Carmen L. Jorge

Cerebellar hemorrhage after supratentorial surgery for treatment of epilepsy: report of three cases.

OBJECTIVE AND IMPORTANCE We report three cases of cerebellar hemorrhage complicating supratentorial craniotomies for the treatment of epilepsy. In a literature review, we identified only four similar cases of cerebellar hemorrhage after temporal lobectomy for the treatment of epilepsy. CLINICAL PRESENTATION AND RESULTS Three young and otherwise healthy patients underwent frontal, occipital, and temporal resections for the treatment of refractory epilepsy. The hemorrhage manifested as peduncular tremor, ataxia, and decerebrate posturing presenting early in the postoperative period. The diagnosis was established by computed tomography and/or magnetic resonance imaging. Benign outcomes were observed for all patients. CONCLUSION Based on the available data, it is our opinion that brain dislocation resulting from excessive intraoperative cerebrospinal fluid drainage is a possible mechanism for this rare complication of supratentorial craniotomy. The overdrainage seems to be less hazardous when the procedure is performed for the removal of space-occupying mass lesions. In contrast, the resection of nonexpanding tissues, such as in lobectomies for the treatment of epilepsy, may be an additional risk factor, because the incidence of this complication seems to be higher in these situations.

Neuroimaging Findings in Rasmussen's Syndrome

Rasmussens syndrome is a progressive childhood disease of unknown cause characterized by severe epilepsy, hemiparesis, mental deterioration, inflammation of one cerebral hemisphere, and brain atrophy. Computed tomography, single‐photon emission computed tomography (SPECT), and magnetic resonance (MR) neuroimaging findings of 8 patients with pathologically confirmed Rasmussens syndrome were evaluated retrospectively. All patients showed a predominance of the atrophy in the temporo‐insular region and cerebral hemispheric alterations on MR images in a similar extension as seen on SPECT studies. Focal increase in regional cerebral blood flow was observed in the 4 patients presenting with epilepsia partialis continua at the time of hexamethyl‐propyleneamineoxime injection. Extensive cortical hypoperfusion was noted in the other 4 patients who received the injection during the interictal state. Cerebellar functional abnormalities were present in 6 patients, 2 of them with structural damage.

Double pathology in Rasmussen's encephalitis : Etiologic considerations

In a 7‐year‐old girl with epilepsia partialis continua (EPC) involving the left face, arm, and leg for 1 year, serial neuroimaging studies showed progressive, brain atrophy. Because medical treatment was ineffective, she underwent a large fronto‐temporal surgical resection. Neuropathological examination showed loss of lamination and dysplastic neurons, gliosis, microglial nodules, and perivascular cuffing. Such “double pathology” (dysgenesia and a chronic inflammatory process) may have implications for the pathophysiology of Rasmussens syndrome.

GRAPH THEORETICAL CHARACTERIZATION AND TRACKING OF THE EFFECTIVE NEURAL CONNECTIVITY DURING EPISODES OF MESIAL TEMPORAL EPILEPTIC SEIZURE

Via a detailed case study of mesial temporal lobe epilepsy, we show that a method of determining the direction of information flow among signals is able to provide focal localization via the simultaneous analysis of multiple EEG channels. This determination is accomplished by representing information flow direction via directed graphs, where focal electrodes are associated with high observed rates of pertinence to strongly connected subgraphs. Further clinical support to this finding is provided by results for an additional 9 cases of focal epilepsy cases. The graph theoretical approach is a tool for describing and analyzing the effective connectivity dynamics behind epileptic seizures and may provide a common language for studying other complex dynamic relationships between neural structures.

Epilepsy syndromes associated with hypothalamic hamartomas

PURPOSE Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and their relationship with the epileptic syndrome in HH. METHODS We documented all current seizure types in six adult patients with HH-epilepsy with video-EEG monitoring, characterized clinical-electrographic features of gelastic and non-gelastic seizures and correlated these findings with cognitive profile, as well as MRI and ictal SPECT data. RESULTS Only four seizure types were seen: GS, complex partial (CPS), tonic seizures (TS) and secondarily generalized tonic-clonic seizures (sGTC). An individual patient presented either CPS or TS, but not both. GS progressed to CPS or TS, but not both. Ictal patterns in GS/TS and in GS/CPS overlapped, suggesting ictal spread from the HH to other cortical regions. Ictal SPECT patterns also showed GS/TS overlap. Patients with GS-CPS presented a more benign profile with preserved cognition and clinical-EEG features of temporal lobe epilepsy. Patients with GS-TS had clinical-EEG features of symptomatic generalized epilepsy, including mental deterioration. CONCLUSIONS Video-EEG and ictal SPECT findings suggest that all seizures in HH-related epilepsy originate in the HH, with two clinical epilepsy syndromes: one resembling temporal lobe epilepsy and a more catastrophic syndrome, with features of a symptomatic generalized epilepsy. The epilepsy syndrome may be determined by HH size or by seizure spread pattern.

Emotional trauma and abuse in patients with psychogenic nonepileptic seizures.

A history of childhood trauma and the presence of dissociative phenomena are considered to be the most important risk factors for psychogenic nonepileptic seizure disorder (PNESD). This case-control study investigated 20 patients with PNESD and 20 with temporal lobe epilepsy (TLE) diagnosed by video/EEG monitoring who were matched for gender and age. Patients with both conditions were not included in the study. Groups were evaluated for age at onset and at diagnosis, worst lifetime weekly seizure frequency, trauma history, and presence of dissociative phenomena. Age at onset (P=0.007) and age at diagnosis (P<0.001) were significantly higher in the PNESD group than the control group, as were the scores on the Dissociative Experiences Scale (P<0.001) and Childhood Trauma Questionnaire (P=0.014). Only the differences in scores on the Childhood Trauma Questionnaire subscales Emotional Neglect (P=0.013) and Emotional Abuse (P=0.014) reached statistical significance. Dissociative phenomena and a reported history of childhood trauma are more common in patients with PNESD than in those with TLE. However, only emotional neglect and abuse were associated with PNESD in this study.

Serial and prolonged EEG monitoring in anti-N-Methyl-d-Aspartate receptor encephalitis

OBJECTIVE To describe serial electroencephalographic (EEG) findings of three patients with anti-NMDAR encephalitis. METHODS Three women (age 15-34years) with confirmed anti-NMDAR encephalitis underwent serial EEG recordings. Continuous EEG for 72h was performed in one case and 3-day video-EEG monitoring was obtained in two cases. RESULTS Generalized rhythmic delta activity (GRDA) was found in all patients. GRDA persisted for hours, but was not continuous on a 24-h EEG recording, disclosed no frequency, voltage or field evolution, and was not seen on the first EEG of two patients. Extreme delta brush was noted in two patients who presented more severe disease. One patient presented seizures, which were electrographically and clinically different from the GRDA pattern and from dyskinetic movements. CONCLUSIONS Serial or continuous EEG may be necessary to detect GRDA in anti-NMDAR patients. To avoid unnecessary treatment, this pattern should not be interpreted as indicative of ictal activity, unless there is evidence of its ictal nature. SIGNIFICANCE Our findings may contribute to the diagnosis of anti-NMDAR encephalitis in cases with characteristic clinical picture.

Intractable complex partial seizures associated with posterior cerebral artery giant aneurysm: a case report.

Summary: Giant aneurysms have rarely been reported in association with intractable complex partial seizures (CPS). We report a 30‐year‐old man with intractable CPS since age 18 years. Seizure onset was electrically localized to right temporal lobe. Preoperative neuroimaging studies showed a partially thrombosed giant aneurysm of the right posterior cerebral artery. Selective arnygdalo‐ hippocampectomy and occlusion of the posterior cerebral artery did not cause deficits. The patient has been seizure‐free for 15 months after operation. We review the relevant literature on aneurysms as a cause of epilepsy.

Good surgical outcome in discordant ictal EEG-MRI unilateral mesial temporal sclerosis patients.

Purpose: Video electroencephalography (vEEG) monitoring of patients with unilateral mesial temporal sclerosis (uMTS) may show concordant or discordant seizure onset in relation to magnetic resonance imaging (MRI) evidence of MTS. Contralateral seizure usually leads to an indication of invasive monitoring. Contralateral seizure onset on invasive monitoring may contraindicate surgery. We evaluated long‐term outcome after anteromesial temporal lobectomy (AMTL) in a consecutive series of uMTS patients with concordant and discordant vEEG findings, uniformly submitted to AMTL on the MRI evidence of MTS side without invasive monitoring.

Low prevalence but high specificity of material-specific memory impairment in epilepsy associated with hippocampal sclerosis

Material‐specific memory impairment is used as a lateralizing tool in the evaluation of temporal lobe epilepsy. Lateralizing ability of material‐specific memory deficits in temporal lobe epilepsy remains controversial.