About one third of patients with newly diagnosed epilepsy do not achieve seizure control despite medical therapy and should be assessed in detail to confirm the diagnosis, to determine the epilepsy syndrome and to plan treatment strategy. Refractory epilepsy is established when there is inadequate seizure control despite using potentially effective antiepileptic drugs (AEDs) at tolerable levels for 1–2 years, and excluding nonepileptic events and poor compliance. An overview of patient management in everyday clinical practice is given. Risk factors for refractoriness include (1) generalized epilepsy with lesions; (2) focal epilepsy with hippocampal sclerosis, cortical dysplasia or hemorrhages; (3) early epilepsy onset; (4) high seizure frequency; (5) absence of response to the first 2 AEDs; (6) high frequency of interictal spikes, and (7) multifocal spikes. The efficacy of surgery ranges from curative (resection) to palliative (vagus nerve stimulation, callosotomy, multiple subpial transections) depending on the epilepsy syndrome and etiology. Using a new antiepileptic drug benefits about one third of patients. Refractoriness is constitutive in most patients and shows a progressive or remission-relapse course in others. As the transporter and target hypotheses do not entirely explain refractoriness, other hypotheses are emerging. New treatments under investigation are described.
SummaryGiant cell glioblastoma is an infrequent variety of glioblastoma (5% of the cases). It has deserved a separate category in the World Health Organization classification of grade IV tumors. The clinical, imaging, histological and immunohistochemical characteristics, and the genetic alterations are reviewed. Treatment and prognosis are discussed and updated. The case of a patient that survived 19 months and died of spinal leptomeningeal metastases is illustrated.KEYWORDS: Giant cell glioblastoma. Leptomeningeal metastases. Surgery. Temozolomide. Radiotherapy.Glioblastoma de células gigantes: revisión de la literatura e ilustración de un caso Resumen El glioblastoma de células gigantes es una variante rara dentro de los glioblastomas (5% de los casos). En la clasificación de la Organización Mundial de la Salud esta entidad ha merecido una categoría aparte en los tumores de grado IV. Se revisan las características clínicas, radiológicas, histológicas, inmunohistoquímicas y las alteraciones genéticas que caracterizan este tumor. El tratamiento y el pronóstico son discutidos aportando información actualizada. Finalmente se presenta un caso clínico ilustrado, en el cual el paciente sobrevivió durante 19 meses, falleciendo como consecuencia de una diseminación espinal leptomeníngea.PALABRAS CLAVE: Glioblastoma de células gigantes. Metástasis leptomeníngeas. Cirugía. Temozolomida. Radioterapia IntroductionUnlike the "common" glioblastoma, giant cell glioblastoma is not encountered in everyday practice and it probably comes as an unexpected diagnosis most of the times. Despite certain similarities between the two disease processes, there are differences that may be of relevance for the management of the occasional patient harboring this lesion. A case of giant cell glioblastoma managed at the authors' institution is reported and the key features of giant cell glioblastoma are reviewed. Clinical caseA 54 year-old man presented with headache and motor dysphasia of short duration. MR disclosed a voluminous mass in the left temporal lobe (figure 1-A&B). The patient was operated. A frontotemporal approach was employed. The lesion had a good cleavage plane, was moderately hemorrhagic and was totally resected in a piece meal fashion. The postoperative course was uneventful. Histological examination disclosed a highly cellular neoplasm with marked pleomorphism. Prominent giant cells and numerous atypical mitotic figures were found (figure 1-C). Reticulin was abundant in the stroma. Immunohistochemistry showed positivity for GFAP, S100 protein and p53. The picture was consistent with giant cell glioblastoma. Adjuvant radiotherapy (60Gy) and chemotherapy (temozolomide) were given. There was no evidence of relapse at the 14 month follow up MR ( figure 1-D). The patient was symptom free and had no neurological deficits at that time. Three months after, the patient complained of backache, lower limb weakness and deteriorating general condition. MR disclosed spinal leptomeningeal metastases (figure 1-E). Curiously there was no ev...
Operational diagnostic criteria have been recommended by the International League Against Epilepsy and are based on temporal relationship, severity, and type of insult. Antiepileptic drug prophylaxis is recommended in severe head trauma, preeclampsia, and possibly high-risk subarachnoid or intracranial hemorrhage. It is crucial to rapidly identify all insults possibly involved, treat underlying diseases, revert corrigible factors, and in case of central nervous system involvement, use antiepileptic drugs during the acute period. Risk of epilepsy is increased in patients with neurological insults but not with metabolic disorders. Some refractory epilepsies in adults, mostly epilepsy due to hippocampal sclerosis, are preceded by acute symptomatic seizures related to selected insults occurring at a specific time. Mortality rate is globally increased.
Summary:Purpose: To investigate the risk of illnesses in a cohort of patients with epilepsy and in matched nonepilepsy controls, by type and complications.Methods: A total of 951 children and adults with idiopathic, cryptogenic, or remote symptomatic epilepsy and 904 matched controls seen in secondary and tertiary centers in eight European countries (England, Estonia, Germany, Italy, the Netherlands, Portugal, Russia, Slovenia) were followed prospectively for 17,484 and 17,206 person-months and asked to report any spontaneous complaint requiring medical attention (illness), its type and complications (hospitalization, absence from work or school, medical action). Risk assessment was done by actuarial methods, relative risks (RR), and 95% confidence intervals (CIs).Results: During the study period 644 patients (68%) and 504 controls (56%) reported an illness (p < 0.0001); 30% were seizure related. The cumulative probability of illness at 12 and 24 months was 49 and 86% in the cases and 39 and 75% in the controls (p < 0.0001). The largest differences regarded disorders affecting the nervous system (NS) (RR, 3.3; 95% CI, 2.3-4.2) and ear, nose, and throat (ENT) (RR, 1.3; 95% CI, 1.0-1.6). In patients with epilepsy, an NS illness was more likely to be followed by hospital admission, work absence, or medical intervention. All risks were significantly reduced after excluding seizure-related events.Conclusions: Patients with epilepsy are at higher risk of NS and ENT illnesses and complications than the general population. However, the risk of illness is significantly reduced when seizure-related events are excluded. Key Words: EpilepsyMorbidity-Illnesses.Patients with epilepsy have been thought to have a higher risk of illness than the general population, but comorbidity in epilepsy has been investigated in few population-based prevalence or analytic studies. Other clinical conditions and disabilities were reported by 47% of adults with epilepsy in Sweden, with psychiatric, cardiovascular, pulmonary, systemic, and renal diseases in decreasing order (1). Lifetime mental and gastric disorders were reported by 7 and 3% of adults with childhoodonset epilepsy in the Czech Republic (2). In Finland, somatic comorbidity was found in 84-95% of patients with childhood-onset epilepsy and 67-74% of controls (3). In a multicenter European cohort study of children and adults with idiopathic, cryptogenic, or remote symptomatic epilepsy, we found one or more illnesses at entry in 30% of cases compared with 17% of age-and sex-matched controls (4).These contrasting results may be explained by differences in the study populations and in the study design and methods. In addition, the morbidity risk attributable Accepted September 14, 2003. Address correspondence and reprint requests to Dr. E. Beghi at Istituto di Ricerche Farmacologiche "Mario Negri," Via Eritrea 62, 20157 Milano, Italy. E-mail: beghi@marionegri.it to the disease cannot be explored in studies on the prevalence of other clinical conditions in epilepsy. For these reasons, i...
SUMMARYPurpose: We evaluated the role of interictal rhythmical midline theta (RMT) in the identification of frontal lobe epilepsy (FLE) and its differentiation from temporal lobe epilepsy (TLE) and nonepileptic controls. Methods: We included 162 individuals in the study: 54 FLE patients, 54 TLE patients, and 54 nonepileptic controls. Continuous electroencephalographic (EEG)-video monitoring was performed in all individuals. Interictal RMT was included only if it occurred during definite awake states. RMT associated with drowsiness or mental activation and ictal RMT was excluded. Results: We identified RMT significantly more frequently in FLE patients (48.1%, 26 of 54) than in TLE patients (3.7%, 2 of 54) (p < 0.01), and not in the control group. The average frequency was 6 Hz (range 5-7 Hz), and the average RMT bursts lasted 8 s (3-12 s). Interestingly, all mesial FLE patients (n = 4) (as established by invasive EEG recordings) showed RMT, whereas this was less frequently the case in the other FLE patients (44%, 22 of 50) (p = 0.03). Thirteen of our 54 patients with FLE (24%) did not have any interictal epileptiform discharges (IEDs), but RMT was observed in the majority of these patients (62%, 8 of 13). Conclusion: Interictal RMT is common and has a localizing value in patients with FLE, provided that conditions such as drowsiness and mental activation as confounding factors for RMT are excluded. RMT should be included in the evaluation of patients considered for resective epilepsy surgery.
Multiple supratentorial abscesses caused by Listeria monocytogenes are rare. We report the simultaneous occurrence of multiple supratentorial and brainstem abscesses due to Listeria, in a patient under corticotherapy for an exacerbation of ulcerative colitis. MR imaging features before and after successful conservative treatments are depicted. In immunocompromised patients with supratentorial listerial abscesses, the coexistence of brainstem abscedation is exceptional. Despite high mortality associated with listerial abscesses, this case illustrates the possibility of a good clinical outcome, if the appropriate antibiotic regimen is instituted and the immunosuppressant agent is discontinued. © 2007 Elsevier Masson SAS. All rights reserved. MOTS CLÉS
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