Roberto Mai - Academia.edu (original) (raw)

Papers by Roberto Mai

Research paper thumbnail of Intracerebral recordings of high-frequency oscillations evoked by gaze shift perception

Gaze is a social cue communicating the intentions and the dispositions of other individuals. A gr... more Gaze is a social cue communicating the intentions and the dispositions of other individuals. A great interest has been paid to the way the brain codes the direction of others’ gaze and different cognitive processes have been associated to direct-gaze (DG) and averted-gaze (AG) observation. Strong activations of the posterior temporal region during AG were described by different studies employing different techniques and models, including scalp-EEG, human and monkey fMRI. A classic interpretation is that AG triggers a “shared attention” mechanism, i.e. the others’ gaze aversion indicates their shift of attention toward a specific direction, thus inducing in the observer a reflexive shift of attention towards the same direction. Another possible interpretation could be grounded on the social aspect of gaze aversion, that is, the cessation of the eye contact and the intention to interrupt communicative interactions.

Research paper thumbnail of Human cortical activity evoked by gaze shift observation: An intracranial EEG study

Human Brain Mapping, 2014

Research paper thumbnail of Epileptic motor behaviors during sleep: Anatomo-electro-clinical features

Sleep Medicine, 2011

Background: Sleep-related complex motor seizures have long been considered pathognomonic features... more Background: Sleep-related complex motor seizures have long been considered pathognomonic features of Nocturnal Frontal Lobe Epilepsy (NFLE). In recent years, these manifestations have also been reported to have a temporal or insular origin. Method: We describe 40 drug-resistant epileptic patients with complex motor seizures during sleep, submitted to presurgical stereo-EEG (SEEG) evaluation and seizure-free after surgical resection of the epileptogenic zone. Results: In a significant proportion (30%) of these patients, seizures arose from extra-frontal regions, including mainly the temporal lobe and the insular cortex, but also the parietal and occipital lobes. In patients with extra-frontal epilepsy, when complex motor behaviors appeared, SEEG revealed that the ictal discharge involved the cingulate and the frontal regions. Finally, at histology, Taylor's focal cortical dysplasia (TFCD) was the most common finding (90% of patients), independent of the site of seizure onset. Conclusion: As previously reported by other studies, this histologic substrate may be a major determinant of sleep-related seizures in drug-resistant epileptic patients.

Research paper thumbnail of Epilepsy surgery at Niguarda Hospital in Milan: report of the first 4 years activity (96-99)

Research paper thumbnail of Multilobar resections in epilepsy surgery

Research paper thumbnail of Research perspectives in cortical dysplasia and associated epilepsies

Epileptic Disorders, 1999

Our understanding of cortical alterations and related epilepsies has grown enormously in the last... more Our understanding of cortical alterations and related epilepsies has grown enormously in the last decade thanks to the explosion of basic information from laboratory neuroscience combined with advances in diagnostic tools, therapeutic approaches and surgical techniques. In the present paper, we briefly review the most important advances in these fields from the point of view of the clinician concerned with cortical malformation-related epilepsies. We propose that a highly effective way forward, expected not only to widen knowledge of the basic mechanisms of seizure generation, but also to improved the management of patients, would be to promote interdisciplinary research programmes on resected human cortex that involve neurosurgeons, neurologists and laboratory neuroscientists.

Research paper thumbnail of Esplorazione con elettrodi intracerebrali nello studio dell’epilessia parziale farmaco-resistente

Research paper thumbnail of Fear and happiness in the eyes: An intra-cerebral event-related potential study from the human amygdala

Research paper thumbnail of Epilepsy surgery in tuberous sclerosis complex: early predictive elements and outcome

Childs Nervous System, 2008

Aim The aim of the study was to evaluate the surgical treatment of epilepsy and detection of poss... more Aim The aim of the study was to evaluate the surgical treatment of epilepsy and detection of possible early surgery predictive elements in patients with tuberous sclerosis complex (TSC). Materials and methods Forty-two TSC patients with epilepsy were selected and divided into two main groups: definite and fruste forms. Definite forms were divided into different groups: patients with pharmacologically controlled epilepsy, patients with pharmacoresistant epilepsy excluded from surgery after an extensive presurgical assessment, and patients with a pharmacoresistant epilepsy who underwent surgery. We compared the definite TSC groups to identify elements that predict surgical candidacy. Second, we compared all operated patients to assess surgical outcome. Conclusion We found several factors that could predict a surgical intervention even if identification of patients with refractory epilepsy who can benefit from surgery is an evolving process. Also, several positive factors for good surgical outcome were identified. Patients with the fruste form had excellent surgical outcome.

Research paper thumbnail of Focal cortical dysplasia: neuropathological subtypes, EEG, neuroimaging and surgical outcome

Brain, 2002

Abbreviations: AD = architectural dysplasia; CD = cytoarchitectural dysplasia; FCD = focal cortic... more Abbreviations: AD = architectural dysplasia; CD = cytoarchitectural dysplasia; FCD = focal cortical dysplasia; FLAIR =¯uid-attenuated inversion recovery; GFAP = glial ®brillary acidic protein; HE = haematoxylin and eosin; IR = inversion recovery; MCD = malformations of cortical development; SEEG = stereo-EEG; TFCD = Taylor-type cortical dysplasia; TSE = turbo spin-echo; VEEG = video-EEG

Research paper thumbnail of Epileptic motor behaviors during sleep: Anatomo-electro-clinical features

Sleep Medicine

Background: Sleep-related complex motor seizures have long been considered pathognomonic features... more Background: Sleep-related complex motor seizures have long been considered pathognomonic features of Nocturnal Frontal Lobe Epilepsy (NFLE). In recent years, these manifestations have also been reported to have a temporal or insular origin. Method: We describe 40 drug-resistant epileptic patients with complex motor seizures during sleep, submitted to presurgical stereo-EEG (SEEG) evaluation and seizure-free after surgical resection of the epileptogenic zone. Results: In a significant proportion (30%) of these patients, seizures arose from extra-frontal regions, including mainly the temporal lobe and the insular cortex, but also the parietal and occipital lobes. In patients with extra-frontal epilepsy, when complex motor behaviors appeared, SEEG revealed that the ictal discharge involved the cingulate and the frontal regions. Finally, at histology, Taylor's focal cortical dysplasia (TFCD) was the most common finding (90% of patients), independent of the site of seizure onset. Conclusion: As previously reported by other studies, this histologic substrate may be a major determinant of sleep-related seizures in drug-resistant epileptic patients.

Research paper thumbnail of Discussion of “Structural Seismic Damper” by Manuel Aguirre and A. Roberto Sánchez (May, 1992, Vol. 118, No. 5

Journal of Structural Engineering-asce, 1993

Research paper thumbnail of LIBRETTO3: modelling tritium extraction/permeation and evaluation of permeation barriers under irradiation

Journal of Nuclear Materials, 1996

Permeation barriers are required in order to limit the size and cost of the detritiation plants f... more Permeation barriers are required in order to limit the size and cost of the detritiation plants for future fusion reactor blankets of the water-cooled Pb-17Li type. The LIBRETI'O irradiations were performed to evaluate the efficiency of permeation barriers under high flux reactor (HFR) conditions. Tritium extraction and permeation characteristics from Pb-17Li under variable temperatures 553-723 K, H 2 doping (0-1 vol%) and purge gas flow rates 20-100 scc/min were tested in LIBRETTO-3. An external TiC coating, an internal (TiC + A1203), both produced by chemical vapour deposition (CVD), and an internal AI203 produced by pack cementation (PC) on AISI 316L steel were tested as permeation barriers. The release mechanisms, experimental uncertainties and method for permeation barriers qualification are presented. As a result permeation reduction factors (PRF) at 0.1 dpa of 17 and 34 were obtained for the CVD-A1203 at 498 K and for the PC-A1203 at 508 K, respectively. These values were confirmed by a residence time analysis and are higher than in a preliminary analysis .

Research paper thumbnail of Sleep-related hyperkinetic seizures: always a frontal onset

Neurological Sciences, 2005

Hyperkinetic seizures are considered a typical manifestation of nocturnal frontal lobe epilepsy (... more Hyperkinetic seizures are considered a typical manifestation of nocturnal frontal lobe epilepsy (NFLE). Patients with temporal lobe epilepsy with mainly sleeprelated seizures have been described; however they commonly lack hyperkinetic activity and seizure frequency is low. We retrospectively analysed our population of 442 consecutive patients surgically treated between January 1996 and January 2004. Among these there were 25 patients with sleep-related hyperkinetic epileptic seizures, with a frontal lobe onset in 18 cases and a temporal lobe onset in 7. Patients with sleep-related hyperkinetic seizures with temporal lobe origin had anamnestic and clinical features strikingly similar to those with a frontal onset, with agitated movements, high seizure frequency and no history of febrile convulsions. We confirm our previous findings that this kind of epileptic manifestation is not only peculiar to frontal lobe epilepsy.

Research paper thumbnail of Surgical treatment of drug-resistant nocturnal frontal lobe epilepsy

Brain, 2007

Of the cases with nocturnal frontal lobe epilepsy (NFLE) 30% are refractory to antiepileptic medi... more Of the cases with nocturnal frontal lobe epilepsy (NFLE) 30% are refractory to antiepileptic medication, with several patients suffering from the effects of both ongoing seizures and disrupted sleep. From a consecutive series of 522 patients operated on for drug-resistant focal epilepsy, 21 cases (4%), whose frontal lobe seizures occurred almost exclusively (>90%) during sleep, were selected. All patients underwent a comprehensive presurgical evaluation, which included history, interictal EEG, scalp video-EEG monitoring, high-resolution MRI and, when indicated, invasive recording by stereo-EEG (SEEG). There were 11 males and 10 females, whose mean age at seizure onset was 6.2 years, mean age at surgery was 24.7 years and seizure frequency ranged from <20/month to >300/month. Nine patients reported excessive daytime sleepiness (EDS). Prevalent ictal clinical signs were represented by asymmetric posturing (6 cases), hyperkinetic automatisms (10 cases), combined tonic posturing and hyperkinetic automatisms (4 cases) and mimetic automatisms (1 case). All patients reported some kind of subjective manifestations. Interictal and ictal EEG provided lateralizing or localizing information in most patients. MRI was unrevealing in 10 cases and it showed a focal anatomical abnormality in one frontal lobe in 11 cases. Eighteen patients underwent a SEEG evaluation to better define the epileptogenic zone (EZ). All patients received a microsurgical resection in one frontal lobe, tailored according to pre-surgical evaluations. Two patients were operated on twice owing to poor results after the first resection. Histology demonstrated a Taylor-type focal cortical dysplasia (FCD) in 16 patients and an architectural FCD in 4. In one case no histological change was found. After a post-operative follow-up of at least 12 months (mean 42.5 months) all the 16 patients with a Taylor's FCD were in Engel's Class Ia and the other 5 patients were in Engel's Classes II or III. After 6 months post-surgery EDS had disappeared in the 9 patients who presented this complaint preoperatively. It is concluded that patients with drug-resistant, disabling sleep-related seizures of frontal lobe origin should be considered for resective surgery, which may provide excellent results both on seizures and on epilepsyrelated sleep disturbances. An accurate pre-surgical evaluation, which often requires invasive EEG recording, is mandatory to define the EZ. Further investigation is needed to explain the possible causal relationships between FCD, particularly Taylor-type, and sleep-related seizures, as observed in this cohort of NFLE patients. Surgery for drug-resistance NFLE Brain (2007), 130, 561-573 Surgery for drug-resistance NFLE Brain (2007), 130, 561-573 Surgery for drug-resistance NFLE Brain (2007), 130, 561-573 567 by guest on December 13, 2013 http://brain.oxfordjournals.org/ Downloaded from 570 Brain (2007), 130, 561-573 L. Nobili et al. by guest on December 13, 2013 http://brain.oxfordjournals.org/ Downloaded from Surgery for drug-resistance NFLE Brain (2007), 130, 561-573

Research paper thumbnail of Circulating levels of tumour necrosis factor-α and its soluble receptors are increased in the blood of patients with amyotrophic lateral sclerosis

Neuroscience Letters, 2000

An immunologic pathogenesis for amyotrophic lateral sclerosis (ALS) has been recently proposed. W... more An immunologic pathogenesis for amyotrophic lateral sclerosis (ALS) has been recently proposed. We tested the whole tumour necrosis factor (TNF) system in the serum of 51 ALS patients at different stages of the disease and 36 healthy controls. Antigenic TNF-a and its soluble receptors (sTNF-Rs), measured by ELISA, were signi®cantly higher in ALS patients than in healthy controls. However, biologically active TNF-a, corresponding to the sTNF-Rs-unbound trimeric TNFa molecule and assayed by its cytotoxic activity on a sensitive cell line, was similar between ALS patients and healthy controls. Neither antigenic TNF-a, bioactive TNF-a nor sTNF-Rs correlated with disease severity, disease duration, or weight loss. In conclusion, we reported an activation of the TNF system in ALS. The role of this activation in the pathogenesis of the disease remains elusive. q

Research paper thumbnail of Emotions Induced by Intracerebral Electrical Stimulation of the Temporal Lobe

Epilepsia, 2006

Summary: Purpose: To assess the quality and frequency of emotions induced by intracerebral electr... more Summary: Purpose: To assess the quality and frequency of emotions induced by intracerebral electrical stimulation of the temporal lobe.Methods: Behavioral responses were obtained by electrical stimulation in 74 patients undergoing presurgical video-stereo-EEG monitoring for drug-resistant epilepsy. Intracerebral electrical stimulation was performed by delivering trains of electrical stimuli of alternating polarity; the intensity could vary from 0.2 to 3 mA. Stimulation frequency was 1 Hz or 50 Hz. Nine hundred thirty-eight stimulation procedures were performed.Results: Seventy-nine emotional responses (ERs) were obtained (8.4%). Of these, 67 were “'fear responses.” Sad feelings were evoked 3 times, happy-pleasant feelings 9 times. Anger and disgust were never observed. The following variables affected the incidence of ER: (a) Anatomical site of stimulation. ERs (always fear) were maximal at the amygdala (12%) and minimal for lateral neocortical stimulation (3%, p < 0.01). (b) Pathology. Stimulation of a temporal lobe with hippocampal sclerosis was associated with a lower frequency of ERs compared with stimulation of a temporal lobe with no evidence of atrophy in the medial temporal structures. (c) Stimulation frequency. ERs were 12% at 50 Hz versus 6.0% at 1 Hz (p < 0.01). (d) Gender. In women fear responses were 16% compared with 3% in men (p < 0.01). There were no gender differences when analyzing nonemotional responses.Conclusions: These data confirm the role of the medial temporal lobe region in the expression of emotions, especially fear-related behaviors. Fear was observed more frequently in the absence of medial temporal sclerosis, supporting the hypothesis that emotional behaviors induced by stimulation are positive phenomena, strictly related to the physiological function of these regions. Further investigations should address why women express fear behaviors more frequently than men.

Research paper thumbnail of Sleep-related Minor Motor Events in Nocturnal Frontal Lobe Epilepsy

Epilepsia, 2007

Summary: Purpose: Nocturnal frontal lobe epilepsy (NFLE) is characterized by a wide spectrum of s... more Summary: Purpose: Nocturnal frontal lobe epilepsy (NFLE) is characterized by a wide spectrum of sleep-related motor manifestations of increasing complexity, ranging from major episodes to brief motor events (minor motor events, MMEs). NFLE patients may exhibit a large quantity of MMEs in the form of short-lasting stereotyped movements. Whereas major episodes are considered epileptiform manifestations, it remains unclear whether the MMEs are related to epileptiform discharges (EDs).Methods: To study the relation between EDs and the occurrence of MMEs, we report a detailed neurophysiolgical evaluation in NFLE subjects explored by using implanted electrodes.Results: The median value of ED-related movements was 71.8%. Motor expression in relation to epileptiform discharge was surprisingly variable; no peculiar expression of MMEs could be attributed to the presence of EDs.Conclusions: Our data suggest that ED-associated MMEs are extremely polymorphous, and no univocal relation to EDs can be identified. We hypothesize that MMEs are not a direct effect of epileptiform discharge (i.e., not epileptic in origin), but the result of aspecific disinhibition of innate motor patterns. We warn clinicians that the epileptic nature of minimal motor phenomena in NFLE cannot be established on the clinical phenomenology of the event.

Research paper thumbnail of Temporal Lobe Epilepsy in Children: Electroclinical Study of 77 Cases

Epilepsia, 2006

Summary: Purpose: Temporal lobe epilepsy (TLE) is probably more difficult to recognize in childre... more Summary: Purpose: Temporal lobe epilepsy (TLE) is probably more difficult to recognize in children than in adults. In fact, ictal symptoms in children are less stereotyped and less obvious, and the neuropathological substrate is more heterogeneous than in adults. The aim of this study is to examine the relationships between etiology, age at onset and electroclinical findings in 77 children with TLE, 32 of whom were surgically treated.Methods: Electroclinical study including video-EEG recording of seizures in 77 children with TLE. The investigation focused on the first five initial ictal symptoms.Results: Age at onset was less than 3 years in 39 cases, between 3 and 6 years in 17 cases and older than 6 years in 21 cases. Auras also occurred in younger children but were more common after the age of 6 years. A peculiar initial ictal semiology consisted in staring with arrest, lip cyanosis, and very slight oral automatisms. In some cases, EEG recordings documented seizures starting independently on both temporal lobes. Based on electroclinical and neuroradiological features, we recognized three subgroups: symptomatic TLE due to cortical malformations or nonevolutive tumors, TLE with mesial temporal sclerosis, and cryptogenic TLE.Conclusions: A correct electroclinical and neuroradiological approach allows in several cases early recognition of TLE even when onset is earlier than the age of 6 years. A correct definition of the localization relies primarily on video-EEG recording of the seizures, possibly repeated during follow up in cases lacking obvious neuroradiological correlation.

Research paper thumbnail of Ictal headache: Headache as first ictal symptom in focal epilepsy

Epilepsy & Behavior

Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is... more Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is often neglected because of the dramatic neurological manifestations of the seizure. Headache can also be the sole or predominant clinical manifestation of epileptic seizures, although this is a relatively rare condition. We describe two cases of focal symptomatic drug-resistant epilepsy with headache as the first ictal symptom. In both cases, the headache, which lasted a few seconds, was contralateral to the ictal discharge and did not have the clinical features of migraine. Ictal headache is a rare epilepsy symptom that can help to localize ictal EEG discharges. Recently, the term ictal epileptic headache has been proposed in cases in which headache is the sole ictal epileptic manifestation Diagnosis requires the simultaneous onset of headache with EEGdemonstrated ictal discharges.

Research paper thumbnail of Intracerebral recordings of high-frequency oscillations evoked by gaze shift perception

Gaze is a social cue communicating the intentions and the dispositions of other individuals. A gr... more Gaze is a social cue communicating the intentions and the dispositions of other individuals. A great interest has been paid to the way the brain codes the direction of others’ gaze and different cognitive processes have been associated to direct-gaze (DG) and averted-gaze (AG) observation. Strong activations of the posterior temporal region during AG were described by different studies employing different techniques and models, including scalp-EEG, human and monkey fMRI. A classic interpretation is that AG triggers a “shared attention” mechanism, i.e. the others’ gaze aversion indicates their shift of attention toward a specific direction, thus inducing in the observer a reflexive shift of attention towards the same direction. Another possible interpretation could be grounded on the social aspect of gaze aversion, that is, the cessation of the eye contact and the intention to interrupt communicative interactions.

Research paper thumbnail of Human cortical activity evoked by gaze shift observation: An intracranial EEG study

Human Brain Mapping, 2014

Research paper thumbnail of Epileptic motor behaviors during sleep: Anatomo-electro-clinical features

Sleep Medicine, 2011

Background: Sleep-related complex motor seizures have long been considered pathognomonic features... more Background: Sleep-related complex motor seizures have long been considered pathognomonic features of Nocturnal Frontal Lobe Epilepsy (NFLE). In recent years, these manifestations have also been reported to have a temporal or insular origin. Method: We describe 40 drug-resistant epileptic patients with complex motor seizures during sleep, submitted to presurgical stereo-EEG (SEEG) evaluation and seizure-free after surgical resection of the epileptogenic zone. Results: In a significant proportion (30%) of these patients, seizures arose from extra-frontal regions, including mainly the temporal lobe and the insular cortex, but also the parietal and occipital lobes. In patients with extra-frontal epilepsy, when complex motor behaviors appeared, SEEG revealed that the ictal discharge involved the cingulate and the frontal regions. Finally, at histology, Taylor's focal cortical dysplasia (TFCD) was the most common finding (90% of patients), independent of the site of seizure onset. Conclusion: As previously reported by other studies, this histologic substrate may be a major determinant of sleep-related seizures in drug-resistant epileptic patients.

Research paper thumbnail of Epilepsy surgery at Niguarda Hospital in Milan: report of the first 4 years activity (96-99)

Research paper thumbnail of Multilobar resections in epilepsy surgery

Research paper thumbnail of Research perspectives in cortical dysplasia and associated epilepsies

Epileptic Disorders, 1999

Our understanding of cortical alterations and related epilepsies has grown enormously in the last... more Our understanding of cortical alterations and related epilepsies has grown enormously in the last decade thanks to the explosion of basic information from laboratory neuroscience combined with advances in diagnostic tools, therapeutic approaches and surgical techniques. In the present paper, we briefly review the most important advances in these fields from the point of view of the clinician concerned with cortical malformation-related epilepsies. We propose that a highly effective way forward, expected not only to widen knowledge of the basic mechanisms of seizure generation, but also to improved the management of patients, would be to promote interdisciplinary research programmes on resected human cortex that involve neurosurgeons, neurologists and laboratory neuroscientists.

Research paper thumbnail of Esplorazione con elettrodi intracerebrali nello studio dell’epilessia parziale farmaco-resistente

Research paper thumbnail of Fear and happiness in the eyes: An intra-cerebral event-related potential study from the human amygdala

Research paper thumbnail of Epilepsy surgery in tuberous sclerosis complex: early predictive elements and outcome

Childs Nervous System, 2008

Aim The aim of the study was to evaluate the surgical treatment of epilepsy and detection of poss... more Aim The aim of the study was to evaluate the surgical treatment of epilepsy and detection of possible early surgery predictive elements in patients with tuberous sclerosis complex (TSC). Materials and methods Forty-two TSC patients with epilepsy were selected and divided into two main groups: definite and fruste forms. Definite forms were divided into different groups: patients with pharmacologically controlled epilepsy, patients with pharmacoresistant epilepsy excluded from surgery after an extensive presurgical assessment, and patients with a pharmacoresistant epilepsy who underwent surgery. We compared the definite TSC groups to identify elements that predict surgical candidacy. Second, we compared all operated patients to assess surgical outcome. Conclusion We found several factors that could predict a surgical intervention even if identification of patients with refractory epilepsy who can benefit from surgery is an evolving process. Also, several positive factors for good surgical outcome were identified. Patients with the fruste form had excellent surgical outcome.

Research paper thumbnail of Focal cortical dysplasia: neuropathological subtypes, EEG, neuroimaging and surgical outcome

Brain, 2002

Abbreviations: AD = architectural dysplasia; CD = cytoarchitectural dysplasia; FCD = focal cortic... more Abbreviations: AD = architectural dysplasia; CD = cytoarchitectural dysplasia; FCD = focal cortical dysplasia; FLAIR =¯uid-attenuated inversion recovery; GFAP = glial ®brillary acidic protein; HE = haematoxylin and eosin; IR = inversion recovery; MCD = malformations of cortical development; SEEG = stereo-EEG; TFCD = Taylor-type cortical dysplasia; TSE = turbo spin-echo; VEEG = video-EEG

Research paper thumbnail of Epileptic motor behaviors during sleep: Anatomo-electro-clinical features

Sleep Medicine

Background: Sleep-related complex motor seizures have long been considered pathognomonic features... more Background: Sleep-related complex motor seizures have long been considered pathognomonic features of Nocturnal Frontal Lobe Epilepsy (NFLE). In recent years, these manifestations have also been reported to have a temporal or insular origin. Method: We describe 40 drug-resistant epileptic patients with complex motor seizures during sleep, submitted to presurgical stereo-EEG (SEEG) evaluation and seizure-free after surgical resection of the epileptogenic zone. Results: In a significant proportion (30%) of these patients, seizures arose from extra-frontal regions, including mainly the temporal lobe and the insular cortex, but also the parietal and occipital lobes. In patients with extra-frontal epilepsy, when complex motor behaviors appeared, SEEG revealed that the ictal discharge involved the cingulate and the frontal regions. Finally, at histology, Taylor's focal cortical dysplasia (TFCD) was the most common finding (90% of patients), independent of the site of seizure onset. Conclusion: As previously reported by other studies, this histologic substrate may be a major determinant of sleep-related seizures in drug-resistant epileptic patients.

Research paper thumbnail of Discussion of “Structural Seismic Damper” by Manuel Aguirre and A. Roberto Sánchez (May, 1992, Vol. 118, No. 5

Journal of Structural Engineering-asce, 1993

Research paper thumbnail of LIBRETTO3: modelling tritium extraction/permeation and evaluation of permeation barriers under irradiation

Journal of Nuclear Materials, 1996

Permeation barriers are required in order to limit the size and cost of the detritiation plants f... more Permeation barriers are required in order to limit the size and cost of the detritiation plants for future fusion reactor blankets of the water-cooled Pb-17Li type. The LIBRETI'O irradiations were performed to evaluate the efficiency of permeation barriers under high flux reactor (HFR) conditions. Tritium extraction and permeation characteristics from Pb-17Li under variable temperatures 553-723 K, H 2 doping (0-1 vol%) and purge gas flow rates 20-100 scc/min were tested in LIBRETTO-3. An external TiC coating, an internal (TiC + A1203), both produced by chemical vapour deposition (CVD), and an internal AI203 produced by pack cementation (PC) on AISI 316L steel were tested as permeation barriers. The release mechanisms, experimental uncertainties and method for permeation barriers qualification are presented. As a result permeation reduction factors (PRF) at 0.1 dpa of 17 and 34 were obtained for the CVD-A1203 at 498 K and for the PC-A1203 at 508 K, respectively. These values were confirmed by a residence time analysis and are higher than in a preliminary analysis .

Research paper thumbnail of Sleep-related hyperkinetic seizures: always a frontal onset

Neurological Sciences, 2005

Hyperkinetic seizures are considered a typical manifestation of nocturnal frontal lobe epilepsy (... more Hyperkinetic seizures are considered a typical manifestation of nocturnal frontal lobe epilepsy (NFLE). Patients with temporal lobe epilepsy with mainly sleeprelated seizures have been described; however they commonly lack hyperkinetic activity and seizure frequency is low. We retrospectively analysed our population of 442 consecutive patients surgically treated between January 1996 and January 2004. Among these there were 25 patients with sleep-related hyperkinetic epileptic seizures, with a frontal lobe onset in 18 cases and a temporal lobe onset in 7. Patients with sleep-related hyperkinetic seizures with temporal lobe origin had anamnestic and clinical features strikingly similar to those with a frontal onset, with agitated movements, high seizure frequency and no history of febrile convulsions. We confirm our previous findings that this kind of epileptic manifestation is not only peculiar to frontal lobe epilepsy.

Research paper thumbnail of Surgical treatment of drug-resistant nocturnal frontal lobe epilepsy

Brain, 2007

Of the cases with nocturnal frontal lobe epilepsy (NFLE) 30% are refractory to antiepileptic medi... more Of the cases with nocturnal frontal lobe epilepsy (NFLE) 30% are refractory to antiepileptic medication, with several patients suffering from the effects of both ongoing seizures and disrupted sleep. From a consecutive series of 522 patients operated on for drug-resistant focal epilepsy, 21 cases (4%), whose frontal lobe seizures occurred almost exclusively (>90%) during sleep, were selected. All patients underwent a comprehensive presurgical evaluation, which included history, interictal EEG, scalp video-EEG monitoring, high-resolution MRI and, when indicated, invasive recording by stereo-EEG (SEEG). There were 11 males and 10 females, whose mean age at seizure onset was 6.2 years, mean age at surgery was 24.7 years and seizure frequency ranged from <20/month to >300/month. Nine patients reported excessive daytime sleepiness (EDS). Prevalent ictal clinical signs were represented by asymmetric posturing (6 cases), hyperkinetic automatisms (10 cases), combined tonic posturing and hyperkinetic automatisms (4 cases) and mimetic automatisms (1 case). All patients reported some kind of subjective manifestations. Interictal and ictal EEG provided lateralizing or localizing information in most patients. MRI was unrevealing in 10 cases and it showed a focal anatomical abnormality in one frontal lobe in 11 cases. Eighteen patients underwent a SEEG evaluation to better define the epileptogenic zone (EZ). All patients received a microsurgical resection in one frontal lobe, tailored according to pre-surgical evaluations. Two patients were operated on twice owing to poor results after the first resection. Histology demonstrated a Taylor-type focal cortical dysplasia (FCD) in 16 patients and an architectural FCD in 4. In one case no histological change was found. After a post-operative follow-up of at least 12 months (mean 42.5 months) all the 16 patients with a Taylor's FCD were in Engel's Class Ia and the other 5 patients were in Engel's Classes II or III. After 6 months post-surgery EDS had disappeared in the 9 patients who presented this complaint preoperatively. It is concluded that patients with drug-resistant, disabling sleep-related seizures of frontal lobe origin should be considered for resective surgery, which may provide excellent results both on seizures and on epilepsyrelated sleep disturbances. An accurate pre-surgical evaluation, which often requires invasive EEG recording, is mandatory to define the EZ. Further investigation is needed to explain the possible causal relationships between FCD, particularly Taylor-type, and sleep-related seizures, as observed in this cohort of NFLE patients. Surgery for drug-resistance NFLE Brain (2007), 130, 561-573 Surgery for drug-resistance NFLE Brain (2007), 130, 561-573 Surgery for drug-resistance NFLE Brain (2007), 130, 561-573 567 by guest on December 13, 2013 http://brain.oxfordjournals.org/ Downloaded from 570 Brain (2007), 130, 561-573 L. Nobili et al. by guest on December 13, 2013 http://brain.oxfordjournals.org/ Downloaded from Surgery for drug-resistance NFLE Brain (2007), 130, 561-573

Research paper thumbnail of Circulating levels of tumour necrosis factor-α and its soluble receptors are increased in the blood of patients with amyotrophic lateral sclerosis

Neuroscience Letters, 2000

An immunologic pathogenesis for amyotrophic lateral sclerosis (ALS) has been recently proposed. W... more An immunologic pathogenesis for amyotrophic lateral sclerosis (ALS) has been recently proposed. We tested the whole tumour necrosis factor (TNF) system in the serum of 51 ALS patients at different stages of the disease and 36 healthy controls. Antigenic TNF-a and its soluble receptors (sTNF-Rs), measured by ELISA, were signi®cantly higher in ALS patients than in healthy controls. However, biologically active TNF-a, corresponding to the sTNF-Rs-unbound trimeric TNFa molecule and assayed by its cytotoxic activity on a sensitive cell line, was similar between ALS patients and healthy controls. Neither antigenic TNF-a, bioactive TNF-a nor sTNF-Rs correlated with disease severity, disease duration, or weight loss. In conclusion, we reported an activation of the TNF system in ALS. The role of this activation in the pathogenesis of the disease remains elusive. q

Research paper thumbnail of Emotions Induced by Intracerebral Electrical Stimulation of the Temporal Lobe

Epilepsia, 2006

Summary: Purpose: To assess the quality and frequency of emotions induced by intracerebral electr... more Summary: Purpose: To assess the quality and frequency of emotions induced by intracerebral electrical stimulation of the temporal lobe.Methods: Behavioral responses were obtained by electrical stimulation in 74 patients undergoing presurgical video-stereo-EEG monitoring for drug-resistant epilepsy. Intracerebral electrical stimulation was performed by delivering trains of electrical stimuli of alternating polarity; the intensity could vary from 0.2 to 3 mA. Stimulation frequency was 1 Hz or 50 Hz. Nine hundred thirty-eight stimulation procedures were performed.Results: Seventy-nine emotional responses (ERs) were obtained (8.4%). Of these, 67 were “'fear responses.” Sad feelings were evoked 3 times, happy-pleasant feelings 9 times. Anger and disgust were never observed. The following variables affected the incidence of ER: (a) Anatomical site of stimulation. ERs (always fear) were maximal at the amygdala (12%) and minimal for lateral neocortical stimulation (3%, p < 0.01). (b) Pathology. Stimulation of a temporal lobe with hippocampal sclerosis was associated with a lower frequency of ERs compared with stimulation of a temporal lobe with no evidence of atrophy in the medial temporal structures. (c) Stimulation frequency. ERs were 12% at 50 Hz versus 6.0% at 1 Hz (p < 0.01). (d) Gender. In women fear responses were 16% compared with 3% in men (p < 0.01). There were no gender differences when analyzing nonemotional responses.Conclusions: These data confirm the role of the medial temporal lobe region in the expression of emotions, especially fear-related behaviors. Fear was observed more frequently in the absence of medial temporal sclerosis, supporting the hypothesis that emotional behaviors induced by stimulation are positive phenomena, strictly related to the physiological function of these regions. Further investigations should address why women express fear behaviors more frequently than men.

Research paper thumbnail of Sleep-related Minor Motor Events in Nocturnal Frontal Lobe Epilepsy

Epilepsia, 2007

Summary: Purpose: Nocturnal frontal lobe epilepsy (NFLE) is characterized by a wide spectrum of s... more Summary: Purpose: Nocturnal frontal lobe epilepsy (NFLE) is characterized by a wide spectrum of sleep-related motor manifestations of increasing complexity, ranging from major episodes to brief motor events (minor motor events, MMEs). NFLE patients may exhibit a large quantity of MMEs in the form of short-lasting stereotyped movements. Whereas major episodes are considered epileptiform manifestations, it remains unclear whether the MMEs are related to epileptiform discharges (EDs).Methods: To study the relation between EDs and the occurrence of MMEs, we report a detailed neurophysiolgical evaluation in NFLE subjects explored by using implanted electrodes.Results: The median value of ED-related movements was 71.8%. Motor expression in relation to epileptiform discharge was surprisingly variable; no peculiar expression of MMEs could be attributed to the presence of EDs.Conclusions: Our data suggest that ED-associated MMEs are extremely polymorphous, and no univocal relation to EDs can be identified. We hypothesize that MMEs are not a direct effect of epileptiform discharge (i.e., not epileptic in origin), but the result of aspecific disinhibition of innate motor patterns. We warn clinicians that the epileptic nature of minimal motor phenomena in NFLE cannot be established on the clinical phenomenology of the event.

Research paper thumbnail of Temporal Lobe Epilepsy in Children: Electroclinical Study of 77 Cases

Epilepsia, 2006

Summary: Purpose: Temporal lobe epilepsy (TLE) is probably more difficult to recognize in childre... more Summary: Purpose: Temporal lobe epilepsy (TLE) is probably more difficult to recognize in children than in adults. In fact, ictal symptoms in children are less stereotyped and less obvious, and the neuropathological substrate is more heterogeneous than in adults. The aim of this study is to examine the relationships between etiology, age at onset and electroclinical findings in 77 children with TLE, 32 of whom were surgically treated.Methods: Electroclinical study including video-EEG recording of seizures in 77 children with TLE. The investigation focused on the first five initial ictal symptoms.Results: Age at onset was less than 3 years in 39 cases, between 3 and 6 years in 17 cases and older than 6 years in 21 cases. Auras also occurred in younger children but were more common after the age of 6 years. A peculiar initial ictal semiology consisted in staring with arrest, lip cyanosis, and very slight oral automatisms. In some cases, EEG recordings documented seizures starting independently on both temporal lobes. Based on electroclinical and neuroradiological features, we recognized three subgroups: symptomatic TLE due to cortical malformations or nonevolutive tumors, TLE with mesial temporal sclerosis, and cryptogenic TLE.Conclusions: A correct electroclinical and neuroradiological approach allows in several cases early recognition of TLE even when onset is earlier than the age of 6 years. A correct definition of the localization relies primarily on video-EEG recording of the seizures, possibly repeated during follow up in cases lacking obvious neuroradiological correlation.

Research paper thumbnail of Ictal headache: Headache as first ictal symptom in focal epilepsy

Epilepsy & Behavior

Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is... more Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is often neglected because of the dramatic neurological manifestations of the seizure. Headache can also be the sole or predominant clinical manifestation of epileptic seizures, although this is a relatively rare condition. We describe two cases of focal symptomatic drug-resistant epilepsy with headache as the first ictal symptom. In both cases, the headache, which lasted a few seconds, was contralateral to the ictal discharge and did not have the clinical features of migraine. Ictal headache is a rare epilepsy symptom that can help to localize ictal EEG discharges. Recently, the term ictal epileptic headache has been proposed in cases in which headache is the sole ictal epileptic manifestation Diagnosis requires the simultaneous onset of headache with EEGdemonstrated ictal discharges.