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Biomedical subjects

F Vigevano

Publications and source records attributed to F Vigevano.

At least 37 records · Page 2Linked to original sources
PubMed1999

The effect of video-game software in video-game epilepsy.

PURPOSE: The individual role of video-game (VG) programs in VG activation is still unclear. Strict relations between VG seizures and photo- and pattern sensitivity suggest that programs per se may have a role in seizure activation. METHODS: We tested a series of 12 commercially available VG programs in 30 subjects aged 7-28 years; test protocol comprised intermittent photic stimulation (IPS), pattern stimulation, and a game session with 12 programs, each played for 5 min, delivered from a 50-Hz screen. RESULTS: Activation was observed in 17 subjects; marked differences were observed between different games: two programs activated 13 subjects; one program did not provoke activation. High variability between scenes makes it impossible to define mean brightness for the whole program. Activation correlated with "steady maximal brightness" (SMB) within a program; SMB is defined as the brightness in lux of the brightest scene steadily present in a program. SMB varied between 6 and 305 lux in tested programs. Difference in activation between different games was statistically significant (p < 0.001). Pattern sensitivity is strictly correlated with the probability of VG activation (p < 0.001). CONCLUSIONS: Our study demonstrates a strong variation in activation between different game programs and a strict relation between VG activation and pattern sensitivity. Programs with SMB >100 lux should be regarded as potentially dangerous: programs with SMB <50 lux may be considered relatively safe.

Adolescent↗
PubMed1999

No evidence of a major locus for benign familial infantile convulsions on chromosome 19q12-q13.1.

PURPOSE: A locus for benign familial convulsions (BFICs) has been recently mapped on chromosome 19q12-13.1 by studying five families of Italian descent. The main goal of this study was to investigate the role of this locus in a set of seven newly identified families with at least three affected cases. METHODS: Five polymorphic microsatellite markers covering the BFIC locus on chromosome 19q have been typed, and parametric linkage analysis has been performed to analyze the segregation of the BFIC locus within our families. RESULTS: Cumulative 2-point lod scores and multipoint analysis showed no evidence of linkage between chromosome 19 markers and the BFIC phenotype. The analysis of family-specific 2-point lod scores and haplotypes, however, indicated the presence of linkage to chromosome 19q in a single family, suggesting genetic heterogeneity within our family sample. CONCLUSIONS: Our study demonstrates that the previously reported BFIC locus on chromosome 19q12-13.1 is not a major locus for BFICs. We suggest that genetic heterogeneity may have generated our discordant linkage findings, as it was reported in benign familial neonatal convulsions, a related idiopathic mendelian syndrome.

Chromosome Mapping↗
PubMed1999

Benign myoclonus of early infancy.

Benign myoclonus of early infancy, first described by Fejerman and Lombroso, is a paroxysmal phenomenon of the first 2 years of life which occurs in neurologically healthy infants during wakefulness, and is usually triggered by excitement or frustration. We studied the neurophysiological features of the phenomenon in five children, aged 7 to 11 months, who were monitored by video-EEG recording, and by polygraphic recording in 3 of the 5 cases. The phenomenon is characterized by a shudder-type, paroxysmal motor manifestation involving mainly the trunk and sometimes the head, associated with tonic limb contractions of variable intensity, from hardly noticeable to more sustained. The EEG counterpart never showed modifications, the polygraphic study demonstrated a brief tonic limb contraction. The clinical manifestation should not be confused with the spasms of epileptic infantile spasms syndrome, or with tonic reflex seizures of early infancy. Although the phenomenon is already widely known, its polygraphic recording is rarely reported in literature. The polygraphic data of our patients contributes to the diagnosis and understanding of the pathophysiology of this paroxysmal manifestation.

Brain Mapping↗
PubMed1999

Repetitive sleep starts in neurologically impaired children: an unusual non-epileptic manifestation in otherwise epileptic subjects.

Sleep starts, also called hypnagogic or hypnic jerks, are bilateral, sometimes asymmetric, usually single, brief body jerks that coincide with sleep onset. We describe sleep starts occurring repetitively in three epileptic children with spastic-dystonic diplegia and mental retardation. Repetitive sleep starts began at age 18 months in two children and at 9 months in the third. All three children had had feto-neonatal asphyxia; two presented with spastic and one with dystonic tetraparesis. One had West syndrome and two had partial motor seizures in the first year of life. Seizures were controlled in all three patients by antiepileptic drug therapy. Video/EEG recordings of all the children during the afternoon nap revealed clusters of sleep starts during the transition between wakefulness and sleep. Cluster lasted 4-15 min and comprised from twenty to twenty-nine contractions. The EEG counterpart of the event sometimes showed an arousal response, at times inducing complete awakening. Repetitive sleep starts should be recognized and clearly differentiated from epileptic seizures, especially if they appear in epileptic subjects. In neurologically compromised patients, they could represent an intensification of an otherwise normal event, due to the lack of strong inhibitory influence of the pyramidal tract resulting from the pyramidal lesion.

Cerebral Cortex↗
PubMed1998

Epilepsy provoked by television and video games: safety of 100-Hz screens.

Television (TV) and video games (VG) can provoke seizures in patients with photosensitive epilepsies. Flicker frequency is the most important factor in screen activation. We tested conventional 50-Hz versus 100-Hz monitors during TV viewing and VG playing in 30 photosensitive subjects, 23 of whom had a history of TV or VG seizures or both. Fifteen subjects' discharges were activated by 50-Hz TV; 17 by 50-Hz VG; and one by a 100-Hz screen. Thus, 100-Hz screens protect against screen activation.

Adolescent↗
PubMed1997

Linkage mapping of benign familial infantile convulsions (BFIC) to chromosome 19q.

Benign familial infantile convulsions (BFIC) are an autosomal-dominant epileptic syndrome characterized by an age of onset within the first year of life. Although they were first reported in families of Italian descent, BFIC have also been described in non-Italian families. We have mapped the BFIC gene to chromosome 19 by linkage analysis in five Italian families with a maximum two-point lod score of 6.36 at D19S114; maximum multipoint lod scores > 8 were obtained for the interval D19S250-D19S245. BFIC are therefore the third idiopathic partial epileptic syndrome to be mapped on the human genome.

Chromosome Mapping↗
PubMed1997

Vigabatrin versus ACTH as first-line treatment for infantile spasms: a randomized, prospective study.

PURPOSE: To compare the efficacy and tolerability of vigabatrin (VGB) and adrenocorticotrophic hormone (ACTH) as first-line therapy in infantile spasms. METHODS: Forty-two infants (22 males, 20 females) aged 2-9 months with newly diagnosed infantile spasms, were included in the trial. Patients were randomized to receive VGB 100-150 mg/kg/day or Depot ACTH 10 IU/day. The alternative drug was given if spasms were not controlled within 20 days or in cases of intolerance to initial therapy. Twenty-three patients (7 cryptogenic, 16 symptomatic) received VGB as first-line therapy; 19 patients (8 cryptogenic, 11 symptomatic) received ACTH as the first drug. RESULTS: Cessation of spasms was observed in 11 (48%) of the patients randomized to VGB and in 14 (74%) of those randomized to ACTH. Response to VGB was observed within 1-14 days, but two-thirds of patients (7/11) responded within 3 days. In the group treated with VGB, side effects such as drowsiness, hypotonia and irritability were observed in 13% of patients, compared with 37% in the group treated with ACTH. VGB was more effective than ACTH as treatment for cerebral malformations or tuberous sclerosis, whereas ACTH proved more effective in perinatal hypoxic/ischemic injury. The efficacy of the two drugs was similar in cryptogenic cases. Disappearance of interictal EEG abnormalities occurred sooner in patients randomized to ACTH than in those who received VGB as initial therapy. During the second phase, the alternative drug was given to the resistant patients. Spasms ceased in 2 of 5 patients treated with VGB and in 11 of 12 patients treated with ACTH. After 3 months, relapses of spasms were observed in 6 patients treated with ACTH and in 1 treated with VGB. VGB produced a therapeutic response in nearly half the patients receiving this drug. CONCLUSIONS: Our data lend further support to the view that VGB may be considered a first-choice drug in the treatment of IS.

Adrenocorticotropic Hormone↗
PubMed1997

Early-onset benign occipital seizure susceptibility syndrome.

PURPOSE: Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. METHODS: This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting. RESULTS: One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year. CONCLUSIONS: Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.

Adolescent↗
PubMed1996

Felbamate in therapy-resistant epilepsy: an Italian experience. Felbamate Italian Study Group.

We evaluated the use of felbamate in 379 adults and children with refractory epilepsies in an open-label, compassionate clinical use setting. Prior to the termination of the program, because of reports of aplastic anemia, 351 patients had completed 2 months of treatment with felbamate at a dose of 2400-3600 mg/day for adults or 30-45 mg/kg/day for children. Of the 246 patients who had a diagnosis of therapy-refractory localization-related epilepsy with or without secondary generalization, 52% (126/246) achieved a seizure reduction of 50% or more, including 10% (25/246) who became seizure free. There was no difference in response rate between adults and children. Of the 80 patients who had a diagnosis of Lennox-Gastaut syndrome (LGS), 60% (48/80) achieved a seizure reduction of 50% or more, including 6% (5/80) who became seizure free. Of the 25 patients with a diagnosis of generalized epilepsy (other than LGS) or undetermined epilepsy whether focal or generalized, 60% (15/25) achieved a seizure reduction of 50% or more, including 12% (3/24) who became seizure free. The results of this uncontrolled study suggest that felbamate could be useful in patients with epilepsies which are refractory to other antiepileptic drugs after careful risk-benefit assessment and consideration of all circumstances involved.

Adolescent↗
PubMed1995

Hemimegalencephaly, hemihypertrophy and vascular lesions.

UNLABELLED: We report on two children with hemihypertrophy and ipsilateral hemimegalencephaly. Vascular lesions in one were consistent with a diagnosis of the Klippel-Trénaunay-Weber Syndrome. MRI performed in the first days of life and at 1 month of age revealed the presence of the neuronal anomaly. CONCLUSION: The occurrence of hemimegalencephaly in our patients indicates that hemihypertrophy and vascular dysplasia are pathogenetically related phenomena of a continuous spectrum in which this brain disorder may appear.

Blood Vessels↗
PubMed1995

Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study).

Patients having a hypothalamic hamartoma frequently present epileptic attacks of laughter, and they later experience multiple additional seizure types, which invariably lead to a severe drug-resistant epilepsy. If this association is now well-known, relationships between the hypothalamic mass and the different types of seizures remain still mysterious. We report the case of a 16-year-old girl suffering from this peculiar epileptic picture, in whom a stereo-EEG study was performed, allowing us to record both the hamartoma, the neighboring hypothalamic structures, and other bilateral cortical areas. It showed that gelastic fits were strictly linked to ictal discharges which began and remained well localized in the hamartoma. Conversely, atonic seizures, which might result from a secondary epileptogenesis, admitted a widely extended bilateral frontal cortical origin, sparing the lesion, and slightly involving the posterior hypothalamus. Stereotactic radiosurgery of the hamartoma proved to be ineffective on both types of seizures, probably because of the too low dose of X-rays delivered (18 grays), as suggested by the absence of hypothalamic mass changes on MRI. Such data, never reported to our knowledge, seem able to contribute to a better understanding of this very peculiar epileptic syndrome, and perhaps to a better adapted therapeutic management.

Adolescent↗
PubMed1995

Reflex myoclonic epilepsy in infancy: a new age-dependent idiopathic epileptic syndrome related to startle reaction.

Benign myoclonic epilepsy of infancy (BMEI) is an idiopathic disorder characterized by spontaneous myoclonic attacks with onset in the first 2 years of life. We observed 6 neurologically normal infants (aged 6-21 months) with attacks that resembled those of BMEI but that occurred as reflex responses to unexpected auditory and tactile stimuli. four infants also had rare spontaneous attacks. These reflex attacks consisted of isolated muscle jerks or clusters of up to eight symmetric limb jerks affecting mainly the arms. Five of the children had a family history of epilepsy or febrile convulsions. Myoclonic attacks disappeared in 4-14 months. In 3 patients, the jerks stopped spontaneously; the others responded to valproate (VPA). Myoclonus could be elicited in wakefulness and in sleep. Ictal EEGs showed brief generalized spike- or polyspike-and-wave discharges. Interictal EEGs were normal during wakefulness; during sleep, brief generalized discharges were evident. We propose that reflex myoclonic epilepsy of infancy (RMEI) is a new age-dependent idiopathic generalized epileptic (IGE) syndrome, with an apparently good prognosis.

Acoustic Stimulation↗
PubMed1995

Efficacy and tolerability of vigabatrin in children with refractory partial seizures: a single-blind dose-increasing study.

The efficacy and tolerability of vigabatrin (VGB) in children with refractory partial epilepsy were assessed in a single-blind, add-on, fixed-sequence, placebo-controlled trial. After 1-month observation, the patients entered a 7-month treatment period that involved administration of placebo for 1 month followed by VGB at the initial dosage of 40 mg/kg/day, to be increased to 60 and 80 mg/kg/day at 2-month intervals if seizures persisted. Of the 46 children enrolled in the study, 7 dropped out prematurely due to lack of efficacy of the drug (n = 6) or increased seizure frequency (n = 1). In 11 patients who either became seizure-free (n = 3) or improved markedly (n = 8), treatment was completed at a dose < 80 mg/kg/day. The average number of seizures per month in the 39 patients who completed the study decreased from 97 during placebo to 21, 12, and 9 after 2, 4, and 6 months of VGB treatments respectively (p < 0.0001 at each time). Response to VGB remained statistically significant when dropouts were included in the evaluation. The number of patients who had > 50% reduction in seizure frequency after 2, 4, and 6 months was 28, 33, and 35, respectively. Eight patients became seizure-free during the last 2 months of VGB treatment (3 at 40, 3 at 60, and 2 at 80 mg/kg/day, as compared with none during placebo treatment). Serum levels of associated antiepileptic drugs (AEDs) showed no significant changes, except for serum phenytoin (PHT) concentration, which significantly (p < 0.01) decreased after VGB treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Anticonvulsants↗
PubMed1994

Bilateral, reversible, selective thalamic involvement demonstrated by brain MR and acute severe neurological dysfunction with favorable outcome.

We report on two children who presented acute, severe, neurological dysfunction with bilateral, reversible, selective thalamic lesions demonstrated by brain MRI. In both children neurological symptoms appeared two weeks after a febrile respiratory illness. Clinical conditions worsened in a few days to a stuporous state and tetraplegia in one and to coma with decerebrate posturing in the other. Three weeks after the onset, both children improved and recovered within one month. During the acute phase, brain MRI showed in both children bilateral hyperintense areas on T2-weighted sequences limited to both thalamic regions. During the follow-up, repeated brain MRI showed complete disappearance of abnormalities in one patient and a small residual left thalamic lesion in the other. In both patients hematological routine exams were normal. Bacterial and viral studies of serum and CSF were negative. CSF findings showed elevated white blood cell count and protein levels, with no oligoclonal IgG bands. Urine and CSF organic acids by GC/MS and plasma as well as CSF amino acids were normal. We believe that the benign evolution of this disorder and CSF findings strongly suggest a postinfectious process of the central nervous system.

Child, Preschool↗
PubMed1994

[Surgical treatment of epileptogenic hemimegalencephaly].

Thirteen children affected by hemimegalencephaly were observed in the Pediatric Section of the Institute of Neurosurgery of the Catholic University of Rome in the last six years. Nine of them were operated because of an intractable epilepsy. Seven were males and 2 females; the age at operation ranged between 7 months and 11 years (mean: three years and five months); the follow-up period varied between 1 and 6 years (mean 3 years and 10 months). All the patients had a clinical history of daily epileptic seizures not responsive to medical treatment; all of them presented with severely delayed psychomotor development. At neurological examination, six children showed a motor deficit of variable severity contralateral to the affected hemisphere and two patients a severe tetraparesis. The remaining child did not present with motor deficit. In all the cases the diagnosis had been obtained by CT scan and MRI. One of the cerebral hemispheres was abnormally enlarged with associated dilation of the lateral cerebral ventricle. The cortical architecture was obviously deranged with several areas of heterotopia of the gray substance suggesting an alteration of the neuronal cell migration. All the children underwent an extrathalamocaudato hemispherectomy. A post-operative ventriculo-peritoneal shunt was required in two cases. There were neither operative, nor late deaths. A dramatic reduction in the frequency and severity of epileptic seizures was observed in all but one of the patients of the series.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗
PubMed1993

Benign neonatal sleep myoclonus: clinical features and video-polygraphic recordings.

Benign neonatal sleep myoclonus is a syndrome characterized by the occurrence of repetitive myoclonic jerks of the extremities exclusively during non-rapid eye movement sleep in the early life of healthy newborns. No etiological factors are present. The onset is within the first 15 days of life with spontaneous disappearance within 3-4 months. These myoclonic events are commonly diagnosed as epileptic seizures. We observed 12 newborns with this clinical pattern; the follow-up ranges from 12 to 60 months. Long-term videopolygraphic electroencephalographic (EEG) monitoring demonstrated normal EEG activity, thus confirming that these unusual events were nonepileptic.

Brain Damage, Chronic↗
PubMed1993

Occipital seizures provoked by intermittent light stimulation: ictal and interictal findings.

Occipital seizures provoked by intermittent light stimulation are rare events described in patients with symptomatic and idiopathic epilepsy as well as in nonepileptic individuals. We observed five patients ranging in age from 10 to 32 years, who presented with focal seizures provoked by photic stimuli; none of them had demonstrable occipital lesions. Four patients were epileptic, and one was nonepileptic. Three patients had occipital epilepsy with visual seizures; one had generalized epilepsy with absence attacks; the nonepileptic had migraine. In the patients with visual seizures, interictal EEGs showed occipital foci in two cases and bilateral spontaneous and evoked spikes as well as generalized discharges in the third. The patient with absence attacks had generalized discharges. The migraine patient had a normal EEG. Ictal EEGs showed a focal lateralized theta activity at the onset of seizures in three patients, in one a fast recruiting rhythm intermixed with generalized paroxysms reflex to blinking, and in the other a rapid bioccipital fast rhythm spreading to the anterior regions. Occipital seizures provoked by intermittent light stimulation probably constitute a rare type of photosensitivity, sometimes appearing in occipital epilepsies, generalized epilepsies, and migraine.

Adolescent↗