Multimodal Evoked Potentials Research Articles

Multimodal evoked potentials are useful for the diagnosis of pediatric acute disseminated encephalomyelitis.

The application of evoked potentials (EPs) to the diagnosis of acute disseminated encephalomyelitis (ADEM ) has not been investigated in detail. The aim of this study, therefore, was to analyze the value of multimodal EPs in the early diagnosis of pediatric ADEM. This was a retrospective study in which we enrolled pediatric ADEM patients and controls (Cs) from neurology units between 2017 and 2021. We measured indices in patients using brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs) and somatosensory evoked potentials (SEPs), and then we analyzed their early diagnostic value in ADEM patients. The mean age of the ADEM group was 6.15 ± 3.28 years (range,1-12 years) and the male/female ratio was 2.1:1 The mean age of the Cs was 5.97 ± 3.40 years (range,1-12 years) and the male/female ratio was 1.3:1. As we used magnetic resonance imaging (MRI) as the diagnostic criterion, the sensitivity, specificity, and accuracy (κ was 0.88) of multimodal EPs were highly consistent with those of MRI; and the validity could be ranked in the following order with respect to the diagnosis of ADEM: multimodal Eps > single SEP > single VEP > single BAEP. Of 34 patients with ADEM, abnormalities in multimodal EPs were 94.12%, while abnormalities in single VEPs, BAEPs and SEPs were 70.59%,64.71%and 85.3%, respectively. We noted significant differences between single VEP/BAEPs and multimodal EPs (χ2 = 6.476/8.995,P = 0.011/0.003). The combined application of multimodal EPs was superior to BAEPs, VEPs, or SEPs alone in detecting the existence of central nerve demyelination, and we hypothesize that these modalities will be applicable in the early diagnosis of ADEM.

Multimodal Evoked Potentials as Potential Biomarkers of Disease Activity in Patients With Clinically Isolated Syndrome.

ObjectiveThere is an ongoing search for markers useful in monitoring and predicting disease activity at the early stage of multiple sclerosis (MS). The goals of this study were to prospectively evaluate the changes in parameters of multimodal evoked potentials (EP) and cognition within a 3-year follow-up period in patients with clinically isolated syndrome (CIS), and to assess the prognostic value of baseline findings with regard to the disease outcomes.MethodsIn 29 patients (20 women, nine men, mean age 31.1) multimodal (visual, brainstem auditory, somatosensory, event-related) EP and neuropsychological tests (NT) were performed at baseline (T0) and after 1 (T1) and 3 (T3) years. Their results were compared longitudinally between baseline, T1, and T3. Baseline results confirmed conversion of CIS into multiple sclerosis (MS) and disability level at T1 and T3 using multiple comparisons and a logistic regression model.ResultsApart from mean N13/P16 SEP (somatosensory evoked potentials) amplitude (lower at T1 and T3 than at baseline (T0 1.02 ± 0.37 μV, T1 0.90 ± 0.26 μV, T3 0.74 ± 0.32 μV, p < 0.05 for both comparisons), no significant changes of EP or NT parameters were found in longitudinal assessment. Baseline P300 Pz latency was longer for the patients with MS than for those with CIS at T1 (352.69 vs. 325.56 ms). No predictive value was shown for any of the analyzed baseline variables with regard to conversion from CIS into MS.SignificanceBaseline ERP abnormalities were associated with their short-term conversion into MS. ERP are worth considering in multimodal EP evaluation at the early stage of MS.

Multimodal evoked potentials influence multiple sclerosis phenotype and disability accrual

Previous studies have suggested that Evoked Potentials (EPs) correlate with Multiple Sclerosis (MS) phenotype and disability rate. The goal of this study is to investigate whether multimodal EPs have distinct features between primary progressive (PPMS) and relapsing MS (RRMS) and are predictors of disability accrual.

Cuba’s contribution in the diagnosis of brain death/death by neurologic criteria

I read with interest the paper by Lewis et al. to identify the countries in the Latin America/Caribbean Group of the United Nations (GRULAC) that have protocols for brain death/death by neurologic criteria (BD/DNC). Curiously, the authors don’t mention Cuba, which has been one of the most active countries in the area, since the early ’90s. The first kidney transplant in Cuba was performed on 24 February 1970, using a cadaveric donor. In September 1992, the First Symposium on Brain Death was held in Havana, with the attendance of the main and world-known authors at that time, like Cristopher Pallis, Earl Walker, among others. These conferences uninterruptedly continued over the years, and the last Symposium was held in December 2018. In the First Symposium, the Cuban Commission for Death Determination presented for the first time the Guidelines for the Determination of BD/DNC in Cuba. Since that time, Machado proposed a concept of death, based on the basic pathophysiological mechanism of consciousness generation. This author also proposed as ancillary tests in BD/DNC diagnosis the use of a test battery composed of multimodality evoked potentials (MEP) and electroretinography (ERG). Later, a Cuban Law for the determination of death was issued. The Cuban Commission The Commission stated that there is only one kind of death, based on the irreversible loss of brain functions based on the whole brain criteria. Furthermore, the Cuban law did not even mention the term ‘transplants’. It is clear the human beings die regardless bodies would be useful or not for transplantation.

Multi-modal (visual, acoustic, cognitive) evoked potentials in children with cerebral palsy

The objective. To assess and compare the multi-modal evoked potentials (EP) (flash visual [fVEP], brainstem acoustic [BAEP] and cognitive evoked responses [CER]) parameters in children with cerebral palsy (CP) and healthy ones.Materials and methods. There are 100 children aged 3–17 years were examined. Experimental group included 55 children with CP, diagnosed according to International cerebral palsy criteria (P. Rosenbaum et al., 2006), and mild motor deficit (levels I–II of Gross Motor Function Classification Scale), undergoing rehabilitation course. Control group was presented by 45 healthy children aged 3–17 years with no neurological signs. Registration of the fVEP, BAEP and CER and their comparative analysis with non-parametric tests was performed. Neurophysiological recording performed on EMG-VP4 system (MBN Scientific Company, Moscow, Russia).Results. Slowing of maturation and significant delaying of main EP components were observed in experimental group.Conclusion. Findings in this investigation indicate the retardation of cortical sensory processing in children with CP. Analyzing multi-modal EP may be helpful in early detecting sensory abnormalities as complications of CP.

Multimodal Evoked Potentials as Candidate Prognostic and Response Biomarkers in Clinical Trials of Multiple Sclerosis.

Evoked potentials (EPs) measure quantitatively and objectively the alterations of central signal propagation in multiple sclerosis and have long been used for diagnosis. More recently, their utility for prognosis has been demonstrated in several studies, summarizing multiple EP modalities in a single score. In particular, visual, somatosensory, and motor EPs are useful because of their sensitivity to pathology in the frequently affected optic nerve, somatosensory tract, and pyramidal system. Quantitative EP scores show higher sensitivity to change than clinical assessment and may be used to monitor disease progression. Visual EP and the visual system have served as a model to study remyelinating therapies in the setting of acute and chronic optic neuritis. This review presents rationale and evidence for using multimodal EP as prognostic and response biomarkers in clinical trials, targeting remyelination or halting disease progression in multiple sclerosis.

O101 Evoked potentials in multiple sclerosis – Still a useful tool?

In the early diagnosis of multiple sclerosis (MS) the evoked potentials (EP) have been in the recent decades displaced by magnetic resonance imaging, which is reflected in their marginal position in the current McDonald’s diagnostic criteria. However, the parameters of EP were demonstrated to correspond well with clinical symptoms of MS and the degree of disability. Thus they have been used as measures of the disease progression, in monitoring of the MS natural course as well as effectiveness of disease-modifying drugs. There is also some evidence of the prognostic value of EP, performed at the early stage of the disease, with regard to subsequent relapses and a long-term disability accumulation. The advanced techniques of stimulation and collective analysis of multimodal EP further increase their sensitivity and specificity in revealing the dysfunction of the central nervous system pathways. Overall, EP have remained useful electrophysiological biomarkers of the degree and dynamics of neurological deficit in the course of MS. The integration of EP with neuroimaging and clinical issues may also provide a better insight into pathological processes underlying the disease.

Early disturbances in multimodal evoked potentials as a prognostic factor for long-term disability in relapsing-remitting multiple sclerosis patients.

The aim of this study was to investigate whether early alterations in evoked potentials (EPs) have a prognostic value in relapsing-remitting multiple sclerosis (RRMS). We retrospectively selected 108 early MS patients with a neurological follow-up ranging from 5 to 15years, in whom multimodal EPs (visual, brainstem auditory, somatosensory and motor) were performed at diagnosis. A conventional ordinal score was used to quantify the observed abnormalities. The extent of change in the composite EP score was well correlated to the Expanded Disability Status Scale (EDSS) at ten years (Y10) and up to 15years (Y11-15) after disease onset. Analysis of the predictive value of the EP score showed an increased risk of disability progression at Y10 and Y11-15 of 60% (p<0.0001) and 73% (p<0.0001) respectively in patients with an EP score >4. Conversely, the risk of disability progression at Y10 and Y11-15 associated with a lower EP score (⩽4) was reduced to 16% and 20% respectively. Our data support the good predictive value for long-term disability progression of multimodal EPs performed early after disease onset in RRMS patients. This study, performed in a homogeneous RRMS cohort with long term follow-up, demonstrates the value of an early comprehensive neurophysiological assessment as a marker for future disability.

MRI AND EVOKED POTENTTIALS IN DIFFERENTIAL DIAGNOSIS OF ENCEPHALITIS, DISSEMINATED ENCEPHALOMYELITIS AND MULTIPLE SCLEROSIS IN CHILDREN

Last years are characterized by the growth of encephalitis (Ef) frequency and demyelination diseases of CNS in children, especially, among teenagers. The diseases are characterized by similar of clinical features of the disease onset, absence of differential diagnostical criteria and frequency of the disease chronisation and possibility of transformation in multiple sclerosis (MS), all this determines the problem of differential diagnosis. The aim is to determine differential-diagnostical features of Ef, disseminated encephalomyelitis (DEM) and MS in children by analysis of structure MRI and brain multi-modal evoked potentials (MEP). We observed 35 children with Ef, 40 with DEM and 40 with MS. All patients were from 3 to 17 years old. All patients were diagnosed clinically in acute period and in catamnesis — after 3 and 6 months. MS diagnosis was based on McDonald criteria, 2010 year. ADEM diagnosis was also based on international criteria. We analyzed brain MRI, spinal MRI and MEP in all patients during the first day of hospitalization and after 3 and 6 months later. The analysis of the MRI of the brain and spinal cord in encephalitis (EF, n=35), disseminated encephalomyelitis (ADEM, n=60) and multiple sclerosis (MS, n=60) of children on admission to hospital, and then — after 3 and 6 months. Up to 85% of foci with ADEM and EF had irregular shapes, accompanied by swelling phenomena, and mass effect and in 2⁄3 cases were localized in the gray or white and gray matter of the CNS. In 60% of cases in patients with EF and ADEM based on the localization of foci could suggest the etiology of the disease. In MS 67,5% were detected multifocal white matter lesions (more than 10 lesions), and most of the foci had a size of up to 1 cm and the correct round-oval and the absence of signs of inflammation. The presence of lesions in the gray matter at EF, ADEM and MS respectively observed in 82,5%, 40% and 7,5% of cases. After 3–6 months of MRI in the majority of EF and DEM foci regressed, and in MS were reduced in size, but not completely disappeared. According to the study of evoked potentials (EP) of the brain in children with MS and ADEM often increased latency of the responses, while the EF decreased cortical or brainstem response amplitude. The majority of patients with MS have changed the parameters both 3–4 different EP (somatosensory from the upper and lower limbs, acoustic stem and/or visual), while ADEM and EF-1–2 evoked potentials. Chronic EF, and the progression of neurological symptoms was not associated with the advent of the «new» lesion, and up to 75% of the foci can regress completely after 3–6 months. Chronic EF characterized by increase of only brain atrophy, which involves communication of these changes only with neuronal process. Whereas in MS and multiphase ADEM appeared «new» foci accumulating contrast agent that allows you to talk about preserving the pathogenesis of vascular component in their progression. Study of MEP and MRI in children with Ef, DEM and MS in dynamics can be useful for differential diagnosis and monitoring of therapy efficacy.

Evoked potential in multiple sclerosis: As important as 40 years ago but not for the same reasons

Until the beginning of the 1980s, evoked potentials (EPs) were considered a unique diagnostic tool for multiple sclerosis (MS), particularly because of their capacity to reveal the presence of clinically hidden dysfunction in a sensory system when MS was suspected owing to clinically evident dysfunction in another brain or spinal cord area. Since the advent of magnetic resonance imaging (MRI), multiple lesions can be detected much more easily and reliably, which led some clinicians to consider that EPs had lost their place in the workup of MS patients. This holds not true as EPs remain of utmost importance in two indications: – as a method to objectivize ill-specific symptoms: provided that the stimulus should be adapted to the precise sensory sub-modality in which a deficit has been suspected, EPs may be used to decide whether or not the patient should be orientated toward MRI, less accessible and more expensive; – as a follow-up tool: based on some observations over time periods exceeding 30 years, we know that EPs remain remarkably stable in the absence of lesion appearance or evolution; therefore, we frequently use them to reliably reassure patients fearing relapses, based on non-specific symptoms. Consequently, we consider it both medically and economically justified getting a baseline recording of multimodality-evoked potentials in any patient in whom MS has been diagnosed. While the classical stimulus modalities (pattern visual, brainstem auditory, electrical somatosensory and motor EPs) should be preferred, other less-classical modalities (laser, vestibular myogenic, blink reflex) may prove useful in some specific cases.

7. Neurophysiology in diagnosis and prognosis of multiple sclerosis in age of magnetic resonance imaging

Objective The advent of magnetic resonance imaging techniques has greatly reduced the diagnostic value of neurophysiological tests in MS, particularly evoked potentials. In disease progression, most of MS patients accumulate inflammatory lesions, axonal damage, and progressive brain atrophy, along with an increasing degree of disability. More promising is the utilization of neurophysiological tests to quantify the severity of white matter involvement. Methods Evoked potentials (EP) have been still used in multiple sclerosis (MS) especially in its early diagnosis. Recently, multimodal EPs appears to be a prognostic factor for disease progression ( Schlaeger et al., 2012 , Invernizzi et al., 2011 , Schlaeger et al., 2012 ). Deeper insights about causal and functional relationships in plasticity of the motor system in patients with MS were gained by neurophysiological techniques, predominantly by transcranial magnetic stimulation. Results Visual evoked potential (VEP) are preferentially used in early diagnosis of MS for detection of subclinical optic neuritis. Somatosensory (SEP) and motor (MEP) evoked potentials reveal subclinical lesions in the CNS and could be a supplementary diagnostic tests for sensory and motor system disturbances. MEP abnormalities correlate with the degree of motor impairment and disability. A number of functional imaging studies have assessed patterns of brain activation during simple motor tasks in MS patients and their relationship with CNS damage and motor function. Conclusion Evoked potentials are functional neurophysiological methods highly sensitive in revealing ,,silent lesions“ especially at the beginning of MS. Multimodal evoked potentials have indisputable benefit of prognostic value to determine non-responders, ,,benign“ course of the disease and identification of patients with significant disease progression. A battery of neurophysiological tests could be useful in monitoring the disease progression in individual patient and as surrogate endpoints in clinical trials. Supported by Research Project of Charles University PRVOUK P34, Czech Republic.

P06. Prognostic value of multimodal neurophysiological testing in spinal cord injury

The disability secondary to traumatic spinal cord injury (SCI) is known to be highly variable among patients due to the vast variety of lesion levels and varying severity, resulting in a mixture of central and peripheral neural lesion burden. In this context prognostication of outcome becomes a challenge, yet is paramount in planning rehabilitation and clinical trials. Next to clinical assessment by International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) neurophysiological techniques have been used for diagnosing lesion severity and prognostication as they are objective and early available. Evoked potentials (EPs) have been suggested as diagnostic and prognostic tools in SCI, although their importance and prognostic value has not been completely established. Prognostic significance of multimodal EPs was assessed in a cohort of 633 SCI cases from the European Multicenter Study about Spinal Cord Injury database (EMSCI, www.emsci.org ). SCI patients underwent complete neurophysiological evaluation (NPE) prospectively, including nerve conduction studies, somatosensory and motor EPs within 4 weeks after spinal injury. Functional recovery after SCI as the endpoint was assessed at 3, 6 and 12 months during and after rehabilitation by standardized and validated testing using the international Spinal Cord Independence Measure (SCIM). EPs abnormalities were quantified through an index of global EPs alteration (EP score). The relationship between EP score and disability in SCIM was analyzed by Spearman ρ correlation coefficient. Receiver operated characteristic (ROC) curves were used to determine best EP score cut off values to predict SCIM endpoints within sub-score categories such as mobility and hand function. Significant correlations could be shown between EP score (motor and somatosensory EPs) and SCIM score at the time of NPE and at 3, 6 and 12 months of follow-up. Goodness of fit increased over time indicating true predictive value of early EP assessment for late functional outcome. For each functional sub-score endpoint, significant sensitivity, specificity, positive and negative predictive values were obtained. Multimodal EPs are valuable objective procedures to predict disability in traumatic SCI patients. This prediction can be achieved independent of clinical assessment (ISNCSCI) and without knowledge of lesion level.

Significance of multiple neurophysiological measures in patients with chronic disorders of consciousness

ObjectiveThe aim of this study was to verify the value of multiple neurophysiological tests in classifying disorders of consciousness (DOCs) in patients in a chronic vegetative or minimal consciousness state categorised on the basis of the Coma Recovery Scale (CRS). MethodsThe study included 142 patients, all of whom underwent long (18h) EEG-polygraphic recordings including one night. The EEG was scored using the Synek scale and sleep patterns using an arbitrary scale. Absolute total power and relative EEG power were evaluated in different frequency bands. Multimodal evoked potentials (EPs), including auditory event-related potentials, were also evaluated and scored. ResultsThe most information came from the combined multimodal EPs and sleep EEG scores. A two-step cluster analysis based on the collected information allowed a satisfactory evaluation of DOC severity. Spectral EEG properties seemed to be significantly related to DOC classes and CRS scores, but did not seem to make any significant additional contribution to DOC classification. ConclusionsMultiple electrophysiological evaluations based on EEG, sleep polygraphic recordings and multimodal EPs are helpful in assessing DOC severity and residual functioning in patients with chronic DOCs. SignificanceSimple electrophysiological measures that can be easily applied at patients’ bedsides can significantly contribute to the recognition of DOC severity in chronic patients surviving a severe brain injury.

7. Evoked potentials in diagnosis and prognosis of multiple sclerosis

Objective Evoked potentials (EP) are functional neurophysiological methods that have been used in multiple sclerosis (MS) especially in its early diagnosis. Recently, multimodal EPs appears to be a prognostic factor for disease progression. Methods The review is based on a literature search in PubMed and the author’s long experience of neurophysiological diagnostics. Results Visual evoked potential (VEP) are preferentially used in early diagnosis of MS. They are highly sensitive to detect subclinical optic neuritis more than magnetic resonance. Somatosensory (SEP) and motor (MEP) evoked potentials can reveal subclinical lesions in the central nervous system and could be a supplementary diagnostic tests for sensory and motor system disturbances. MEP abnormalities correlate with the degree of motor impairment and disability assessed by EDSS. Abnormal multimodal EPs (”positive EP score”) at the first session are worse prognostic factor. Low and stable EP scores along with stable EDSS identify a ”benign” form of RS. The assessment of the disease progression and/or monitoring of the therapeutic effects have some limitations, e.g. abnormal response persists even in relative inactivity of the disease and it’s localization is non-specific. The sensitivity of EPs increases with the length of the tested neural pathways and with used EP modalities. Conclusion Evoked potentials are highly sensitive in revealing ”silent lesions” especially at the beginning of MS and/or when no obvious neurological symptoms occur. Multimodal evoked potentials have indisputable benefit of prognostic value to determine non-responders, ”benign” course of the disease and patients with significant disease progression. Supported by Research Project of Charles University PRVOUK P34, Czech Republic.

Prediction of MS disability by multimodal evoked potentials: Investigation during relapse or in the relapse-free interval?

ObjectiveLittle is known about optimal timing of multimodal evoked potential (EP)-investigations regarding prediction of MS disability. The aim of this study was to investigate whether timing of EP-investigations during a relapse or in the relapse-free interval influences prediction of MS disability. MethodsTwo groups of MS patients with similar age and gender distributions received visual, motor and somatosensory EPs either during a relapse (Group 1) or in the relapse-free interval (Group 2). Expanded Disability Status Score (EDSS) was obtained at baseline (T0) and year 3 (T2). Linear regression analysis was performed to examine the association between EDSST2 and a baseline EP compound measure (s-EP-QT0) for each group. ResultsMedian EDSST0 was 3.0 for Group 1 and 1.5 for Group 2. Mean disease durations were 2.0 and 2.8years, respectively. Median EDSST2 was 2.0 for both groups. The s-EP-QT0 significantly predicted EDSST2 (R2=0.47) for patients in Group 2, but not for patients in Group 1 (R2=0.07). ConclusionIn early MS the functional remnants of relapses are a better predictor for development of medium-term disability than is the extent of impulse propagation impairment measured during relapse. SignificanceThis suggests a role of multimodal EPs in prediction of MS disability if performed in the relapse-free interval.

The utility of multimodal evoked potentials in multiple sclerosis prognostication

The ability to predict disability development in multiple sclerosis (MS) is limited. While abnormalities of evoked potentials (EP) have been associated with disability, the prognosticating utility of EP in MS remains to be fully elucidated. The present study assessed the utility of multimodal EP as a prognostic biomarker of disability in a cohort of clinically heterogeneous MS patients. Median and tibial nerve somatosensory, visual, and brainstem auditory EP were performed at initial assessment on 63 MS patients (53 relapsing–remitting and 10 secondary progressive) who were followed for an average of 2years. A combined EP score (CEPS) was calculated consisting of the total number of abnormal EP tests, and was correlated with the Expanded Disability Status Scale (EDSS) at baseline and follow-up. There was a significant correlation between multimodal EP and baseline and follow-up EDSS. Specifically, tibial nerve P37 latencies correlated with EDSS (RBASELINE=0.49, p<0.01; RFOLLOW-UP=0.47, p<0.01), as did the median nerve N13 (RBASELINE=0.40, p<0.01; RFOLLOW-UP=0.35, p<0.05) and N20 latencies (RBASELINE=0.43, p<0.01; RFOLLOW-UP=0.47, p<0.01), and P100 full-field (RBASELINE=0.50, p<0.001; RFOLLOW-UP=0.45, p<0.001) and central field latencies (RBASELINE=0.60, p<0.001; RFOLLOW-UP=0.50, p<0.001). In addition, there was a significant correlation between the CEPS with baseline (R=0.65, p<0.001) and follow-up (R=0.57, p<0.01) EDSS. In contrast, white matter disease burden, as measured by T2 lesion load, exhibited a weaker correlation with EDSS (RBASELINE=0.28, p<0.05). In conclusion, these findings suggest that abnormalities of EP, as quantified by the novel CEPS, may be a useful biomarker for prognosticating clinical disability in MS, and may aid in the quantification of MS disease severity and in guiding therapeutic decisions.

Electrophysiological markers and predictors of the disease course in primary progressive multiple sclerosis

Background: Currently no valid surrogate marker exists for primary progressive multiple sclerosis (PPMS). Objective: Our aim was to prospectively investigate multimodal evoked potentials (EPs) as markers and predictors of the disease course in PPMS. Methods: Twenty-two PPMS patients were prospectively examined with visual, somatosensory and motor EPs and Expanded Disability Status Scale (EDSS) assessments at baseline (T0) and at six-month intervals over three years. Spearman rank correlation was used to determine the relationship between EP measures and EDSS. The relationship between disease evolution and a numerical score derived from z-transformed EP-latencies (s-EP-Q) and baseline characteristics was further assessed using multivariable linear regression analysis. Results: s-EP-Q correlated with EDSS score at all points in time in cross-sectional comparison (0.53≤rs ≤0.68; 0.0007≤p≤0.0232) and also longitudinally by trend (rs=0.46, p=0.0740). The s-EP-QT0 correlated with the EDSS score at year 3 (T6) (rs=0.77, p<0.0001). The s-EP-Q changes became statistically significant six months before corresponding changes were seen in the EDSS score. EDSST6 as predicted by EDSST6= −1.027+0.037* age+0.217* s-EP-QT0 + 0.695* EDSST0 correlated with the observed values (rs=0.92, p<0.0001). Conclusions: Multimodal EPs correlate well with disability in PPMS, and allow some prediction of the disease course over three years. These findings support a role of EPs as surrogate markers in clinical trials in PPMS.

The diagnostic value of multimodal evoked potentials in the determination of subclinical neurological involvement of Wilson’s disease

Wilson's disease (WD) is a progressive degeneration of hepatolenticular tissue caused by excessive tissue-damaging copper accumulation and in which liver involvement most frequently presents in childhood. Neurological signs also accompany liver disease with time. However, subclinical neurological involvement may occur earlier and diagnostic methods that reveal this subclinical involvement are not well established. The aim of the current study is to assess the subclinical neurological involvement by using multimodality evoked potential (EP) measurements and to explore the relationship between neurological disease and the severity of liver damage. The patient group included 28 children (mean age 11.8 ± 2.9 years, range 5.5-17) diagnosed with WD and a control group included 24 age-matched healthy children. Multimodality EP tests (Nihon Kohden Neuropack 8 4200K) of both groups were performed at the Department of Neurology Electrophysiology Laboratory of Ege University. At least one abnormal EP value was observed in 53.5% of the children in the patient group. At least on one side, there were abnormal values for visual evoked potential (VEP) P100, brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SEP), where the ratios were 25, 28.5, and 11%, respectively. Absolute latency values of patients with right-side VEP P100 and left-side BAEP I, and the interpeak latency values of right-side BAEP I-III were significantly high. The difference in right-side BAEP I-III interpeak latency between cirrhotic and noncirrhotic groups was found to be statistically significant (P<0.05). The EP examinations can be an indicator of subclinical brain damage in non-neurological WD; however, cirrhosis because of WD does not cause an increase in the EP values. Detection of changes in the EP values periodically, especially at the time of diagnosis and during the treatment follow-up, may be valuable for revealing subclinical impairment.

Prognostic Value of Vitamin D in Clinically Isolated Syndromes (CIS) (P05.104)

Objective: to evaluate the correlation between 25(OH)D levels in CIS patients and the development of Clinically Defined Multiple Sclerosis (CDMS). Background the risk of developing MS is related to both genetic and environmental factors. Vitamin D could play a role in CDMS conversion. Design/Methods: this is a single-center retrospective study. 107 CIS patients hospitalized from 2000 to 2009 at San Raffaele Hospital were recruited. We evaluated baseline serum 25(OH)D levels as well as clinical, brain MRI, Multimodal Evoked Potential and CSF data. Results: 21% patients developed CDMS at 12-month follow-up, 36% at 2-year follow-up and 44% at 5 year follow-up. A statistically significant inverse correlation between 25(OH)D levels and the risk to develop CDMS (OR 2.197) was observed. The difference between serum 25(OH)D levels in patients who developed CDMS and patients who did not, is statistically higher during summertime (p Conclusions: the presence of at least 3 negative clinical/paraclinical prognostic factors highly predicts short term conversion to CDMS (OR 5.39, p Disclosure: Dr. Martinelli has received personal compensation for activities with Biogen Dompe, Merck Serono, Bayer Schering, Teva, and Sanofi Aventis as a speaker. Dr. Dalla Costa has nothing to disclose. Dr. Colombo has nothing to disclose. Dr. Dalla Libera has nothing to disclose. Dr. Leocani has nothing to disclose. Dr. Furlan has nothing to disclose. Dr. Rubinacci has nothing to disclose. Dr. Filippi has received personal compensation for activities with ECTRIMS, MSIF, MS Ireland, US NMSS, Bayer-Schering, Biogen-Dompe AG, Genmab, Merck Serono, Pepgen Corporation, Teva, and Sanofi-Aventis. Dr. Filippi has received research support from Teva, Bayer-Schering and Genmab. Dr. Comi has received personal compensation for activities with Novartis, Teva Neuroscience, Sanofi-Aventis Pharmaceuticals, Inc., Merck Serono, Bayer Schering, and Biogen Dompe.

The Utility of Multimodal Evoked Potentials in Multiple Sclerosis Prognostication (P01.162)

The Utility of Multimodal Evoked Potentials in Multiple Sclerosis Prognostication (P01.162)