Anastasios Orologas

Multiple sclerosis registries in Europe – results of a systematic survey

Background: Identification of MS registries and databases that are currently in use in Europe as well as a detailed knowledge of their content and structure is important in order to facilitate comprehensive analysis and comparison of data. Methods: National MS registries or databases were identified by literature search, from the results of the MS Barometer 2011 and by asking 33 national MS societies. A standardized questionnaire was developed and sent to the registries’ leaders, followed by telephone interviews with them. Results: Twenty registries were identified, with 13 completing the questionnaire and seven being interviewed by telephone. These registries differed widely for objectives, structure, collected data, and for patients and centres included. Despite this heterogeneity, common objectives of the registries were epidemiology (n=10), long-term therapy outcome (n=8), healthcare research (n=9) and support/basis for clinical trials (n=8). While physician-based outcome measures (EDSS) are used in all registries, data from patients’ perspectives were only collected in six registries. Conclusions: The detailed information on a large number of national MS registries in Europe is a prerequisite to facilitating harmonized integration of existing data from MS registries and databases, as well as comprehensive analyses and comparison across European populations.

Learning of a novel visuo-postural co-ordination task in adults with multiple sclerosis.

OBJECTIVE A behavioural study was conducted to investigate how cerebellar dysfunction associated with multiple sclerosis affects the ability to learn a novel visuo-postural co-ordination task. DESIGN A prospective design, 2 group by 1 treatment (4 practice blocks). SUBJECTS Ten patients with multiple sclerosis diagnosed with cerebellar ataxia and 10 age-matched healthy controls. METHODS Participants stood over a dual force platform (ERBE Balance System) and performed visually guided lateral weight-shifting movements. The task required subjects to gradually transfer weight between sides while maintaining each foots force vector within visually specified force constraints ranging from 0% to 100% of bodyweight with maximum allowed variation set to +/-20%. The time required to complete the task and the number of spatial errors (noted each time the foots vector exceeded the +/-20% force constraint) were recorded. Training consisted of 3 blocks of 5 trials separated by 1-minute intervals and followed by 5 retention trials. RESULTS AND CONCLUSION Statistics revealed a significant decrease in movement time and spatial errors across trial blocks in both groups; however, the group with multiple sclerosis showed a limited and slower rate of performance improvement characterized by increased within- and between-subject variability. These findings may have important implications in the design of rehabilitation protocols for improving motor skill performance in adults with multiple sclerosis.

A comparison of platelet monoamine oxidase activity and phosphatidylserine content between chronic paranoid schizophrenics and normal controls

Mitochondrial monoamine oxidase (MAO), type B, has been implicated in the etiology of schizophrenia. We have found the phospholipid, phosphatidylserine (PS) to be a highly specific inhibitor of MAO-B, which has led us to postulate that the PS-MAO interaction might offer a basis for the lower MAO levels observed in platelets from certain schizophrenic populations. In this study we compared platelet MAO activity with phospholipid composition in a group of normals and chronic paranoid schizophrenics. The phospholipids in platelets and erythrocytes were extracted and separated by high-performance liquid chromatography into major classes phosphatidylcholine (PC), phosphatidylethanolamine, lysophosphatidylethanolamine, phosphatidylinositol and PS. The paranoid subjects showed statistically significantly lower MAO activity as well as higher mean levels of PS and lower levels of PC in both platelets and erythrocytes, consistent with our hypothesis. The Ca2+-stimulated synthesis of serine-lipid in platelets was also monitored by incorporation of radioisotope into lipid extracts from 14C-labelled serine substrate, and no significant differences were found between subjects groups with respect to this parameter.

Neurological complications of beta-thalassemia

The thalassemias are the most common single gene disorder in the world. Over the last years, several reports have demonstrated neurological complications in beta-thalassemia patients. In most cases, these complications remained subclinical and were detected only during neuropsychological, neurophysiological, or neuroimaging evaluation. Cognitive impairment, abnormal findings on evoked potentials, complications due to extramedullary hematopoiesis, cerebrovascular disease, and peripheral neuropathy comprise the broad spectrum of neurological involvement. Chronic hypoxia, iron overload, desferrioxamine neurotoxicity, and bone marrow expansion are implicated, but sufficient explanatory evidence is lacking and development of biomarkers is needed. This review summarizes current knowledge of the neurological complications. As life expectancy for beta-thalassemia patients increases, we support the use of neurophysiological, neuropsychological, or neuroimaging monitoring, enabling the evaluation of neural pathway impairment, to achieve appropriate management and as a result a better quality of life for this patient group.

Animal models of central nervous system immune-mediated diseases: therapeutic interventions with bioactive peptides and mimetics.

Experimental allergic encephalomyelitis (EAE) is a T helper 1 (Th1) mediated autoimmune disease and the principal animal model for multiple sclerosis (MS). Like MS, EAE is characterized by a coordinated inflammatory attack on the myelin sheath in the central nervous system (CNS), with damage to axons. No matter whether the ideal animal model is not yet available, much knowledge concerning the pathogenesis of MS has been achieved through studies on EAE. Dissecting the underlying immune mechanisms provided recognition of several myelin antigens that are vulnerable in autoimmune attack. The beneficial effect and the mechanism of action of a number of the currently used immunomodulating agents in MS therapy were first indicated in EAE. Altered peptide ligands (APL) can modulate T-cell responses to native peptide antigens implicated in the pathogenesis of autoimmune diseases such as MS and EAE. However, peptide therapy is hindered due to the sensitivity of peptides to proteolytic enzymes as well as due to some immune-mediated side effects. A number of cyclic myelin peptide analogs seem to be potential candidates in maintaining the biological function of the original peptide and effective in controlling inflammation in EAE. Additional data regarding the immunomodulating and neuroprotective effect of these much promising agents is required. Based on the data from studies on EAE models, clinical trials should also be designed in order to elucidate the impact of such APL-induced immune responses in MS disease activity. These clinical trials should carefully incorporate monitoring of both clinical, neuroimaging and immunological parameters.

Multiple Sclerosis Patients Valuing Their Own Health Status: Valuation and Psychometric Properties of the 15D.

An ongoing debate on decision and cost-utility analyses is whether to use preferences of general public or patients. The aim of this study was to replicate the valuation procedure of the multi-attribute utility generic measure, 15D, using a sample of multiple sclerosis (MS) patients and to assess its psychometric properties. Consecutive outpatient MS patients were recruited from two MS centers in Greece. The three-stage valuation procedure was applied and, with the use of elicited preference weights, an MS patients’ algorithm was developed. The original Finnish value set derived from healthy individuals was also used to calculate scores and a comparison between the two algorithms was made. A total of 64 MS patients were evaluated. The 15D scores obtained with the MS patients’ valuation algorithm were higher than the original one. The derived utilities differed significantly with respect to age, depressive symptoms, Expanded Disability Status Scale score and clinical form. MS patients indicated as most important domains mobility, mental functioning and vitality. Cronbach’s alpha was estimated 0.876 and correlations between relevant dimensions of the instruments were moderate to high. The 15D was generally feasible and reliable in patients with MS and the valuation system yielded acceptable psychometric properties.

Neuronal morphology of nucleus accumbens-drug addicted brain region

Address: 1Institute of Anatomy, School of Medicine Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia and Montenegro, 2Institute of Anatomy, School of Medicine, Belgrade, Serbia and Montenegro, 3University department of neurology, Aristotle University of Thessaloniki, Greece, 4Institute of Biological Sciences, National Research Council, Ottawa, Canada and 5Clinic of Neurology, School of Medicine, Kragujevac, Serbia and Montenegro * Corresponding author

Polyneuropathy and myopathy in beta-thalassemia major patients

The thalassemias are the most common single gene disorder in the world. Nowadays, the average life expectancy of patients in developed countries has increased significantly, while, there was an increase of complications. We aimed to investigate peripheral neuropathy and myopathy in this patient group using a neurophysiological study. We performed nerve conduction studies and electromyography of upper and lower extremities on 36 beta-thalassemia major (β-thal) patients. The electrophysiological findings were correlated with demographic data and laboratory parameters of the disease. Patients with β-thal present polyneuropathy or myopathy at (50%). Polyneuropathy was detected in (38.9%) and myopathy in (27.8%), while polyneuropathy and myopathy were present at (16.7%) with an overlap of the diseases in 1/3 of the patients. There was not a statistically significant correlation of polyneuropathy and myopathy with age, sex, splenectomy, nor with respect to laboratory parameters, hemoglobin, and ferritin. However, there was a statistically significant correlation of polyneuropathy and myopathy with iron overload, as recorded by the magnetic resonance imaging (MRI) of the heart and the liver. Our findings suggest that iron overload plays a key role in the pathogenesis of polyneuropathy and myopathy in β-thal patients, and performing heart and liver MRI for the prediction of such lesions in an annual basis is warranted.

Nasopharyngeal tumor diagnosed with the use of computerized axial tomography

Abstract The skull base and the nasopharynx was studied with Computerized Axial Tomography in a patient with a suspected tumor in this region. Cuts of 5 mm thickness at 5 mm intervals were performed. Invasion of the clivus and projection within the pontine cistern was revealed. Pathology study proved that it was a case of chordoma.

Hypoparathyroidism with severe brain calcinosis presenting as non-convulsive status epilepticus

2nd Congress of the European Academy of Neurology, June, 2016, Copenhagen, Denmark, Neurology and Neurosurgery, Chisinau, Moldova, Municipal Clinical Emergency Hospital, Chisinau, Moldovacancelled P21005 Clinical features of adult-onset neuronal intranuclear inclusion disease Y. Harigaya1, Y. Nakano2, H. Hatsuta2, M. Sato1, K. Mizushima1, S. Murayama2 1Neurology Service, Maebashi Red Cross Hospital, Maebashi, Japan, 2Department of Neurology and Neuropathology, Tokyo Metropolitan Institute of Gerontology, Itabashi, Japan Background and aims: Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder characterised by eosinophilic intranuclear hyaline inclusions in neuronal cells. Recently ante-mortem diagnosis can be made by identification of those inclusions in skin biopsy on the basis of characteristic findings in brain diffusion-weighted imaging (DWI). However, the onset and clinical symptoms of NIID are variable Methods: We evaluated features of 8 adult-onset NIID patients (5 males, 3 females, aged 63-75 years) with clinical examination, electrophysiological study, brain MRI, and pathological findings by skin biopsy. Results: Six patients were sporadic, while 2 were brothers. Mean age at onset was 62.8 years. The initial symptom was memory impairment, disorientation, syncope, tremor, or gait disturbance. The mean scores of MMSE, Japanese version of Montreal Cognitive Assessment and Frontal Assessment Battery were 22.5/30, 16.6/30 and 10.3/18, respectively, indicating frontal lobe dysfunction. Neurological examinations demonstrated generalized hypoareflexia in 8, urinary incontinence and orthostatic hypotension in 3, ataxic gait and parkinsonism in 2. Electrophysiological findings revealed mildly reduced motor and sensory conduction velocities and amplitudes in all. Each patient showed high intensities of corticomedullary junctions in DWI and subcortical white matter lesions with ventricular dilatation and brain atrophy in FLAIR images. Skin biopsies from all demonstrated eosinophilic intranuclear inclusions immunostained with anti-ubiquitin and anti-p62 antibodies in sweat gland cells, adipocytes and fibroblasts, which were composed of filamentous structure without limiting membranes by electron microscopy. Conclusion: Our findings indicate that peripheral nerves are highly involved in NIID. In cases of neuropathy with leukoencephalopathy, NIID should be considered for differential diagnosis. Disclosure: Nothing to disclose