RESUMEN
The International League against Epilepsy (ILAE) proposed a diagnostic scheme for psychogenic non-epileptic seizure (PNES). The debate on ethical aspects of the diagnostic procedures is ongoing, the treatment is not standardized and management might differ according to age group. The objective was to reach an expert and stakeholder consensus on PNES management. A board comprising adult and child neurologists, neuropsychologists, psychiatrists, pharmacologists, experts in forensic medicine and bioethics as well as patients' representatives was formed. The board chose five main topics regarding PNES: diagnosis; ethical issues; psychiatric comorbidities; psychological treatment; and pharmacological treatment. After a systematic review of the literature, the board met in a consensus conference in Catanzaro (Italy). Further consultations using a model of Delphi panel were held. The global level of evidence for all topics was low. Even though most questions were formulated separately for children/adolescents and adults, no major age-related differences emerged. The board established that the approach to PNES diagnosis should comply with ILAE recommendations. Seizure induction was considered ethical, preferring the least invasive techniques. The board recommended looking carefully for mood disturbances, personality disorders and psychic trauma in persons with PNES and considering cognitive-behavioural therapy as a first-line psychological approach and pharmacological treatment to manage comorbid conditions, namely anxiety and depression. Psychogenic non-epileptic seizure management should be multidisciplinary. High-quality long-term studies are needed to standardize PNES management.
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Trastornos Psicofisiológicos/terapia , Convulsiones/terapia , Adulto , Niño , Electroencefalografía/métodos , Femenino , Humanos , Masculino , Trastornos Psicofisiológicos/diagnóstico , Convulsiones/diagnósticoRESUMEN
INTRODUCTION: Seizures represent a potential source of accidents/death. Permission to drive may, therefore, be granted in a seizure-free period. Laws and regulations regarding this issue vary widely, and the onus of reporting seizures ultimately rests on the individual. Unfortunately, as some patients are unaware of their seizures, their reports may be unreliable. METHODS: In this retrospective study, we selected, from a group of 1100 consecutive patients, 57 cases (26 males/31 females; mean age: 42.5 years) in whom the AEEG documented ictal events (UIEs) not reported in a self-kept diary. By means of a simple questionnaire, we interviewed all these patients to collect information on driving licenses. We, thus, assessed how many of these patients (both drug resistant and seizure free) drove regularly. RESULTS: Our study shows a relatively large number of patients with epilepsy and UIEs. Fifteen patients suffered from idiopathic generalized epilepsy (IGE) while 42 had partial epilepsy (PE). The patients were seizure free in 21 cases and 36 had drug-resistant seizures. Many patients in both these subgroups had a driving license and drove normally (active driving in 12/36 drug-resistant patients and in 18/21 seizure-free patients). Worthy of note is the finding that an "apparently" seizure-free group of patients drove regularly. CONCLUSIONS: This study revealed a large number of patients (both drug resistant and seizure free) with AEEG-documented UIEs. This finding highlights the usefulness of AEEG in clinical practice as a means of more accurately ascertaining seizure freedom and supporting decisions involving the renewal or granting of a driving license.
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Conducción de Automóvil/legislación & jurisprudencia , Concienciación , Epilepsia/fisiopatología , Epilepsia/psicología , Concesión de Licencias , Adulto , Electroencefalografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare condition whose pathogenesis is unknown, though autoimmune-mediated mechanisms are thought to be involved. The prevalent neurological manifestations of this disorder are epileptic seizures and psychocognitive disorders associated with EEG alterations. High anti-thyroid antibody titers (particularly in cerebrospinal fluid) and the effectiveness of steroid therapy are usually considered to be crucial elements in the diagnostic process. We describe a 19-year-old female patient who had been referred to the psychiatric unit because of behavioral disorders characterized predominantly by delirium with sexual content. She developed recurrent focal seizures characterized by atypical ictal semiology (repetitive forceful yawning) and a rare EEG pattern (recurrent seizures arising from the left temporal region without evident "encephalopathic" activity). The presence of anti-thyroperoxidase antibodies in her cerebrospinal fluid and a good response to steroids confirmed the diagnosis of HE. The atypical presentation in the case we describe appears to widen the electroclinical spectrum of HE and highlights its importance for differential diagnosis purposes in the neuropsychiatric setting.
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Encefalopatías/fisiopatología , Epilepsias Parciales/fisiopatología , Enfermedad de Hashimoto/fisiopatología , Bostezo/fisiología , Encefalitis , Epilepsias Parciales/diagnóstico , Femenino , Humanos , Adulto JovenRESUMEN
OBJECTIVES: To assess the efficacy and tolerability of zonisamide in a study allowing flexible dosing in a more diverse and less refractory population than assessed in randomized controlled trials. METHODS: This 19-week, non-comparative study of adjunctive zonisamide included 281 adults who had at least four partial-onset seizures within 8 weeks on one or two antiepileptic drugs. Alterations to zonisamide doses were allowed after titration, except during two fixed-dose periods (weeks 10-13 and 16-19). RESULTS: At the end of the second fixed-dose period (median dose 300 mg/day), the median reduction in monthly seizure frequency was 33.3-41.1%; > or =50% responder rate was 40.9-44.2%; and seizure freedom rate was 15.0-15.9%, depending on the analysis used. The most common adverse events were fatigue (16.7%) and somnolence (15.3%). CONCLUSIONS: Zonisamide demonstrated efficacy in a setting more reflective of clinical practice and was generally well tolerated.
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Anticonvulsivantes/uso terapéutico , Epilepsias Parciales/tratamiento farmacológico , Isoxazoles/uso terapéutico , Adolescente , Adulto , Anciano , Análisis de Varianza , Intervalos de Confianza , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Quimioterapia Combinada , Epilepsias Parciales/psicología , Femenino , Humanos , Cooperación Internacional , Masculino , Persona de Mediana Edad , Calidad de Vida , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Adulto Joven , ZonisamidaRESUMEN
BACKGROUND: Status epilepticus is a condition of prolonged/repetitive seizures that often occurs in the elderly. Treatment in the elderly can be complicated by serious side effects associated with traditional drugs. OBJECTIVE: The aim of this pilot study was to evaluate the short-term efficacy/safety of intravenously administered LEV (IVLEV) as the treatment of choice for SE in the elderly. METHODS: We enrolled nine elderly patients (five female/four male; median age 78 years) with SE. Two patients had a previous diagnosis of epilepsy; in the remaining seven, SE was symptomatic. SE was convulsive in five and non-convulsive in four. All the patients presented concomitant medical conditions (arrhythmias/respiratory distress/hepatic diseases). As the traditional therapy for SE was considered unsafe, IVLEV was used as first-line therapy (loading dose of 1500 mg/100 ml/15 min, mean maintenance daily dose of 2500 mg/24 h) administered during video-EEG monitoring. RESULTS/CONCLUSIONS: In all the patients but one, IVLEV was effective in the treatment of SE and determined either the disappearance of (7/8), or significant reduction in (1/8), epileptic activity; no patient relapsed in the subsequent 24 h. No adverse events or changes in the ECG/laboratory parameters were observed. These data suggest that IVLEV may be an effective/safe treatment for SE in the elderly.
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Anticonvulsivantes/administración & dosificación , Geriatría , Piracetam/análogos & derivados , Estado Epiléptico/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Inyecciones Intravenosas/métodos , Levetiracetam , Masculino , Proyectos Piloto , Piracetam/administración & dosificación , Resultado del TratamientoRESUMEN
INTRODUCTION: Status epilepticus (SE) is frequently observed in epileptic patients. We reviewed a series of video-EEG documented SE to define the characteristics of SE in this population. MATERIALS AND METHODS: Retrospective evaluation of 50 epileptic patients with SE, revision of the electro-clinical data and therapies, and definition of the semeiological subtypes, aetiology, outcome and related epileptic syndromes. RESULTS: We identified 28 convulsive (19 focal and 9 generalized) and 22 non-convulsive (8 focal and 14 generalized) SE patients. In 13 patients, SE was situation-related (poor compliance, AED reduction, worsening seizures). In the remaining 37 patients, SE was related to the natural history of epilepsy (progression of underlying pathologies or intrinsic expression of epileptic syndromes); in these last cases, our results show a higher occurrence in cryptogenic frontal epilepsy (p=0.01). We identified two subgroups according to the duration of the event, i.e. SE lasting <12h and SE lasting >12h. Our results showed a worse response to therapy in SE lasting >12h (p=0.01), a better response to therapy in non-convulsive SE than in convulsive SE (p<0.05) and a relationship at statistical significance limit between a poor response to therapy/worse outcome and symptomatic epileptic syndromes (p=0.06). CONCLUSION: SE in epileptic patients has a wide spectrum of electro-clinical features. It may be related to the withdrawal or reduction of AEDs, or may even be the expression of the evolution of epileptic syndromes. Response to therapy is dependent on early diagnosis and therapy.
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Anticonvulsivantes/uso terapéutico , Estado Epiléptico/complicaciones , Estado Epiléptico/tratamiento farmacológico , Estado Epiléptico/epidemiología , Adolescente , Adulto , Anciano , Niño , Planificación en Salud Comunitaria , Electroencefalografía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Estado Epiléptico/etiología , Resultado del Tratamiento , Grabación en Video/métodosRESUMEN
Partial epilepsy with auditory features occasionally segregates in families as an autosomal dominant trait. In some families mutations in the leucine-rich glioma inactivated (LGI1) gene have been identified. Sporadic cases might harbour either denovo or low-penetrant LGI1 mutations, which will substantially alter the family risk for epilepsy. We selected sixteen sporadic patients with cryptogenic temporal lobe epilepsy and partial seizures with auditory features. We compared clinical features of these patients with those of published autosomal dominant family cases. We screened these patients for LGI1 mutations. Comparing the sporadic patients with the published familial cases no difference in either the primary auditory features or in the other associated epileptic manifestations was identified. Sequence analysis of the whole LGI1 gene coding regions in sporadic patients did not reveal changes in the LGI1 gene. The genetic analysis demonstrates that LGI1 is not a major gene for sporadic cases of partial epilepsy with auditory features at least in the Italian population. Screening of sporadic patients for LGI1 mutations appears not useful in genetic counselling of these patients.
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Epilepsia Parcial Sensorial/genética , Epilepsia del Lóbulo Temporal/genética , Predisposición Genética a la Enfermedad/genética , Mutación/genética , Proteínas/genética , Adulto , Análisis Mutacional de ADN , Epilepsia Parcial Sensorial/diagnóstico , Epilepsia Parcial Sensorial/fisiopatología , Epilepsia del Lóbulo Temporal/diagnóstico , Epilepsia del Lóbulo Temporal/fisiopatología , Femenino , Pruebas Genéticas , Humanos , Péptidos y Proteínas de Señalización Intracelular , Italia , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Visual-paired associative stimulation (V-PAS) is a transcranial magnetic stimulation (TMS) technique able to investigate long-term potentiation (LTP) and depression (LTD)-like plasticity in the primary motor cortex (M1) arising through early visuomotor integration. OBJECTIVE/HYPOTHESIS: Abnormal early visuomotor integration might contribute to the pathophysiology of intermittent photic stimulation (IPS)-induced photoparoxysmal response (PPR). METHODS: We applied V-PAS in 25 healthy subjects (HS), 25 PPR-positive patients, with and without idiopathic generalized epilepsy (IGE), and 8 PPR-negative patients with IGE. V-PAS consisted of primary visual area activation achieved by visual evoked potentials coupled with TMS-induced M1 activation at 100 ms interstimulus interval (ISI) (V-PAS100). Before and after V-PAS, we measured changes in motor evoked potentials (MEPs). We compared MEPs after 1 Hz repetitive TMS (rTMS) and 0.25 Hz-V-PAS100. To examine possible V-PAS-induced after-effects at other ISIs, we delivered V-PAS at 40 (V-PAS40) and 140 ms ISIs (V-PAS140). To clarify whether V-PAS100 increases parieto-/premotor-to-M1 connectivity, before and after V-PAS100, we examined MEPs evoked by paired-pulse techniques. RESULTS: V-PAS100 increased MEPs more in PPR-positive patients than in HS. PPR-negative patients had normal response to V-PAS100. 1 Hz-rTMS, 0.25 Hz-V-PAS100 and V-PAS40 elicited similar responses in HS and PPR-positive patients, whereas V-PAS140 induced stronger after-effects in PPR-positive patients than HS. After V-PAS, MEPs elicited by facilitatory paired-pulse protocols decreased similarly in HS and PPR-positive patients. Conversely, MEPs elicited by inhibitory protocols decreased in HS, whereas in PPR-positive patients, they turned from inhibition to facilitation. CONCLUSION: We suggest that abnormal early visuomotor integration contributes to the pathophysiology of PPR.
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Epilepsias Mioclónicas/fisiopatología , Epilepsia Generalizada/fisiopatología , Potenciales Evocados Motores/fisiología , Corteza Motora/fisiología , Trastornos por Fotosensibilidad/fisiopatología , Corteza Visual/fisiología , Adolescente , Adulto , Estudios de Casos y Controles , Potenciales Evocados Visuales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estimulación Luminosa , Estimulación Magnética Transcraneal , Adulto JovenRESUMEN
Using functional MRI (fMRI) with concurrent EEG monitoring the authors studied cortical activation associated with epileptiform discharges in three patients with fixation-off sensitivity. The EEG paroxysmal activity elicited by eliminating central vision correlated significantly with an increased blood oxygen level-dependent signal in the extrastriate cortex (Brodmann areas 19 and 37). fMRI provides a unique opportunity for localizing precisely the cortical areas generating paroxysmal activity in patients with fixation-off sensitivity.
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Corteza Cerebral/fisiopatología , Electroencefalografía/métodos , Epilepsia/fisiopatología , Imagen por Resonancia Magnética/métodos , Trastornos de la Visión/fisiopatología , Disparidad Visual/fisiología , Adulto , Técnicas de Diagnóstico Oftalmológico , Epilepsia/diagnóstico , Femenino , Humanos , Trastornos de la Visión/diagnóstico , Visión OcularRESUMEN
OBJECTIVE: This prospective, open-label study was conducted to evaluate the effectiveness, tolerability, and safety of levetiracetam in patients with epilepsy in whom unfavorable metabolism, complex drug interactions, or direct toxic effects of antiepileptic drugs (AEDs) had caused a worsening of comorbid conditions. METHODS: Study design included the introduction of levetiracetam, discontinuation of other AEDs, and a serial assessment comprising electroencephalograms and blood tests at baseline and 2, 6, and 12 months. Of 21 patients, 16 had partial and five generalized epilepsy. Concomitant pathologies were gastroenterological (six), vascular (four), endocrinological (four), or complex conditions including hematological (four) or dermatological (three) disease. A change of regimen was necessitated by drug-drug interactions in four patients, direct real or potential toxic effects of previous AEDs in 13, and a combination of interactions/toxic effects in four. RESULTS: After 12 months, 12 patients were seizure-free, nine had reductions in seizure frequency of 50-75%, and improvement in concomitant medical conditions was observed. No side effects were reported. CONCLUSION: Levetiracetam appears to be effective, well tolerated, and safe in patients with epilepsy and other medical conditions that are difficult to manage because of drug interactions or AED-related side effects.
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Anticonvulsivantes/uso terapéutico , Epilepsias Parciales/tratamiento farmacológico , Epilepsia Generalizada/tratamiento farmacológico , Piracetam/análogos & derivados , Adolescente , Adulto , Anciano , Anticonvulsivantes/administración & dosificación , Quimioterapia Combinada , Epilepsias Parciales/complicaciones , Epilepsia Generalizada/complicaciones , Femenino , Estudios de Seguimiento , Humanos , Levetiracetam , Masculino , Persona de Mediana Edad , Piracetam/administración & dosificación , Piracetam/uso terapéutico , Polifarmacia , Estudios Prospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: To describe causes of syncope in outpatients in whom structural heart disease was ruled out as a cause, and to analyse the role of a multidisciplinary approach in a syncope unit for the diagnosis of patients with syncope of unknown origin. METHODS: Cardiovascular autonomic nervous system (ANS) function was evaluated extensively in 521 outpatients by careful history, physical examination including orthostatic blood pressure measurement and standard ECG, and tilt testing. RESULTS: Causes of syncope remained unknown in 29.2% of cases. ANS dysfunction was found in 58.6% of those presenting with either neurally mediated syncope (53.6%) or chronic autonomic failure (5%); 3.8% of the patients suffered from syncope of cardiogenic origin (2.5%) or non-neurogenic hypotension (1.3%), and 8.4% had loss of consciousness of non-syncopal origin. Loss of consciousness was confirmed as being related to seizures in under 30% of patients initially diagnosed as having epilepsy. CONCLUSIONS: Neurally mediated syncope represents the commonest type of syncope. ANS evaluation including tilt testing should be considered as preliminary screening in patients with syncope in the absence of definite heart abnormalities. Neurologists should consider syncope from ANS failure as a comorbid factor in patients with seizures where the clinical characteristics are not straightforward.
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Atención Ambulatoria , Comunicación Interdisciplinaria , Grupo de Atención al Paciente , Síncope/diagnóstico , Adulto , Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Enfermedades del Sistema Nervioso Autónomo/epidemiología , Epilepsia/diagnóstico , Epilepsia/epidemiología , Femenino , Cefalea/diagnóstico , Cefalea/epidemiología , Humanos , Hiperventilación/diagnóstico , Hiperventilación/epidemiología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Síncope/epidemiología , Síncope/fisiopatología , Enfermedades Vestibulares/diagnóstico , Enfermedades Vestibulares/epidemiologíaRESUMEN
The aim of a Stereo-EEG investigation is to verify and prove that the hypothesis, done on the basis of the preliminary investigations (clinical, EEG, neuroradiological), are correct. This task is particularly hard in frontal lobe epilepsies, because of anatomical and physiopathological reasons. Among 277 consecutive patients, 86 were explored for a probable frontal epilepsy. The stereotactically introduced electrodes. 1) simultaneously record the electrical activity on both, mesial and lateral cortical areas, and, 2) in 3/4 of cases also investigate extra-frontal, mainly temporal, areas. Two small, non-surgical haematomas were provoked in one patient. The spatial trajectory of the discharges, evaluated with this methodology, permits of limiting the surgical removal in many cases.
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Electroencefalografía , Epilepsias Parciales/fisiopatología , Lóbulo Frontal/fisiopatología , Técnicas Estereotáxicas , Adolescente , Adulto , Mapeo Encefálico/instrumentación , Niño , Preescolar , Dominancia Cerebral/fisiología , Electrodos Implantados , Electroencefalografía/instrumentación , Epilepsias Parciales/cirugía , Potenciales Evocados , Lóbulo Frontal/cirugía , Humanos , Persona de Mediana Edad , Técnicas Estereotáxicas/instrumentaciónRESUMEN
INTRODUCTION: We studied the hippocampal region using Fluid Attenuation Inversion Recovery (Turbo-FLAIR) sequences to detect signal intensity abnormalities, and volumetric sequences to detect cortical thickness changes, in patients with drug-resistant temporal epilepsy. MATERIALS AND METHODS: We examined 30 patients with drug-resistant temporal epilepsy with a 1.5 Tesla unit (NT 15 Philips Gyroscan). Conventional SE, Turbo SE, IR, Turbo-FLAIR, volumetric 3D sequences on coronal plane, PD, T2-weighted SE sequences on axial plane, T1-weighted SE on sagittal plane were performed. Signal intensity and volumetric computerized measurements were obtained using the SUN system. RESULTS: Differences in signal intensity values between the two hippocampal regions were found in 18 patients with Turbo-FLAIR sequences. In 6 of these patients no significant differences in computerized evaluation of signal intensity were detected with either conventional or Turbo-SE sequences. Volumetric analysis showed hippocampal cortex thinning in 9 of 18 patients with hippocampal signal intensity abnormalities. CONCLUSIONS: Turbo-FLAIR were the best sequences for the detection of signal intensity changes in the hippocampal region. Such changes are strongly suggestive of hippocampal sclerosis, especially when associated with cortical atrophy.
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Epilepsia del Lóbulo Temporal/patología , Hipocampo/patología , Imagen por Resonancia Magnética/métodos , Resistencia a Medicamentos , Epilepsia del Lóbulo Temporal/tratamiento farmacológico , HumanosRESUMEN
Stereo-EEG activity, which was recorded precisely from the same location as stereotactic biopsies, was studied on 11 patients with low-grade glioma and severe partial epilepsy. The volume of the lesions varied from 6 to 72 cm3 (mean 23 cm3). A very depressed activity was recorded in all the 25 specimens of solid tumor and in more than half of infiltrated white matter. The background activity was still conserved in all 'normal' fragments, while it is never found in solid tumor; sometimes it could be found in the infiltrating tumors of white or gray matter. High voltage theta and delta activity was recorded in neither 'normal' nor solid tumors. We emphasize the importance of considering the stage of evolution when we evaluate the significance of the stereo-EEG activity.