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BACKGROUND Deciding on a disease modifying therapy (DMT) for the treatment of pediatric onset multiple sclerosis (POMS) often presents a challenge to families. Parents are often overwhelmed by DMT choices, but they desire to be an integral part of the decision making process for their child. There is no standard approach for how best to involve families in this process. The aim of this study was to describe the experience of decision making related to the use of disease modifying therapy in parents of children and adolescents with POMS. METHODS The research aim was addressed using a descriptive survey design. Participants were recruited from the Pediatric MS and Related Disorders Program at Boston Children's Hospital as well as from the Pediatric Multiple Sclerosis Alliance online Facebook group. RESULTS Overall, fewer than half of parents felt very satisfied with the DMT they chose for their child with POMS (44%). Parental satisfaction with the decision making process increased with a high level of control of the process (p

AIM To determine predictors of full-scale IQ (FSIQ) in an international pediatric opsoclonus myoclonus syndrome (OMS) cohort. METHOD In this retrospective and prospective cohort study at three academic medical centers (2006-2013), the primary outcome measure, FSIQ, was categorized based on z-score: above average (≥+1), average (+1 to -1), mildly impaired (-1 to -2), and impaired (

Tuberous sclerosis complex (TSC) is an autosomal dominant tumor suppressor gene syndrome due to germline mutations in either TSC1 or TSC2. 10–15% of TSC individuals have no mutation identified (NMI) after thorough conventional molecular diagnostic assessment. 53 TSC subjects who were NMI were studied using next generation sequencing to search for mutations in these genes. Blood/saliva DNA including parental samples were available from all subjects, and skin tumor biopsy DNA was available from six subjects. We identified mutations in 45 of 53 subjects (85%). Mosaicism was observed in the majority (26 of 45, 58%), and intronic mutations were also unusually common, seen in 18 of 45 subjects (40%). Seventeen (38%) mutations were seen at an allele frequency < 5%, five at an allele frequency < 1%, and two were identified in skin tumor biopsies only, and were not seen at appreciable frequency in blood or saliva DNA. These findings illuminate the extent of mosaicism in TSC, indicate the importance of full gene coverage and next generation sequencing for mutation detection, show that analysis of TSC-related tumors can increase the mutation detection rate, indicate that it is not likely that a third TSC gene exists, and enable provision of genetic counseling to the substantial population of TSC individuals who are currently NMI., Author Summary Tuberous sclerosis complex (TSC) is a human genetic disorder due to mutations in the TSC1 or TSC2 genes. A mystery for many years has been the fact that with standard genetic testing 10–15% of TSC patients have had no mutation identified (NMI) in either TSC1 or TSC2. We examined the genetic cause of TSC in patients who were ‘NMI’ after previous testing. We found a mutation in TSC1 or TSC2 in the vast majority of the subjects studied: 45 of 53 (85%). The majority of mutations identified were either in introns or mosaic or both. Usually we expect to find mutations causing human disease in exons, coding parts of genes. However, mutations can also be found in introns, the non-coding parts of genes, and we found intronic mutations in 18 of 45 subjects (40%). Mosaic mutations were seen in 26 of 45 subjects (58%). Mosaicism is the situation in which different cells in the body have a different genetic make-up, and in this case the mutations in TSC1/TSC2 were present in only a fraction of the cells from the patient. So these two types of hard-to-find mutations (in introns and/or mosaic) explain the majority of TSC patients who were NMI.

Background Although epilepsy affects most patients with tuberous sclerosis complex (TSC), little is known about the natural history of epilepsy in this genetic disease.

To review the efficacy, cognitive outcome and safety profile in children treated with vigabatrin (VGB) for infantile spasms (IS) and partial epilepsies related to tuberous sclerosis complex (TSC) and other etiologies.Retrospective review of children followed in the Pediatric Epilepsy Program of Massachusetts General Hospital for Children between May 2001 and March 2006 who were treated with VGB.Eighty-four children were treated with VGB, 68 of them were treated for IS, and 59 were treated for partial seizures (PS). Etiology (TSC or other) was the only predictive factor for IS control with VGB (p0.0003). IS control was achieved in 73% of children with TSC and 27% of children with other etiologies (combined 56%). Partial onset seizures were controlled in 34% of all children, (17% seizure free,17%reduction in seizure frequency50%) and no predictive factor was found. Shorter time from seizure onset to VGB treatment (p0.027) and longer total time on VGB (p0.045) was associated with better IQ-developmental quotient (DQ) outcome in children treated for IS, but not with IS control. Adverse events were seen in 13%. Electroretinogram and/or behavioral visual field (VF) testing was done in 52%. VGB was discontinued in one case due to abnormal electroretinogram (ERG) findings.We confirm the efficacy of VGB in the treatment of IS and PS in an American population. VGB may improve cognitive outcome in the absence of complete IS control, but this finding is of uncertain clinical significance. VGB was well tolerated, and ophthalmologic side effects were uncommon.

To evaluate the sensitivity of a simultaneous whole-head 306-channel magnetoencephalography (MEG)/70-electrode EEG recording to detect interictal epileptiform activity (IED) in a prospective, consecutive cohort of patients with medically refractory epilepsy that were considered candidates for epilepsy surgery.Seventy patients were prospectively evaluated by simultaneously recorded MEG/EEG. All patients were surgical candidates or were considered for invasive EEG monitoring and had undergone an extensive presurgical evaluation at a tertiary epilepsy center. MEG and EEG raw traces were analysed individually by two independent reviewers.MEG data could not be evaluated due to excessive magnetic artefacts in three patients (4%). In the remaining 67 patients, the overall sensitivity to detect IED was 72% (48/67 patients) for MEG and 61% for EEG (41/67 patients) analysing the raw data. In 13% (9/67 patients), MEG-only IED were recorded, whereas in 3% (2/67 patients) EEG-only IED were recorded. The combined sensitivity was 75% (50/67 patients).Three hundred and six-channel MEG has a similarly high sensitivity to record IED as EEG and appears to be complementary. In one-third of the EEG-negative patients, MEG can be expected to record IED, especially in the case of lateral neocortical epilepsy and/or cortical dysplasia.

SUMMARY: Distinguishing propagated epileptic activity from primary epileptic foci is of critical importance in presurgical evaluation of patients with medically intractable focal epilepsy. We studied an 11-year-old patient with complex partial epilepsy by using simultaneous magnetoencephalography (MEG) and electroencephalography (EEG). In EEG, bilateral interictal discharges appeared synchronous, whereas MEG source analysis suggested propagation of spikes from the right to the left frontal lobe.

Context We have previously shown that serum VEGF-D is elevated at baseline, correlates with kidney angiomyolipoma size at baseline and 12 months, and decreases with sirolimus treatment in adults with tuberous sclerosis complex (TSC). To further investigate the utility of serum VEGF-D for longer term monitoring of TSC kidney disease, we present VEGF-D level results with 24 month follow-up. Objective To compare 24 month VEGF-D levels in two subgroups of sirolimus treated patients (OFF SIROLIMUS AFTER 12 MONTHS or ON SIROLIMUS AFTER 12 MONTHS). Design and Intervention(s) Serum VEGF-D was measured in samples collected from subjects enrolled in a phase 2 multicenter trial evaluating sirolimus for the treatment of kidney angiomyolipomas associated with TSC or TSC/LAM. All participants were treated with sirolimus from 0–12 months. During months 12–24, sirolimus was discontinued in one subgroup. The other subgroup was treated with additional sirolimus. Setting Adult TSC participants were recruited from six clinical sites in the United States (comprehensive TSC clinics, 5; urology clinic, 1). Patients There were 28 TSC patients who completed all 24 months of the study and serum samples were available at 24 months from 18/28 patients. Main Outcome Measure(s) We compared the percent change in VEGF-D levels (baseline to 24 months) in patients from the two treatment subgroups. Results At 24 months, VEGF-D levels decreased by 67% compared with baseline (to 787±426 pg/ml) in the ON SIROLIMUS AFTER 12 MONTHS group versus a 13% decrease (to 2971±4014 pg/ml) in the OFF SIROLIMUS AFTER 12 MONTHS group (p = 0.013, Mann-Whitney test). A similar trend was observed in kidney angiomyolipoma size but not in pulmonary function tests. Conclusions Serum VEGF-D may be useful for monitoring response to treatment with sirolimus and kidney angiomyolipoma size in patients with TSC, but confirmation is needed. Trial Registration Clinical trials.gov NCT00126672.

Objective The aim of the work described here was to systematically analyze the developmental trajectory of patients with tuberous sclerosis complex (TSC). Methods A retrospective longitudinal chart review was performed, selecting patients who received multiple neuropsychological assessments. Intellectual/Developmental Quotients, Adaptive Behavior Composite scores, and clinical data were collected. On available EEGs, interictal epileptiform discharges were counted. Results Sixty-six (18%) patients with TSC received multiple cognitive and adaptive development assessments. The mean intelligence of this study group remained relatively stable, albeit variable. Significant decline in adaptive functioning was observed, associated with lower age at seizure onset. Patients who underwent neurosurgery prior to baseline testing showed cognitive improvement. Developmental declines were significantly associated with increased numbers of antiepileptic drugs, with a trend toward association with mutation type and interictal epileptiform discharges. Conclusion This study suggests that the developmental course of patients with TSC may be altered by epilepsy comorbidity and neurosurgery, underlining the need for early and effective interventions in this population.

Background Tuberous sclerosis (TSC) related tumors are characterized by constitutively activated mTOR signaling due to mutations in TSC1 or TSC2. Methods We completed a phase 2 multicenter trial to evaluate the efficacy and tolerability of the mTOR inhibitor, sirolimus, for the treatment of kidney angiomyolipomas. Results 36 adults with TSC or TSC/LAM were enrolled and started on daily sirolimus. The overall response rate was 44.4% (95% confidence intervals [CI] 28 to 61); 16/36 had a partial response. The remainder had stable disease (47.2%, 17/36), or were unevaluable (8.3%, 3/36). The mean decrease in kidney tumor size (sum of the longest diameters [sum LD]) was 29.9% (95% CI, 22 to 37; n = 28 at week 52). Drug related grade 1–2 toxicities that occurred with a frequency of >20% included: stomatitis, hypertriglyceridemia, hypercholesterolemia, bone marrow suppression (anemia, mild neutropenia, leucopenia), proteinuria, and joint pain. There were three drug related grade 3 events: lymphopenia, headache, weight gain. Kidney angiomyolipomas regrew when sirolimus was discontinued but responses tended to persist if treatment was continued after week 52. We observed regression of brain tumors (SEGAs) in 7/11 cases (26% mean decrease in diameter), regression of liver angiomyolipomas in 4/5 cases (32.1% mean decrease in longest diameter), subjective improvement in facial angiofibromas in 57%, and stable lung function in women with TSC/LAM (n = 15). A correlative biomarker study showed that serum VEGF-D levels are elevated at baseline, decrease with sirolimus treatment, and correlate with kidney angiomyolipoma size (Spearman correlation coefficient 0.54, p = 0.001, at baseline). Conclusions Sirolimus treatment for 52 weeks induced regression of kidney angiomyolipomas, SEGAs, and liver angiomyolipomas. Serum VEGF-D may be a useful biomarker for monitoring kidney angiomyolipoma size. Future studies are needed to determine benefits and risks of longer duration treatment in adults and children with TSC. Trial Registration Clinicaltrials.gov NCT00126672

P796 – Figure 1 LP31 EEG and MEG source localization of the epileptogenic foci in tuberous sclerosis complex: a pediatric case report A. Hunold1, J. Haueisen1, B. Ahtam2,3, C. Doshi2,4, C. Harini4, S. Camposano4, S.K. Warfield5,6, P.E. Grant2,3,6, Y. Okada2,4, C. Papadelis2,4 1Technische Universitaet Ilmenau, Institute of Biomedical Engineering and Informatics, Ilmenau, Germany; 2Boston Children’s Hospital, Harvard Medical School, Fetal-Neonatal Neuroimaging & Developmental Science Center, Boston, MA, United States; 3Boston Children’s Hospital, Harvard Medical School, Department of Newborn Medicine, Boston, MA, United States; 4Boston Children’s Hospital, Harvard Medical School, Department of Neurology, Boston, MA, United States; 5Boston Children’s Hospital, Harvard Medical School, Department of Radiology, Boston, MA, United States; 6Boston Children’s Hospital, Harvard Medical School, Computational Radiology Laboratory, Boston, MA, United States Question: Tuberous sclerosis complex (TSC) is a disorder of tissue growth and differentiation, characterized by benign hamartomas in the brain triggering epilepsy in up to 90% of TSC patients. There is an ongoing debate on whether or not the epileptogenic zone is within the tuber itself or in abnormally developed surrounding tissue. Methods: We examined a four-year old patient with TSC-related refractory epilepsy undergoing magnetoencephalography (MEG) and electroencephalography (EEG) recordings. For MEG, we used a prototype system that offers higher spatial resolution and sensitivity compared to the conventional adult systems. EEG was simultaneously recorded from 32-leads according to the 10-20 international system. The source analysis of interictal activity was performed using both EEG and MEG data. Equivalent current dipoles (ECD) were fitted to the peak of individual interictal spikes. For averaged interictal spike signals, we performed ECD localizations to the spike upslope. Further, we estimated the minimum norm estimates (MNEs) to averaged interictal spike signals. Results: Multiple cortical tubers were identified in patient’s MRI including one prominent calcified tuber in the right parietal-occipital lobe. The simultaneously recorded spikes in EEG and MEG data provided a time shift of 20 ms between peak latencies. ECDs localized to individual and averaged interictal activity in EEG and MEG consistently clustered in the millimeter Abstracts of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S175s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S175 vicinity of the large calcified cortical tuber. The ECD trace localized to the averaged EEG data located on the posterior side ∼5 mm superior to the tuber. The ECD trace localized to the averaged MEG spike located ∼4 mm anterior to the tuber. MNE and ECDs indicated epileptiform activity in the same areas. Conclusion: Our source analysis indicated generators of epileptiform activity in the millimeter vicinity of the tuber margin outside the tuber volume. Separate EEG and MEG source analysis provided distinct source characteristics. LP32 Scalp EEG in malformations of cortical development (MDC). Analysis by localization S. Cieza, A. Gomez, F. Escobar, N. Pereira, C. Viteri, J. Artieda, E. Urrestarazu Clinica Universidad de Navarra, Neurophysiology, Pamplona, Spain Objectives: The aim of our study was to describe the EEG findings of the MDC according to the localisation in the MRI. Patients and methods: We analyze the scalpEEGs of the patients diagnosed of MDC by RM in the Clinica Universidad de Navarra, and we considerate: the background activity, the presence and regional localisation of focal slow waves, interictal epileptiform discharges and focal fast frequencies. Results: We identified 51 patients with MDC (31 women and 20 men). In 21 patients the MDC was located in the temporal lobe, 12 showed focal slow waves and 13 showed epileptiform discharges. These EEG abnormalities had a high topographic correlation with MRI findings (>80% for slow waves and>90% for epileptiform discharges). In 19 patients the lesion was located in the frontal lobe, and the EEG showed focal slow waves in 12 of these patients, and epileptiform discharges in 9; however, these abnormalities coincide with the location in the MRI in less than 50% of the cases. In 8 patients the lesion was located in the parietal lobe, and just in only one case the EEG abnormalities correctly identified the location showed by MRI. Finally in 3 patients the lesion was located in the occipital lobe, and EEG showed slow waves in one case and epileptiform discharges in 2 cases. Only 2 patients had theta-alpha rhythm and were well correlated with the MRI findings (frontal and temporal MDC). One patient with temporal lobe MDC had a no localising continuos beta rhythm. Conclusions: Most of the MDC were located in the temporal and frontal lobes. The slow waves and the epileptiform discharges were highly well localising when the lesion was located in the temporal lobe, but failed in more than 50% of the cases when it was extra temporal. Focal rhythms were rare but have a good correlation with dysplasia. Therefore the presence and locator value of the EEG abnormalities are highly dependent on the location of the focus. Poster session 27. ICU monitoring P472 Epileptiform activity in patients treated with therapeutic hypothermia after cardiac arrest C. Santos-Sanchez1, M. Agundez Sarasola2, X. Mancisidor Solaberrie3, A. Martin Lopez3, P. Goiriena Seijo3, T. Perez Concha2, I. Yurrebaso Santamaria1 1Cruces University Hospital, Clinical Neurophysiology, Barakaldo, Spain; 2Cruces University Hospital, Neurology, Barakaldo, Spain; 3Cruces University Hospital, Cardiology, Barakaldo, Spain Question: Since the implementation of therapeutic hypothermia (TH) after cardiac arrest (CA), some researchers have been trying to redefine the value of the parameters traditionally used as predictors of neurologic outcome. In this context, the EEG represents an important tool to improve prognostication in postanoxic coma. Methods: We retrospectively reviewed the electronic medical records of all patients undergoing TH after CA from January 2011 until October 2013. We identified the patients with any type of epileptiform activity and reviewed their EEGs. Results: Forty patients were included. Sixteen died (40%). Epileptiform activity was found in 11 patients (27.5%), from whom only 2 (18.2%) had a favorable outcome (CPC 1 or 2). This epileptiform activity appeared in the following patterns; 2 patients had isolated interictal discharges, 2 had 2-2.5 Hz generalized periodic epileptiform discharges (GPEDs) over a continuous, diffusely slowed background, 2 had 2-2.5 Hz GPEDs over a suppressed background and 6 fulfilled non convulsive status epilepticus (NCSE) criteria. In one of the patients with NCSE, the EEG evolved into the pattern of GPEDs at 2-2.5 Hz over a suppressed background. When treating these 3 patients with benzodiazepine trials, even though they remained comatose, the epileptiform discharges stopped, and a continuous theta background appeared. Two of these patients died eventually, and the other one remains in a persistent vegetative state. Conclusions: Epileptiform activity is common in comatose patients treated with TH after CA. The presence of epileptiform discharges during the TH or shortly after was associated with a poor outcome. P473 Defining EEG reactivity in patients treated with therapeutic hypothermia after cardiac arrest C. Santos-Sanchez1, M. Agundez Sarasola2, X. Mancisidor Solaberrie3, P. Goiriena Seijo3, A. Martin Lopez3, T. Perez Concha2, I. Yurrebaso Santamaria1 1Cruces University Hospital, Clinical Neurophysiology, Barakaldo, Spain; 2Cruces University Hospital, Neurology, Barakaldo, Spain; 3Cruces University Hospital, Cardiology, Barakaldo, Spain Question: In patients treated with therapeutic hypothermia (TH) after cardiac arrest (CA), the EEG reactivity in the first 24 hours has an important prognostic value. However, it has not been reported yet whether or not there are differences between the type of stimuli required or the reactivity patterns obtained. Methods: We prospectively assessed EEG background continuity and reactivity in 16 consecutive patients undergoing TH after CA from August 2012 to October 2013. EEG background reactivity was tested to auditory, tactile and painful stimuli. We analyzed the relation between the continuity of the background, the EEG reactivity patterns, and the neurologic outcome. Results: All of our patients had the first EEG done within 24 hours after the CA. We established 4 backgrounds categories; continuous, discontinuous, burst-suppression and low voltage, and 2 reactivity patterns; increase of faster frequencies and background attenuation. All survivors (11/16) had different degrees of background reactivity in the first EEG (

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome caused by mutations in TSC1 and TSC2. However, 10 to 15% TSC patients have no mutation identified with conventional molecular diagnostic studies. We used the ultra-deep pyrosequencing technique of 454 Sequencing to search for mosaicism in 38 TSC patients who had no TSC1 or TSC2 mutation identified by conventional methods. Two TSC2 mutations were identified, each at 5.3% read frequency in different patients, consistent with mosaicism. Both mosaic mutations were confirmed by several methods. Five of 38 samples were found to have heterozygous non-mosaic mutations, which had been missed in earlier analyses. Several other possible low frequency mosaic mutations were identified by deep sequencing, but were discarded as artifacts by secondary studies. The low frequency of detection of mosaic mutations, 2 (6%) of 33, suggests that the majority of TSC patients who have no mutation identified are not due to mosaicism, but rather other causes, which remain to be determined. These findings indicate the ability of deep sequencing, coupled with secondary confirmatory analyses, to detect low frequency mosaic mutations.

Left handers and women show less anatomical brain asymmetry, larger corpus callosum and more bilateral representation of specific functions. Sensory and cognitive components of cortical auditory evoked potentials (AEF) have been shown to be asymmetric in right handed males and to be influenced by stimulus intensity. In this study the influence of sex, handedness and stimulus intensity upon AEP components is investigated under basal conditions of passive attention. 14 right handed males, 14 right handed females, 14 left handed males, and 14 left handed females were studied while lying awake and paying passive attention to auditory stimulation (series of 100 binaural clicks, duration 1 msec, rate 1/sec, at four intensities). Cz, C3 and C4 referenced to linked mastoids and right EOG were recorded. Analysis time was 400 msec, average evoked potentials were based on 100 clicks. Stimulus intensity and gender affect early sensory components (P1N1 and N1P2) at central leads, asymmetry is influenced only by handedness, right handers showing larger P1N1 amplitudes over the right hemisphere. Los sujetos zurdos y de sexo femenino tienden a exhibir menores grados de asimetria anatómica, mayores diámetros del cuerpo calloso y mayor representación bilateral de funciones específicas. Los componentes sensoriales y cognitivos del potencial evocado auditivo (PEA) cortical han demostrado sensibilidad a la intensidad de estimulación y asimetría en sujetos masculinos. En este estudio se investiga la influencia de sexo, ma-nualidad e intensidad de la estimulación sobre los componentes corticales del PEA. 56 sujetos voluntarios fueron sometidos a estimulación auditiva (series de 100 clicks binaurales, duración de 1 mseg, frecuencia de 1/seg, a cuatro diferentes intensidades) instruyéndoseles a prestar atención pasiva. Se registró derivaciones Cz, C3 y C4 referidas a mastoides bilateral y electrooculograma derecho, el tiempo de análisis fue de 400 mseg, los promedios fueron obtenidos sobre 100 clicks. La intensidad de la estimulación y el sexo afectan los componentes sesoriales corticales P1N1 y N1P2 en la derivación central. La asimetria sólo es influenciada por la manualidad; los Ss diestros exhiben mayores amplitudes P1N1 en el hemisferio derecho.

Tuberous Sclerosis Complex (TSC) is a multi-system disorder that is highly variable in its clinical presentation. Current molecular diagnostic methods permit identification of mutations in either TSC1 or TSC2 in 75–85% of TSC patients. Here we examine the clinical characteristics of those TSC patients who have no mutation identified (NMI). A retrospective review of our patient population that had comprehensive testing for mutations in TSC1/TSC2 identified 23/157 (15 %) that were NMI. NMI patients had a lower incidence of brain findings on imaging studies, neurological features, and renal findings than those with TSC2 mutations. In contrast, NMI patients had a lower incidence of seizures than TSC patients with TSC1 mutations, but had a higher incidence of both renal angiomyolipomas and pulmonary lymphangioleiomyomatosis. This distinct constellation of findings suggest that NMI patients may have a unique molecular pathogenesis, different from that seen in TSC patients with the usual mutations in TSC1 and TSC2. We suggest that the mechanisms of disease in these patients include both mosaicism for a TSC2 mutation, and unusual non-coding region mutations in TSC2.

In congenital perisylvian syndrome, there is polymicrogyric cortex distributed in variable extensions around the sylvian fissure. Unilateral cases usually present with congenital hemiparesis, while bilateral cases have pseudobulbar paralysis of the oropharingoglossal region. Both unilateral and bilateral cases have a high rate of epilepsy. Polymicrogyric cortex is characterized by too many small convolutions. Often there are no intervening sulci, and almost no white matter can be seen under them. On MRI they appear to have increased thickness. Bilateral and symmetric polimycrogiria can be hard to recognize on standard MRIs. Accurate and automated methods for measuring the thickness of cerebral cortex are available. They have mainly been used to study a variety of disorders with diminished cortical thickness. We studied a case of right perisylvian polymicrogyria, who presented in adult life with epilepsy and had a normal neurological exam. Fischl and Dale's automated cortical thickness analysis rendered a very clear picture of increased cortical thickness with values up to 9 mm in the affected areas (normal cortical thickness varies between 1 and 4.5 mm). The thickest areas were seen over grossly abnormal gyri on the reconstructed cerebral cortex. On MEG he presented a prominent and monotonous 9 Hz activity that was located within the limits of a thick gyrus. There was a significant difference of thickness between homologous hemispheric areas. To our surprise some areas of the left hemisphere also appeared to have increased thickness, raising the question of a bilateral asymmetric case.

Visual imagery is used in a wide range of mental activities, ranging from memory to reasoning, and also plays a role in perception proper. The contribution of early visual cortex, specifically Area 17, to visual mental imagery was examined by the use of two convergent techniques. In one, subjects closed their eyes during positron emission tomography (PET) while they visualized and compared properties (for example, relative length) of sets of stripes. The results showed that when people perform this task, Area 17 is activated. In the other, repetitive transcranial magnetic stimulation (rTMS) was applied to medial occipital cortex before presentation of the same task. Performance was impaired after rTMS compared with a sham control condition; similar results were obtained when the subjects performed the task by actually looking at the stimuli. In sum, the PET results showed that when patterns of stripes are visualized, Area 17 is activated, and the rTMS results showed that such activation underlies information processing.

The goal of this paper was to study the usefulness of standardised tests in elderly and assess the relationship among sociodemographic factors, neuropsychological indicators and psychiatric diagnosis, 40 subjects (31 women and 9 men, mean age 71.5 years, sd 6.16) who attended a primary care clinic in Santiago were interviewed using a sociodemographic interview, the Sarason's social support questionnaire, the mini mental state examination, the Goldberg health questionnaire, the Beck's inventory for measuring depression, the Wechsler adult intelligence scale and the Wechsler memory scale. Event related potentials were evoked in response to an auditory "Odd Ball" paradigm. A psychiatric interview, according ICD10 criteria gave 67.5% of psychiatric disorders. Anova analysis considering the psychiatric diagnostic groups showed significant differences for MMSE, GHQ12, BI, and WAIS. It is concluded that there is a high prevalence of psychiatric disorders and that standardised tests could be used for their detection.

La teoría de la personalidad de Eysenck postula 3 dimensiones ortogonales de personalidad: extraversión (E), neuroticismo (N), psicoticismo (P). Formula predicciones conductuales y fisiológicas relacionándolas a la predisposición a ciertos trastornos. La base biológica de las dimensiones E y N se ha evidenciado en diferencias electrofisiológicas. La dimensión P, agregada posteriormente, ha sido más controvertida, postulándose que no es independiente de las dimensiones antes descritas. Con objeto de estudiar las dimensiones de Eysenck, en particular P, en relación a la reactividad sensorial y a procesos de codificación se registró potencial evocado visual (PEV) por flash a 3 diferentes intensidades y P300 auditiva en 20 voluntarios sanos (x 28,5; ds 9,4 años). Se midió amplitud peak to peak y latencia de los componentes III, IV-V-VI y VII de PEV y de P300 mediante programa cursor. Hubo correlación positiva entre dimensiones N y P (spearman, r=0,52), entre P y amplitud del PEV (r=0,58) a intensidad alta y negativa entre E y latencia de P300 (r=-0,58). Según nuestros hallazgos, la dimensión P no es independiente y demostró relación con la reactividad sensorial. La dimensión E se relacionó a la velocidad de codificación, apoyando las aseveraciones de Eysenck sobre memoria y aprendizaje. Eysenck's personality theory postulates 3 orthogonal dimensions of personality: extraversión (E), neuroticism (N) and psychoticism (P), predicting conductual and physiological predispositions to suffer mental illness. Biological bases of Eysenck's personality traits have been documented electrophysiologically. Psychoticism, the latest described dimension, is controverted, since there is some evidence of common factors with the other two. In order to assess the relation between Eysenck's dimensions and sensorial reactivity and information encoding processes we studied 20 healthy young subjects (x age 28.5 years) with flash visual cortical evoked potentials (VEP, 3 intensities, peak to peak amplitude of III, IV-V-VI, VII components), and auditory cognitive evoked potentials (odd ball paradigm, P300 latency). There was a positive correlation between N and P dimensions (Spearman, r=0.52), between N and VEP amplitude at high intensity (r=0.58) and a negative correlation between E and P300 latency (r=-0.58). In short we found that P is not an independent dimension, but is related to sensorial reactivity. E dimension was related to encoding processes supporting Eysenck's aseverations about memory and learning differences.

Eysenck's personality theory postulates 3 orthogonal dimensions of personality: extraversion (E), neuroticism (N) and psychoticism (P), predicting conductual and physiological predispositions to suffer mental illness. Biological bases of Eysenck's personality traits have been documented electrophysiologically. Psychoticism, the latest described dimension, is controverted, since there is some evidence of common factors with the other two. In order to assess the relation between Eysenck's dimensions and sensorial reactivity and information encoding processes we studied 20 healthy young subjects (mean age 28.5 years) with flash visual cortical evoked potentials (VEP, 3 intensities, peak to peak amplitude of III, IV-V-VI, VII components), and auditory cognitive evoked potentials (odd ball paradigm, P300 latency). There was a positive correlation between N and P dimensions (Spearman, r = 0.52), between N and VEP amplitude at high intensity (r = 0.58) and a negative correlation between E and P300 latency (r = 0.58). In short we found that P is not an independent dimension, but is related to sensorial reactivity. E dimension was related to encoding processes supporting Eysenck's observations about memory and learning differences.

Vigabatrin (VGB) is an effective treatment of infantile spasms (IS) that controls spasms of all etiologies in about 50% of patients when used asmonotherapy. In tuberous sclerosis complex, VGB controls spasms in up to 95% of patients and should be used as the drug of choice. HigherVGB doses correlate with shorter times to response and higher response rates. Its most serious side effect is retinal toxicity characterized byirreversible bilateral concentric constriction of the visual fields (BCCVF). Maximum VGB dose, total VGB dose, and duration of VGB treatmentconstitute risk factors for BCCVF. In each particular patient, dose and duration of treatment should be kept at a minimum, while ensuringeffectiveness and preventing relapse. Every effort should be made to evaluate retinal function, even though it may require specializedophthalmological services. The addition of this new US Food and Drug Administration (FDA)-approved drug as an alternative in the treatmentof IS represents a major contribution to an armamentarium that contains only one other treatment.

Sex and handedness influence upon EPQ-R scores of 27 right-handers (13 men, 14 women) and 25 left-handers (13 men, 12 women) was non-significant as tested through ANOVA.

Augmenting/reducing (A/R) of visual evoked potentials (VEP) has been repeatedly observed in central derivations, with some subjects increasing, and others decreasing, VEP amplitude with increasing intensity of stimulation. Central derivations also exhibit hemispheric lateralization regarding A/R. This paper explores central and occipital VEP in the same population of 16 healthy, right-handed male subjects, stimulated with binocular light flashes 10 usec in duration delivered by a Grass PS2 stimulator at a rate of 1/sec and at the intensities of 0.36, 0.72 and 1.44 joules at source. Amplitudes and latencies of components P1, N1 and P2 agreed with those reported in the literature. On the basis of amplitude/intensity slope functions of «peak to peak» amplitudes (P1N1 and NIP2) at Cz, 10 augmenters (slope greater than 0) and 6 reducers (slope smaller than 0) were found. Compared to central leads (C3 and C4) occipital ones (O1 and 02) did not exhibit significant interhemispheric differences. Vertex augmenters for N1P2 were occipital reducers and vice-versa. The different characteristics of A/R at occipital and central leads are interpreted in terms of stages of visual information processing in primary and association areas and functional significance of VEP components.

Correlations between Vando's RAS scores, EPQ-R variables and amplitude/intensity slopes of vertex auditory evoked potentials in a Chilean sample of 17 male and 17 female young normal volunteers are presented. RAS was positively associated with Extraversion and Psychoticism and negatively with Lie. Electrocortical measures (P 1 -N 1 and N 1 -P 2 slopes) behaved differently in males and females. No association with age was found for psychometric variables. Findings suggest a modulating influence of sex. The pattern of relationships between personality variables studied seems to be generalizable across cultures and suggests a compensatory relation between overt behaviour and central nervous system properties.

Augmenting/reducing (A/R) of visual evoked potentials (VEP) has been repeatedly observed in central derivations, with some subjects increasing, and others decreasing, VEP amplitude with increasing intensity of stimulation. Central derivations also exhibit hemispheric lateralization regarding A/R. This paper explores central and occipital VEP in the same population of 16 healthy, right-handed male subjects, stimulated with binocular light flashes 10 usec in duration delivered by à Grass PS2 stimulator at a rate of 1/sec and at the intensities of 0.36, 0.72 and 1.44 joules at source. Amplitudes and latencies of components P1, N1 and P2 agreed with those reported in the literature. On the basis of amplitude/intensity slope functions of "peak to peak" amplitudes (P1N1 and NIP2) at Cz, 10 augmenters (slope greater than 0) and 6 reducers (slope smaller than 0) were found. Compared to central leads (C3 and C4) occipital ones (O1 and O2) did not exhibit significant interhemispheric differences. Vertex augmenters for N1P2 were occipital reducers and vice-versa. The different characteristics of A/R at occipital and central leads are interpreted in terms of stages of visual information processing in primary and association areas and functional significance of VEP components.

The concept of augmenting/reducing (A/R) postulates a central mechanism controlling the intensity of incoming signals and affecting the response to them. Using a kinesthetic figural aftereffect (KFA) task, Petrie (1967) classified as augmenters (Aug) subjects who judged a standard stimulus as larger after kinesthetic stimulation, and reducers (Red) as showing the opposite tendency. The stimulus intensity control mechanism could also be inferred from amplitude changes in evoked potential (EP) components elicited by stimuli of different intensities. The amplitude/intensity slope function of the vertex visual evoked potential (VEP) PIN, component became a standard measure of A/R (Buchsbaum et al. 1983). Although correlations between KFA scores and VEP slope have been reported, the crossmodal stability of A/R has been questioned (Kaskey et al. 1980; Raine et al. 1981). Absence of auditory reducing at the vertex and no correlations between visual and auditory P,N, or