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.
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