment modality for children with medically intractable epilepsy due to a well-localized seizure focus that is difficult to excise by conventional techniques or for whom they are deemed unsuitable. More widespread application in childhood epilepsy should be investigated in larger series.     

Oligoastrocytoma presenting with intractable epilepsy.

OBJECTIVE: Oligoastrocytomas (OA) are mixed gliomas with distinct oligodendroglial and astrocytic neoplastic components. Very little about OA has been reported in the intractable epilepsy population. METHODS: We undertook a retrospective review of 923 patients who underwent resective surgery for intractable epilepsy between 1996 and 2004. RESULTS: 6/923 (0.7%) patients were diagnosed with OA. Five patients were female, one was male. Median age at diagnosis was 25 years (range 19-44 years). Tumors arose from the left side in all patients and from the temporal lobe in five patients. Three patients had complex partial seizures. Median length of refractory epilepsy prior to surgery was 10.5 years (range 1-28 years), and the median number of antiepileptic drugs used was 2 (range 1-10). Preoperative WADA testing for language and memory localization was done for three patients; preoperative stereotactical localization was done for three patients. Surgical pathology revealed low-grade OA (WHO II) in five patients, and anaplastic OA in one. There were no surgical complications, clinical or radiographic tumor recurrence at a mean follow up period of 3.2 years (range 2-8). Excellent seizure freedom was achieved in 5/6 patients. CONCLUSION: As a result of our small sample size, general conclusions may be imprecise, but this review suggests that OA behave similar to other tumors related to intractable epilepsy: they usually have a preoperative seizure course of many years, an excellent rate of seizure-freedom following surgery, and are in general, low-grade tumors with an indolent course for which serial imaging is sufficient follow-up.     

大脑半球离断术治疗儿童难治性癫痫

目的 探讨大脑半球离断术治疗儿童难治性癫痫的手术方法及疗效.方法 2007年8月至2011年10月北京三博脑科医院进行12例大脑半球离断手术.经侧裂半球离断术1例,经纵裂半球离断并颞叶切除4例,中央区造瘘半球离断并颞叶切除5例,颞叶、岛盖切除岛周半球离断术2例.手术年龄平均7.6岁(2.1 ～11.9岁).结果 术后随访0.5 -4.5年,Engel Ⅰ级10例,EngelⅡ级1例,Engel Ⅲ级1例.术后因离断不完全再次行离断手术1例.术后患者的认知及生活能力较术前提高,无脑积水等严重神经功能损伤及死亡病例.结论 大脑半球离断术治疗儿童半球性难治性癫痫完全缓解率83％ (10/12),手术疗效确定,是治疗儿童半球病变性癫痫的安全、有效的方法.