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The aim of this study is to investigate the changes of the pupil's light reflex (PLR) and mobility in Parkinson's disease (PD) patients with and without cognitive disorder. Twenty two (22) patients (ten males, twelve females, mean age: 72.7+/-7.3 years) with identified PD entered the study. The patients were examined with the Mini Mental State Examination (MMSE), the Wechsler II Memory Scale (WMS II) and the Hamilton Depression Scale (HAM-D17). Eleven (11) patients (five males, six females, mean age: 72.09+/-7.06 years) were free of any cognitive deficits and eleven (11) patients (five males, six females, mean age: 73.36+/-7.55 years) had cognitive disorder according to the aforementioned scales. None of the patients satisfied the DSM-IV-TR criteria for depression or anxiety disorder. The patients underwent a pupillometric study in both eyes with single flash stimuli of 24.6 candelas/m(2) intensity and 20 ms duration. The pupillometric parameters that were studied were: Latency for the onset of Constriction (T1), Baseline Pupil Radius (R1), Minimum Pupil Radius after the pupil reaction to light (R2), Amplitude (AMP, R1-R2), Time for maximum Miosis (T2), Maximum Constriction Velocity (VCmax) and Maximum Constriction Acceleration (ACmax). The pupillometric findings of each group were compared to those of an age and sex matched group of eleven healthy subjects. Furthermore, a comparison between the findings of the two groups was conducted. ACmax and VCmax were significantly lower in patients without (PD) and with coexisting cognitive impairment (PDC) compared to normal subjects (NC) (p

This article attempts to describe a very unusual case of a boy aged 15, who has had intractable epileptic phenomena, mental retardation, megalocephaly, micrognathy, syndactyly, small tongue, hypoplastic genitalia, gynecomasty, obesity, and slight left body hemiatrophy. Neurologically the patient has had hypotonia of the lower limbs, cerebellar dysfunction including horizontal nystagmus, bilateral intention tremor, dysdiadokokinesia, gait ataxia. The clinical investigation revealed low plasma cholesterol and hypoplasia of the vermis in MRI. The epileptic phenomena were intractable and polymorphous. One should have thought that this is an unusual case of Smith-Lemli-Opitz syndrome associated with features of Joubert syndrome.

Background Antiepileptic drugs (AEDs) are mainly used to control cortical hyperexcitability. Some of them (e.g. phenytoin (PHT) and topiramate (TPM)) have also effects on the peripheral nervous system (PNS). Lacosamide (LCM) is a novel AED that stabilizes hyperexcitable neuronal membranes by selectively enhancing the slow inactivation of voltage-gated sodium channels (VGSCs). Although the mechanism of action of LCM is fairly well understood, there are no in vitro data available regarding any possible PNS effects of LCM. Objective To investigate, in vitro, the effects of LCM on peripheral nerve excitability in comparison with PHT and TPM, two AEDs that act, in part, by stabilizing the fast inactivation state of VGSCs. Methods Experiments were conducted on the isolated sciatic nerve of the adult rat using standard electrophysiological methods. The effects of LCM on the amplitude and latency of the evoked compound action potential (CAP) during a 48 h period of drug exposure were recorded and compared with the effects of PHT and TPM. Results LCM produced inhibitory effects on CAP at concentrations significantly higher than the therapeutic levels (>25 μg/ml). At these concentrations (62.57–125.15 μg/ml), an acute and immediate increment of the latency and decrement of the amplitude of the CAP were observed. In contrast to LCM, PHT caused an acute decrement in the amplitude as well as an increment in the latency of the CAP even at subtherapeutic levels (5 μg/ml). With regard to TPM, the amplitude of the CAP was not affected at the supratherapeutic concentrations but at the therapeutic concentration of 33.94 μg/ml a reduced decrement of the CAP amplitude compared to the controls was observed. Conclusions LCM, PHT and TPM exert differential effects on peripheral nerve excitability. PHT inhibited the sciatic nerve CAP even at subtherapeutic levels whereas LCM was safe within the therapeutic concentration range. TPM did not affect the CAP amplitude even at high supratherapeutic concentrations whereas in the therapeutic range a neuroprotective effect was observed. Possible underlying mechanisms and the clinical implications of these findings are discussed.

Summary An open, prospective, observational study was performed to assess efficacy and adverse-event profile of topiramate as add-on therapy in epilepsy. Outpatient neurology clinics from 11 general hospitals in Greece participated in the study. In total, 211 patients with treatment resistant partial-onset seizures who met the inclusion criteria, were studied. After baseline evaluation, topiramate was given at a target dose of 200mg/day over a 1-month titration period. In the subsequent maintenance period, the topiramate dose could be varied according to the clinical results. Patients were followed for in total 6 months, with monthly visits and regular physical, neurological and laboratory examinations. Seizure frequencies decreased to 35–40% of baseline values following 3 months of treatment and remained relatively constant thereafter. The average monthly seizure frequency over the 6-month study period was 4.61, compared to 9.21 at baseline. The number of responders (patients with at least 50% reduction in seizure frequency) followed a similar pattern, i.e., increase during the first 3 months levelling off at a final 80–85% response rate. Of those completing the study, 30% had been seizure-free for at least 3 months and 12% for 5 months. Topiramate was well tolerated, no deviations in laboratory values were found. Adverse events appeared to occur less frequently, and antiepileptic effects were more pronounced in this prospective open-label study than in earlier reports from randomised controlled trials. The nature of the patient population and the application of individualised dose optimisation are proposed as contributing factors to explain the favourable results of this study.

Cognitive event-related potentials (ERPs) are widely employed in the study of dementive disorders. The morphology of averaged response is known to be under the influence of neurodegenerative processes and exploited for diagnostic purposes. This work is built over the idea that there is additional information in the dynamics of single-trial responses. We introduce a novel way to detect mild cognitive impairment (MCI) from the recordings of auditory ERP responses. Using single trial responses from a cohort of 25 amnestic MCI patients and a group of age-matched controls, we suggest a descriptor capable of encapsulating single-trial (ST) response dynamics for the benefit of early diagnosis. A customized vector quantization (VQ) scheme is first employed to summarize the overall set of ST-responses by means of a small-sized codebook of brain waves that is semantically organized. Each ST-response is then treated as a trajectory that can be encoded as a sequence of code vectors. A subject's set of responses is consequently represented as a histogram of activated code vectors. Discriminating MCI patients from healthy controls is based on the deduced response profiles and carried out by means of a standard machine learning procedure. The novel response representation was found to improve significantly MCI detection with respect to the standard alternative representation obtained via ensemble averaging (13% in terms of sensitivity and 6% in terms of specificity). Hence, the role of cognitive ERPs as biomarker for MCI can be enhanced by adopting the delicate description of our VQ scheme.

The purpose of this study was the evaluation of pupil light reflex (PLR) in patients with Parkinson's disease (PD) by using a modern pupillometry system and the investigation of its potential relationship with dopamine transporter imaging (DaTSCAN), which is an objective method for the evaluation of presynaptic dopaminergic system. PLR was evaluated using pupillometry in 35 patients with PD without clinical evidence of autonomic dysfunction and 44 healthy matched controls. PLR was elicited using a fully automated pupillometry system and six parameters were measured. Dopamine transporter imaging was performed using radioactive ioflupane (123)I-FP-CIT [(123)I-N-ω-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)-nortropane]. A significant increase in latency and a significant decrease in amplitude, maximum constriction velocity, as well as maximum acceleration were observed in PD patients. There was no significant difference in initial radius and minimum radius values. Investigating the relationship between pupillometry parameters and (123)I-FP-CIT binding values, we correlated values from the semiquantitative analysis of radioligand uptake with pupillometry parameters, but we found no significant correlation. This study demonstrates PLR impairment in patients with PD without overt autonomic dysfunction. This impairment does not seem to correspond to the reduction of radioligand binding in the striatum as the result of presynaptic dopaminergic dysfunction, suggesting a different deterioration rate of these systems.

We evaluated pupil light reflex (PLR) in patients with Parkinson's disease (PD) and normal controls by means of pupillometry and explored its possible relation to clinical characteristics in parkinsonian patients. PLR was evaluated using pupillometry in 66 patients with PD without clinical evidence of autonomic dysfunction and 44 healthy matched controls. PLR was elicited by single flash stimuli of 24.6 candelas/m(2) intensity and 20 ms duration, and six parameters were studied after full recording of pupil's movement. A significant increase in latency (T1) and significant decrease in amplitude (R1-R2), maximum constriction velocity (V(max)), as well as maximum acceleration (AC(max)) was found in parkinsonian patients. There was no significant difference in initial radius (R1) and minimum radius (R2) values. Of the parameters studied, AC(max) emerged as a significant predictor for discrimination between PD patients and controls. There was no significant correlation between pupillometry parameters and clinical characteristic of patients (disease duration, stage, and the Unified Parkinson's Disease Rating motor scale). The study demonstrates PLR disorder in PD patients even without overt clinical autonomic dysfunction. Pupillometry appears to be a useful and noninvasive method for exploration of PLR alterations in PD and may prove to be useful for the early detection of subclinical autonomic nervous system dysfunction.

Many studies investigated the brain responses as a reaction in auditory or visual stimuli separately. However a few studies have been published so far investigating the interactions of the two aforementioned stimuli. The current study comes to examine the impact of the audio-visual stimulation with binaural beats and flickering light in four different colors on low and upper alpha oscillations. For this purpose electroencephalogram (EEG) was adopted and Event Related Desynchronization/Event Related Synchronization (ERD/ERS) has been used as an index in order to investigate the alpha brain responses. Statistically significant results suggest that the combination of audio-visual stimuli with binaural beats and flickering light color at 8 and 10 Hz respectively can evoke significant Following Frequency Response (FFR) of the low and upper alpha oscillations.

Purpose of this study was to examine pupil size changes and mobility in normal subjects and in heart failure (HF) patients.Sixteen stable patients with New York Heart Association (NYHA) class II or III heart failure and sixteen control subjects were studied. Pupillary reaction to light was recorded and nine parameters from this data were measured, reported and then compared in both groups of subjects.Patients with HF had abnormal pupillary function compared with normal subjects. Pupillary light reflex variables differed significantly between two groups (p0.05) except baseline radius (R1), minimum radius (R2) and time for maximum constriction (T3). A significant decrease in maximum constriction velocity (VCmax; p0.001) and maximum constriction acceleration (ACmax; p0.001) was observed in HF subjects. Furthermore, significantly higher values in percentage recovery-redilatation (%R; p0.001), percentage R2/R1 (%R2/R1; p0.05), latency (T1; p0.05) and time for maximum velocity (T2; p0.05) were found in the same group.Of the parameters studied, R1 and %R are governed mainly by the action of the sympathetic nervous system, through norepinephrine. The rest are governed mainly by parasympathetic nervous system, through acetylcholine. The results of our study demonstrate generalized adrenergic activation and parasympathetic withdrawal, which are present in HF.

Integrating emotional feedback to educational systems has become one of the main concerns of the affective learning research community. This paper provides evidence that Embodied Conversational Agents (ECAs) could be effectively used as emotional feedback to improve brainwave activity towards learning. Further research, integrating ECAs into tutoring systems is essential to confirm these results.

The aim of the study was to evaluate the cholinergic deficiency in Alzheimer's (AD) and Parkinson's disease (PD). For this purpose, pupil size changes and mobility were assessed using a fast-video pupillometer (263 frames/s). Twenty-three (23) patients with probable AD and twenty-two (22) patients with PD (eleven with cognitive impairment and eleven without) entered the study. A full record of the pupil's reaction to light was registered. From this data ten (10) parameters were measured and reported. Comparison of those parameters in both group of subjects followed. Patients with probable AD had abnormal pupillary function compared to healthy ageing. All the Pupil Light Reflex (PLR) variables significantly differed between the two groups (p0.005) except the Baseline Pupil Diameter after 2-min dark adaptation (D1) and the Minimum Pupil Diameter (D2). Maximum Constriction Acceleration (ACmax) was the best predictor in classifying a subject as normal or as an AD with a perfect classification ability (AUC=1, p0.001). ACmax and Maximum Constriction Velocity (VCmax) were significantly lower in PD patients without and with coexisting cognitive impairment compared to normal subjects (p0.001). Patients with cognitive impairment had significantly lower levels of ACmax, VCmax and amplitude (AMP=D1-D2) than patients with no cognitive deficits. ACmax and secondarily VCmax were the best predictors in classifying a subject as normal or as a PD patient with or without cognitive impairment. Cognitive and memory impairment, which reflects a cholinergic deficit, may be a crucial pathogenetic factor for the decrease in the aforementioned pupillometric parameters. VCmax and ACmax can be considered as the most sensitive indicators of this cholinergic deficiency.

A group of subjects in whom the clinical history fulfilledthe diagnostic criteria for REM behavior disorder (RBD) ofthe criteria of American Sleep Disorders Association andSleep Research Society (1997)described here were identi-fied in our sleep unit between March 2000, and June2007.18 patients, (12) men and (6) women with a mean age of70.5 ± 11 years. They all had a history of dream-enactingbehaviors with a mean duration of 5.5 ± 9.5years. Behav-iors included talking (100%), shouting (78%), punching(74%), gesturing (63%) and kicking (65.3%). 15 subjectsfell out of bed and 11 suffered lacerations in the face.The frequency of episodes is variable, occurring once every2 weeks to 4 times nightly for consecutive nights, andoften increases over time.Patients were interviewed regarding their sleep habits andunderwent full PSG. The clinical evaluation of the patientsconsisted of a neurological examination, an interviewwith him/her,and an interview with his wife/husband.They also underwent a full laboratory and biochemicalevaluation like a complete blood count,test of thyreoidfunction e.t.c. As well as an E.E.G, a brain MRI and psy-chometrics tests (B.D.I, M.P.P.I).

The aim was to study the influence of patients’ age, frequency and type of seizures, disease duration, number of AEDs and use of benzodiazepines on the quality of life of patients with epilepsy. We consecutively identified 223 patients with epilepsy who attended the epilepsy outpatient clinics at three university hospitals in Greece. Quality of life was evaluated by QOLIE-31. One-way analysis was used to assess the association of the studied factors and QOLIE-31 subscales. After checking for co-linearity, we performed multivariate stepwise linear regression analysis with all the variables that on univariate analysis showed a statistically significant effect on each subscale of QOLIE-31 to explore which of the studied factors affect independently on QOLIE-31 scores. Of a total of 223 patients, 118 (52.95%) were men; mean age was 35.18 ± 13.22; mean duration of the disease was 13 years; 58 (26%) patients had generalized seizures, 93 (41.7%) had PS, and 72 (32.3%) of the patients had PsG; 85 (38.1%) were on polytherapy and 38 (17%) were taking benzodiazepines. Independent factors affecting QoL were: seizure frequency (Overall QoL P = 0.0001, Seizure worry and Emotional well-being P

This study is presenting the translation and cultural adaptation into Greek of the Quality of Life in Epilepsy Inventory (QOLIE-31). We adapted the QOLIE-31 to Greek through a procedure of translation-back-translation. Sixty-three patients were interviewed and completed the QOLIE-31 and the GHQ questionnaires. We re-examined a subset of them after a period of 2-5 weeks to evaluate the test-retest reliability of the questionnaire. We assessed the convergent validity by comparison of the QOLIE-31 and the GHQ and QOLIE-31 subscales and external measures. Discriminative validity was evaluated using the method of known-groups comparisons. The internal consistency was high for the QOLIE-31 and its' subscales (Cronbach's alpha 0.92 and 0.59-0.83 respectively). Test-retest reliability was acceptable (intra-class correlation coefficient 0.49-0.89 and Pearson's coefficient 0.53-0.92) for the group of patients who were re-examined. Comparison of the QOLIE-31 and GHQ scores showed agreement between the two questionnaires (Pearson's coefficient -0.61). We demonstrated the discriminative validity by the difference in the QOLIE-31 scores between patients with different seizure frequencies and different employment status. We concluded that the Greek version of the QOLIE-31 has psychometric properties equivalent to those of the original American-English version and is a valid and reliable instrument.

SummaryThe frequency of sleep disturbances in patients with epilepsy and their impact on quality of life (QoL) have been documented in a few reports, and the results are conflicting. We identified 124 consecutive epilepsy out-patients who visited the epilepsy out-patient clinics at the University Hospital of Alexandroupolis, the AHEPA Hospital in Thessaloniki and the Aeginitio Hospital in Athens. We measured excessive daytime sleepiness (EDS) with the Epworth Sleepiness Scale (ESS), obstructive sleep apnea (OSA) with the Sleep Apnea scale of the Sleep Disorders Questionnaire (SA-SDQ), and insomnia with the Athens Insomnia Scale (AIS). We evaluated quality of life by the Quality of Life in Epilepsy Inventory (QOLIE-31). EDS was found in 16.9% (21/124) of epileptic patients, OSA in 28.2% (35/124), and insomnia in 24.6% (30/122). In multivariate analysis, we found that insomnia was an independent negative factor for Total score (p