Adult-onset epilepsy associated with dysembryoplastic neuroepithelial tumors.

RATIONALE: Dysembryoplastic neuroepithelial tumors (DNET) are benign, localized lesions that typically cause localization-related epilepsy of childhood onset. Although excellent seizure outcomes are expected following surgical resection of focal, benign lesions, reports in pediatric epilepsy series suggest that this may not be the case with DNETs, which may exhibit complex and often multifocal epileptogenesis. We report the characteristics and surgical outcome of an adult- and childhood-onset cohort with this condition. METHODS: Retrospective cohort of 23 patients seen at two major epilepsy centers, with localization-related epilepsy associated with histopathologically demonstrated DNETs. We assessed clinical, electrographic and surgical outcome features in patients with adult- and childhood-onset epilepsy. We were particularly interested in the level of congruence of EEG and MRI data and the need for intracranial recordings. We evaluated seizure outcomes at last follow-up. RESULTS: The mean age was 33.3 years (range: 5-56 years). Ten patients had adult-onset epilepsy. Thirteen patients (57%) had simple partial, 21 (91%) had complex partial, 16 (70%) had secondarily generalized seizures and 5 patients had only simple partial seizures. Status epilepticus did not occur. Non-enhancing lesions on MRI were located in the temporal lobe in 17 patients, the frontal lobe in 3 patients and the parietal/occipital region in 2 patients. One patient had a DNET that involved both frontal and temporal areas. Ictal scalp EEG and MRI were congruent in 17 patients (74%). Eleven patients (48%) underwent lesionectomies, while the rest required some resection of extralesional cortex as well. Five patients required intracranial EEG. There was no association with cortical dysplasia. Seventeen patients (74%) had an Engel class 1 outcome, in a follow-up period that ranging from 5 to 98 months. CONCLUSIONS: We found no difference in outcomes between adult- and childhood-onset cases. Although epileptogenicity was complex, congruence between electro-clinical and neuroimaging studies was high and allowed good surgical outcomes at 1 year of follow-up.     

Ganglioglioma and Intractable Epilepsy: Clinical and Neurophysiologic Features and Predictors of Outcome After Surgery

Summary: Purpose: To review the clinical, neurophysiologic, and radiological data of patients with ganglioglioma who had undergone evaluation and surgery in our Epilepsy Program.
Methods: The medical and neurophysiologic records of 38 patients with intractable epilepsy and ganglioglioma were re- viewed. Data underwent statistical analysis.
Results: There were 28 temporal and 10 extratemporal resections, with a mean age at seizure onset of 10.5 years and mean age at surgery of 22 years. Five tumor resections performed earlier were recorded. Twenty-nine patients had auras and 20 had secondarily generalized seizures. All 28 patients with temporal tumor had complex partial seizures. Preoperative MRI demonstrated the tumor in 36 of 36 patients: 17 of 29 demonstrated gadolinium enhancement, and 17 of 36 had mass effect. Scalp interictal sharp waves were present in 32 patients, and in 15 they were multiregional. In two patients, scalp EEG seizure onset was from the hemisphere contralateral to the tumor. Postoperatively, 79% of patients (30 of 38) were seizure-free (Engel's class I) at 6 months, 72% at 1 year (26 of 36), and 63% at 2 years (20 of 32). Excellent outcome was associated with a lower age at operation (p = 0.008), shorter duration of epilepsy (p = <0.01), absence of generalized seizures (p = <0.01), and no epileptiform discharges on a postoperative EEG (p = 0.01).
Conclusions: Good surgical outcome is expected in patients with ganglioglioma despite years of medically resistant seizures. Good outcome may be achieved despite EEG findings that may conflict with tumor location, and is more likely when surgery is performed relatively soon after epilepsy onset.     

Dysembryoplastic neuroepithelial tumour: the Western Australian experience

Dysembryoplastic neuroepithelial tumour (DNET) is a rare tumour that is usually seen in the context of epilepsy surgery. The purpose of this study was to provide a profile of DNET in an unbiased population. The 2020 brain tumours diagnosed in Western Australia in the 10-year period 1982-1991 were reviewed; 5 cases of DNET were identified. All patients were neurologically intact and presented with complex partial seizures from a young age. Surgery was performed for tumour resection or biopsy in all cases; no patient underwent surgery specifically for epilepsy control. The lesions were intracortical, multinodular and were composed of astrocytes, oligodendrocytes and neurons. Three patients underwent total or subtotal resection and their seizures were either abolished or reduced in frequency. Two patients had biopsies only and were clinically unchanged. There has been no evidence of recurrence in up to 8 years' follow-up. The diagnosis of DNET is important because it is a surgically treatable benign tumour.     

Post-ictal psychosis after right temporal lobectomy.

Of 298 patients who had temporal lobectomies for intractable epilepsy, 4 (1.3%) developed post-ictal psychosis for the first time after surgery. All were males of normal intelligence with no pre-operative psychiatric disorder. Psychosis followed both complex partial and generalised seizures. The psychotic symptoms showed polymorphic features. Right temporal lobectomy may increase the susceptibility to post-ictal psychosis in patients who are not seizure free after surgery, particularly in the first post-operative year.     

Acute Psychosis During Intracranial EEG Monitoring: Close Relationship Between Psychotic Symptoms and Discharges in Amygdala

PURPOSE: This report examined the underlying mechanism of psychosis associated with epilepsy. METHODS: An adult patient with epilepsy manifesting acute psychosis during long-term EEG monitoring is presented, together with a literature review on this subject. RESULTS: A 25-year-old woman with intractable temporal lobe epilepsy developed acute psychosis while she underwent long-term intracranial EEG monitoring. After a clustering of seizures, she manifested psychotic symptoms including hallucination, stupor, and repeated fear. The transition of psychotic symptoms corresponded to the changes in frequency and morphology of seizure discharges restricted to the left amygdala. Improvement of psychosis coincided with disappearance of seizure discharges. CONCLUSIONS: This case confirmed a close relationship between psychotic symptoms and seizure discharges in the left amygdala. It is suggested that paroxysmal bombardment of the medial temporal lobe structure may be a pathogenetic factor of acute psychosis associated with epilepsy.     

Surgical Outcome in Patients with Epilepsy with Occult Vascular Malformations Treated with Lesionectomy

Summary: Purpose: This retrospective study reports the long-term surgical outcome of patients with medically refractory epilepsy and vascular malformations who were treated with lesionectomy. A detailed analysis of surgical failures had been performed in an attempt to define predictors of surgical success and failure.
Methods: Fifteen patients with medically intractable epilepsy and angiographically occult vascular malformations (AOVMs) were treated surgically with lesionectomy at Duke University Medical Center. Lesionectomy consisted of removal of the AOVM and surrounding hemosiderin-stained brain only, without the use of electrocorticography (ECoG) to guide resection.
Results: Eleven (73%) patients are seizure free after lesionectomy. Three showed no significant improvement, and one patient died, presumably after a seizure. Age of onset, duration of seizures, age at resection, and gender did not affect outcome. All patients with neocortical AOVMs in whom EEG findings correlated with the site of the lesion were seizure free after lesional resection. Treatment failures were associated with the presence of multiple intracranial lesions, poorly localized or diffuse EEG findings, discordant positron emission tomography (PET) imaging, or with a lesion in close proximity to the limbic system.
Conclusions: Lesionectomy, with removal of surrounding hemosiderin-stained brain, can be considered the procedure of choice in carefully selected patients with epilepsy with occult vascular malformations.     

Postictal psychosis after temporal lobectomy

Three of 282 consecutive patients who had temporal resections for intractable epilepsy developed postoperative postictal psychosis. These three patients had seizure recurrence contralateral to the resection, whereas none of the patients with ipsilateral seizure recurrence developed any psychiatric symptoms after surgery. Two had left amygdalo-hippocampectomy and one right temporal lobectomy. The de novo occurrence of postoperative postictal psychosis is a well-defined complication of surgery for temporal lobe epilepsy, and may relate to contralateral epileptogenesis.     

Psychomotor epilepsy and psychosis. II PHYSICAL ASPECTS

In a controlled investigation the clinical findings in 96 patients with paranoid/hallucinatory psychosis and partial epileptic seizures with complex symptoms, were compared with the findings in 96 control patients with the same type of epilepsy without psychosis of median 24 years' duration. The median age at onset of psychosis was 34 years, after epilepsy of median 21 years' duration. The seizure frequency of complex, partial seizures was significantly lower in the psychotic group, while the frequency of generalized seizures did not differ. A significant preponderance in the psychotic group of left-handed patients, etiological factors and neurological signs reflecting organic damage, and seizures of automatic behaviour indicates that epileptic psychoses are caused by structural lesions affecting the deep parts of the temporal lobe.     

Psychomotor epilepsy and psychosis

The EEG findings in waking, sleep, and sphenoidal electrode recordings in 96 patients with partial epileptic seizures with complex symptoms, who, after a median interval of 18 years developed paranoid/hallucinatory psychosis, were compared with the findings from a group of patients without psychosis, who had had the same type of epilepsy in median 24 years. There were no significant differences between the two groups with regard to median age at onset of epilepsy or complex partial seizures, age, or duration of epilepsy at time of examination. the psychotic patients had a significant preponderance of temporal medio-basal spike foci, recorded on the sphenoidal electrode, indicating deep temporal lobe dysfunction as an important factor in the pathogenesis of psychosis. A significant higher frequency of bilateral and multiple spike foci, together with a significant frequency of slow-wave admixture to the waking background EEG activity, indicated more extensive and severe epileptogenic lesions in the psychotic patients. There was no correlation between psychosis and unilateral EEG foci in either temporal lobe.     

Surgical treatment of temporal lobe epilepsy with interictal psychosis: results of six cases

We describe the postsurgical outcome of six patients with medically intractable temporal lobe epilepsy and interictal psychosis who underwent temporal lobe resection. All patients were submitted to a comprehensive presurgical investigation, including prolonged video-EEG monitoring. Despite their psychotic disorders, all patients were able to provide informed consent and we were able to complete the investigation of all cases. Surgical complications occurred in two cases. Seizure outcome was Engel class I (free from incapacitating seizures) in all except one patient. There was no worsening of their psychoses. Until now, there has been relative improvement in the mental conditions of five patients. Although psychosis has been considered by some authors as a contraindication to epilepsy surgery, with appropriate psychiatric intervention, patients with refractory epilepsy and chronic interictal psychosis may be submitted to prolonged presurgical investigation and undergo surgery successfully.     

颅内节细胞胶质瘤所致癫痫的手术预后相关因素分析

目的探讨颅内节细胞胶质瘤（GG）所致癫痫患者的手术预后相关因素。方法回顾性分析我院2008年4月至2012年12月收治的5l例GG所致癫痫患者的临床资料。结果51例患者中68．6％（35／51）为药物难治性癫痫,72．5％（37／51）的患者肿瘤位于颞叶;70．6％（36／51）行肿瘤全切,29．4％（15／51）行次全切除。74．5％（38／51）患者合并局灶性皮质发育不良（FCD）。随访1。5年,84．3％（43／51）的患者癫痫达到EngelI级。肿瘤全切患者的癫痫缓解（EngelI级）率为94．4％（34／36）,次全切的缓解率为60．0％,二者差异显著（P〈0．05）。结论GG通常为低级别肿瘤,手术全切除患者癫痫控制率较高;GG常合并FCD,为了取得更好的癫痫控制率,手术不仅要切除GG,也应将周围FCD切除。     

Spitting Automatism in Complex Partial Seizures: A Nondominant Temporal Localizing Sign?

Summary: Purpose: Spitting as an ictal automatism has been rarely reported. We aimed to establish its potential lateralizing and localizing significance.
Methods: Review of patients undergoing surgery for intractable epilepsy at two comprehensive epilepsy centers.
Results: Five patients were found who had spitting as a stereotyped automatism of their complex partial seizures. All had evidence of right temporal ictal onset and underwent resective surgery. Two had tumors; one, a cavernous angioma; one, hippocampal gliosis, and one, hippocampal sclerosis. We found no instances of ictal spitting in patients with left hemisphere onset.
Conclusions: Spitting as an automatism in complex partial seizures, although uncommon, may be a localizing sign to the nondominant temporal lobe.     

Violence and Epilepsy: A Close Relation Between Violence and Postictal Psychosis

Summary: Purpose: We investigated the incidence of well-directed violent behavior and suicide attempts in patients with temporal lobe epilepsy, with special attention to postictal psychosis.
Methods: We compared 57 episodes of postictal psychosis with 62 episodes of acute interictal (or alternative) psychosis and with 134 complex partial seizures. All patients were matched for age and for age at onset of seizures.
Results: The incidence of well-directed violent behavior against human beings was significantly higher (23%) during postictal psychotic episodes than during acute interictal episodes (5%) and postictal confusion (1%). Suicide attempts were also more frequent during postictal psychosis (7%) than during either acute interictal psychosis (2%) or postictal confusion (0%).
Conclusions: Our study showed that well-directed violent and self-destructive behavior was not a feature of epileptic psychosis in general but a specific hallmark of postictal psychosis.     

Intractable Epilepsy in a Population-Based Series of Mentally Retarded Children

Summary: Purpose: The characteristics of intractable epilepsy were analyzed in a population-based study of active epilepsy in mentally retarded children aged 6–13 years.
Methods: Diagnostic registers, EEG laboratory registers, and registers for the Education of the Subnormal were searched. Medical files were scrutinized. Clinical examinations and interviews with parents and caregivers or both were performed. EEG recordings, computed tomography (CT) and magnetic resonance imaging (MRI) of the CNS were reevaluated.
Results: Forty-five percent (44 of 98) of the children with mental retardation (MR) and active epilepsy had intractable seizures, defined as one or more seizures every day or week. The median age at onset was 0.8 years, as compared with 3.0 years for those with controlled epilepsy. Predictive factors for frequent seizures were the number of seizure types, severe MR, status epilepticus (SE) and tonic seizures. Epileptiform EEG activity was present in 91%, and focal activity in 65%. Brain lesions were detected on CT and MRI in 70%, with generalized lesions in 60%. Concurrent focal epileptiform activity and focal brain lesions on CTIMRI were detected in 26%. The percentages and prevalence rates for infantile spasms (IS) and Lennox-Gastaut syndrome (LGS) were 18% (0.25 in 1,000) and 7% (0.06 in 1,000), respectively. One of 8 children with IS had had previous neonatal seizures, 3 had SE and 1 later developed LGS.
Conclusions: Children with MR and intractable epilepsy have a high frequency of severe MR and additional major neuroimpairments. EEG recordings frequently showed focal changes despite generalized lesions in neuroradiology.     

Postoperative psychoses in epileptic patients after temporal lobectomy

Introduction – Psychosis is the most severe psychiatric complication after epilepsy surgery. Patients and methods – We evaluated postoperatively at 1 year the psychoses of a series of 57 adult patients with intractable epilepsy who underwent temporal lobe surgery. Results – Five patients (8.8%) developed postoperative psychosis. Two (3.5%) of these 5 revealed postictal psychotic episodes in connection with persisting seizures, both of them had had similar episodes even preoperatively. Two patients (3.5%) exhibited a definite and one patient (1.8%) a probable de novo schizophrenia. Conclusion – Our findings clearly emphasize the need for careful postoperative psychiatric follow-up for patients with temporal lobectomy.     

Susceptibility weighted imaging in the diagnostic evaluation of patients with intractable epilepsy

Aim:   Dedicated magnetic resonance imaging (MRI) protocol can diagnose epileptogenic abnormalities in patients with intractable epilepsy. However, it is not sufficiently sensitive to detect small calcified lesions that may result from infections, tumors, or vascular malformations. This study aims to study the impact of the addition of T2*gradient echo/susceptibility weighted imaging (T2*GRE/SWI) sequence to a dedicated MRI protocol.
Method:   One hundred thirty-seven patients with intractable epilepsy underwent MRI using conventional epilepsy protocol with addition of T2*GRE/SWI sequence. Comparison of the images with and without these sequences was done for detection of calcified abnormalities/vascular abnormalities. In patients with calcified lesions, MRI findings were correlated either with histopathology or computerized tomography (CT) to confirm the presence of calcification.
Results:   In 16 patients the sequence gave additional information compared to conventional imaging protocol. The sequence helped in better characterization of lesions in all patients . In three patients it helped in detecting the lesion and in another three it appeared useful as it best characterized the lesions. Additional lesions were detected in two patients with old calcified granulomas. Important additional information was supplied in four patients, whereas in the remaining patients lesion conspicuity was increased.
Conclusion:   T2*GRE/SWI sequence should form part of routine epilepsy protocol as it increases sensitivity by detecting occult calcified lesions or vascular malformations that may be responsible for the patient's seizures. This is especially important in patients from developing countries who have post-infective calcified lesions responsible for seizures and who undergo only MRI as the imaging modality for intractable seizures.     

Epilepsy surgery in children with developmental tumours

We report our experience regarding evaluation, surgical treatment and outcomes in a population of 21 children with histopathologically confirmed developmental tumours [nine dysembryoplastic neuroepithelial tumours (DNET), ten gangliogliomas (GG) and two gangliocytomas (GC)] and related epilepsy, analyzing video-EEG, MRI and neuropsychological data, before and after surgery.Most children had focal epilepsy correlating well with lesion location. One patient had epileptic spasms and generalized discharges. Tumours were located in the temporal lobe in 13 patients. Mean age at surgery was 11.16 years. Postsurgical MRI showed residual tumour growth in one DNET. One child had a recurrent ganglioglioma with anaplastic transformation. At latest follow-up (mean 4.68 years) 95.2% of patients were seizure-free and no significant neuropsychological declines were observed. Evidence from our study suggests that, in this setting, surgery should be performed before criteria for refractory epilepsy are met, particularly in cases with early seizure onset, in order to optimize cognitive outcome.     

Psychiatric co-morbidity in 75 patients undergoing epilepsy surgery: lack of correlation with pathological findings

BACKGROUND: Psychiatric disorders may occur in patients with intractable partial epilepsy after surgical treatment. Previous reports attributed the presence of psychological adverse events to specific pathological entities such as dysembryoplastic neuroepithelial tumors (DNETs) and gangliogliomas. The rationale for the present study is to evaluate the importance of the surgical pathology in individuals undergoing epilepsy surgery. METHODS: The patients were separated into three groups based on the surgical pathology: group I ganglioglioma (N=25), group II DNETs (N=25), and group III mesial temporal sclerosis (N=25). Thirteen of the 75 patients (17.3%) had a preexisting psychiatric disorder. The most common preoperative psychiatric diagnosis was depression (N=4). Sixty-three of the lesions (84%) were restricted to the temporal lobe. The operative strategy included resection of the lesion and epileptogenic cortex. Sixty-two of the 75 patients (83%) were rendered seizure-free. RESULTS: Eight of the 75 patients (10.7%) had an acquired psychiatric illness following surgical treatment. A mood disorder developed in three patients after surgery. No statistical difference emerged in preoperative psychiatric co-morbidity (no group difference; p=1.0) or in newly diagnosed postoperative psychiatric disease (group I vs. II, p=0.67; group I vs. III, p=1.0; and group II vs. III, p=0.67) within the three surgical pathology groups. CONCLUSION: This study indicates that the presence of psychiatric disease before and after surgery for intractable partial epilepsy, predominantly of temporal lobe origin, was independent of the pathological findings.     

Prolonged preictal psychosis in refractory seizures: a report of three cases

Seizures and psychosis coexist in a large number of patients with epilepsy, and a significant amount of research on their relationship has been published. There are several reports and reviews on postictal and interictal psychosis in patients with epilepsy. We describe three patients with refractory temporal lobe epilepsy, each of whom presented with a history of episodic psychosis that preceded almost all habitual seizures and, thus, served as a useful warning symptom. All three patients had intractable left complex partial seizures; two had right mesial temporal sclerosis, and the third had a gliotic area in the right frontotemporal region on MRI. This is the first report of psychosis preceding seizures. The literature on seizure anticipation, as well as on the complex relationship between seizures and psychosis, is also reviewed.     

Schizophrenia-like psychosis arising de novo following a temporal lobectomy: timing and risk factors

OBJECTIVES: To clarify risk factors for the development of schizophrenia-like psychotic disorders following temporal lobectomy, and to explore the possibility that the early postoperative period is a time of high risk for the onset of such chronic psychotic disorders. METHODS: Patients who developed schizophrenia-like psychosis were identified from a series of 320 patients who had a temporal lobectomy for medically intractable epilepsy. The relationship of their disorders to both the operation and subsequent seizure activity was examined. Using a retrospective case-control design, risk factors for the development of schizophrenia-like psychosis were established. RESULTS: Eleven patients who developed schizophrenia-like psychosis postoperatively were identified and compared with 33 control subjects who remained free of psychosis postoperatively. The onset of de novo psychotic symptoms was typically in the first year following the operation. No clear relationship between postoperative seizure activity and fluctuations in psychotic symptoms emerged. Compared with the controls, patients who become psychotic had more preoperative bilateral electroencephalogram (EEG) abnormalities, pathologies other than mesial temporal sclerosis in the excised lobe and a smaller amygdala on the unoperated side. CONCLUSIONS: Temporal lobectomy for medically intractable epilepsy may precipitate a schizophrenia-like psychosis. Patients with bilateral functional and structural abnormalities, particularly of the amygdala, may be at particular risk for the development of such psychoses.