Abstracts / Journal of the Neurological Sciences 333 (2013) e358–e421
Objectives: Determination of the most sensitive tests to be performed in sensory CIDP and the most frequent clinical findings. Patients and methods: We examined medical records of 16 patients with sensory CIDP according to the EFNS/PNS guideline (revised 2010). In all patients were performed: clinical evaluation, electrophysiological investigations and cerebro-spinal fluid (CSF) macroscopic/microscopic examination. A full routine blood biochemistry was done. Biopsy specimens of the sural nerve were prepared for light and electron microscopic examination. Results: Sex ratio: 12 male and 4 female patients (55–79 years). Duration of progression from onset to maximal disability ranged from 4 months–8 years. CSF protein was elevated in 10 patients, ranging from 0.5-1.9 g/l, and normal in 6 cases. Motor and sensory nerve conduction abnormalities were present in 7 cases. Somatosensory evoked potential (SSEP) tests showed involvement of proximal segments or roots of sensory nerves in all patients. Sural nerve biopsy was performed in 5 cases. A definite decrease in the population of myelinated fibres was in all cases. Conclusion: 1. Nerve conduction study is not a sensitive test to diagnose sensory CIDP, in 56% cases results were normal. 2. SSEP is a highly sensitive test to be used for identifying a possible CIDP. 3. Nerve biopsy is used mainly when electrophysiological studies fail to establish the diagnosis of CIDP. doi:10.1016/j.jns.2013.07.1436
Abstract — WCN 2013 No: 1508 Topic: 6 — MS & Demyelinating Diseases Estimation of the gene polymorphisms role in the progression of multiple sclerosis using MSSS method K. Bacic Baronicaa, K. Mlinacb, A. Vladica, R. Barabaa, I. Zuntarc, S. Kalanj-Bognarb. aUniversity Department of Neurology, University Hospital Sveti Duh, Croatia; bCroatian Institute for Brain Research, School of Medicine, University of Zagreb, Croatia; cFaculty of Pharmacy and Biochemistry, University of Zagreb, Zagreb, Croatia Background: The estimation of the MS progression is important for therapeutic and rehabilitation approach. Commonly used scale for disability evaluation EDSS is not reliable for assessment of progression so MSSS method was developed. Objective: To explore the association of polymorphisms in arylsulfatase A (ASA) and glutathione S-transferase P1 (GSTP1) gene with disability and progression of MS. Subjects and methods: The frequency of N350S and 1524 + 95 A-G polymorphisms associated with ASA-pseudodeficiency (ASA-PD), and of A313G and C341T polymorphisms in GSTP1 gene, was determined in 56 and 58 MS patients, respectively, using PCR-RFLP method. EDSS was used to estimate disability level and MSSS to estimate disease progression. Correlation between genotypes and progression was analyzed by Kruskal–Wallis test. Results: Presence of one or both ASA-PD polymorphisms was determined in 13 patients, from which 9 had mild, 1 moderate and 3 severe disability. No correlation was found between ASA-PD genotypes and MSSS (p N 0.05). However, 10 polymorphism carriers had MSSS N5 which indicates faster disease progression. 32 and 12 patients were found to be carriers of A313 and C341 GSTP1 polymorphism respectively. No correlation was found between investigated GSTP1 genotypes and disability (p N 0.05), however patients-homozygous carriers of mutated A313G genotype had significantly higher mean MSSS than patients with normal or heterozygous genotype (p b 0.05). Conclusion: These results suggest that investigated polymorphisms may be associated with MS disability and progression. MSSS method
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was proved to be useful for estimation of MS progression as well as for identifying factors affecting disease progression such as gene polymorphisms. doi:10.1016/j.jns.2013.07.1437
Abstract — WCN 2013 No: 1507 Topic: 6 — MS & Demyelinating Diseases Neurologic manifestations in inflammatory bowel diseases M. Ben Djebara, A. Nasri, Y. Sidhom, Y. Hizem, I. Kacem, A. Gargouri-Berrechid, R. Gouider. Department of Neurology/Research Unit 03/UR/08-09, Research Unit 12SP21, Razi Hospital, Tunis, Tunisia Introduction: Crohn's disease (CD) and ulcerative colitis (UC) are known as inflammatory bowel diseases (IBD). The association of IBD with neurologic involvement is rare (3%) and controversial. Objective: To report neurologic manifestations in patients with IBD in order to address its clinical characteristics. Patients and methods: We conducted a retrospective study over an 11-year period including all patients diagnosed with IBD and neurologic manifestations. Demographic data, neurologic examination, studies and imaging, and treatment were analyzed. Results: We identified thirteen patients diagnosed with IBD and CNS symptoms: eight with CD and five with UC. Mean age was 51.1 years, mean age of onset of IBD was 38.1 and of neurologic symptoms was 43.3. Sex ratio was 2.5. Most of the patients developed neurologic manifestations after digestive symptoms appeared (11). Cognitive deficits were the most frequent manifestation observed (8 patients), followed by headache (4), epilepsy (3), cerebro-vascular disorders (2), movement disorders (2) and primary progressive MS (1). Two patients had peripheral neuropathy. CNS imaging showed cortical atrophy, white matter abnormalities or vascular lesions. Half of the patients have undergone surgery for their IBD. Conclusion: The high frequency of cognitive deficits in our series constitutes a peculiar feature, in opposition to higher prevalence peripheral neuropathies, cerebro-vascular disorders (0.12–4%) and CNS white matter diseases in other series. The pathogenesis of neurologic manifestations of IBD is probably related to a common dysimmune basis. Neuropsychological assessment and CNS imaging are needed in patients with IBD for a better understanding of CNS involvement in those diseases. doi:10.1016/j.jns.2013.07.1438
Abstract — WCN 2013 No: 1523 Topic: 6 — MS & Demyelinating Diseases A new model of focal inflammatory demyelination in the neocortex of the rat S. Hochmeistera, T. Birngruberb, M. Zeitelhofer-Adzemovicc, M. Haindla, T.R. Pieberd, F. Sinnere. aDepartment of Neurology, Medical University Graz, Austria; bBiomedical Technology and Monitoring, Joanneum Research, Graz, Austria; cDepartment of Clinical Neuroscience, Center of Molecular Medicine, Karolinska Institute, Stockholm, Sweden; dDepartment of Endocrinology, Medical University Graz, Austria; eJoanneum Research, Biomedical Technology and Monitoring, Graz, Austria Experimental Autoimmune Encephalomyelitis (EAE) is a widely used model which mimics many histopathological features of multiple sclerosis. However, there are some important disadvantages; firstly, the lesions commonly affect predominantly the spinal cords. Secondly the
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Abstracts / Journal of the Neurological Sciences 333 (2013) e358–e421
lesion's exact localization is not predictable. We present a new technique of creating a focal lesion in the neocortex of the rat by cerebral open flow microperfusion (cOFM). cOFM is a minimal invasive sampling technique involving implantation of a probe into the neocortex without causing a glial scar encapsulation and enables direct access to the brain tissue. After a healing phase liquids can be introduced continuously into the brain tissue via the cOFM probe and sampling of interstitial fluid with cOFM allows monitoring of biomarkers and metabolites. Two weeks after probe implantation, the animals are subcutaneously injected with 100 μg MOG in incomplete Freund's adjuvant to induce a stable IgG antibody titer against MOG. Then 250 ng recombinant TNF-alpha and 150 U IFN-g in phosphate buffered saline are injected via the cOFM probe. This results in a selective disturbance of the blood brain barrier and an influx of the MOG antibodies. An inflammatory demyelinating lesion forms in direct vicinity of the probe. Conclusion: Our new method enables us to predictably produce an EAE lesion in the neocortex of the rat. The cOFM probe allows monitoring of all stages of lesion formation. doi:10.1016/j.jns.2013.07.1439
Abstract — WCN 2013 No: 1535 Topic: 6 — MS & Demyelinating Diseases Pseudotumoral presentation of multiple sclerosis: Report of seven cases M.Z. Saied, A. Nasr, N. Kermani, A. Khefifi, S. Neija, S. Ben Amor, S. Ben Ammou. Department of Neurology, Sahloul Hospital, Sousse, Tunisia Background: Multiple sclerosis (MS) with focal demyelinating lesions of the cerebrum mimicking a tumor of the central nervous system is a rare phenomenon. Objective: To assess the clinical and radiological characteristics of pseudotumoral forms of MS. Methods: Among the patients seen at the neurological department between 2000 and 2012, we identified all patients fulfilling McDonald Criteria and whose initial MRI (brain or spinal) presented a large tumefactive lesions. Only patients who have had onset since at least 5 years were included. Results: Seven young women with tumor-like lesion on initial MRI (brain and/or spinal) are described. The average age of onset was 29 years. The beginning of neurological signs was progressive in four cases and acute in three cases. The main presentations included motor deficits in three cases, headache in two cases and a partial seizure in two cases. MRI showed a single large lesion in three cases and multifocal tumefactive lesions in four cases. The average duration of follow-up was seven years. In our study no biopsy and no surgery were undertaken. The favorable response to steroid therapy (4 cases), the spontaneous remission of symptoms (3 cases), the MRI control findings and the prolonged survival of patients supported the diagnosis of pseudotumoral MS. Conclusion: This study illustrates the wide variety of MS presentation and underlines the importance of considering MS in the differential diagnosis of a brain mass. doi:10.1016/j.jns.2013.07.1440
Abstract — WCN 2013 No: 1576 Topic: 6 — MS & Demyelinating Diseases Cortical reorganization pattern as a predictor of clinical recovery after the relapse of multiple sclerosis S. Kulikovaa, A. Peresedovab, V. Bryukhova, O. Trifonovab, M. Krotenkovaa, I. Zavalishinb. aNeuroradiology, Research Center
of Neurology RAMS, Moscow, Russia; bNeuroinfection, Research Center of Neurology RAMS, Moscow, Russia Movement disorders are frequent syndrome in multiple sclerosis (MS), leading to severe disability. Early predictors of clinical outcome are important for optimization of rehabilitation therapy. Thus the aim of the study was to investigate cortical reorganization pattern during relapse and in follow-up as a predictor of clinical outcome. Data were acquired from 25 MS patients, all right-handed, age ranged 19–50, during relapse characterized by unilateral light hand palsy, and in three months during persistent remission. All patients underwent full neurological examination; nine-hole peg test (NHPT) was additionally used to test hand function. Functional MRI (fMRI) was performed on 1.5 T scanner using simple movement paradigm for both hands. FMRI data analysis showed differently directed changes during relapse: 15 patients had smaller primary sensorimotor cortex (SM1) activation during palsy hand movement in comparison with non-palsy (G1), 10 patients had larger activation (G2). All patients had NHPT palsy hand performance time increase in comparison with non-palsy: G1(NHPT) ratio median 1.2; G2(NHPT) ratio median 1.315. G2 palsy hand activation volume correlated with NHPT performance time (r = 0.76, Spearman's rho). In three months G1 still had smaller activation during former palsy hand movement in comparison with non-palsy; G2 former palsy hand activation decreased and was comparable with non-palsy. G1 NHPT palsy hand performance time in three months was still elevated (ratio median 1.09) and differed from G2 (Mann–Whitney Utest, p b 0.045), where NHPT performance time of both hands was comparable (ratio median 0.96). Thus decrease of SM1 activation during relapse could be considered as more unfavorable pattern for recovery.
doi:10.1016/j.jns.2013.07.1441
Abstract — WCN 2013 No: 1702 Topic: 6 — MS & Demyelinating Diseases Comparison of McDonald 2005, McDonald 2010 and Swanton criteria for prediction of conversion from clinically isolated syndrome to multiple sclerosis B. Piri Cinar, S. Ozakbas. Dokuz Eylül University School of Medicine, Izmir, Turkey Background: In 2006, Swanton and colleagues modified the MRI criteria to simplify and speed the diagnosis. Recently the Panel has proposed new MRI criteria for the diagnosis of MS in patients with CIS. Purpose: To compare the ability of the McDonald 2005, McDonald 2010 and Swanton's modified criteria to predict conversion CIS to clinically definite multiple sclerosis (CDMS) from baseline MRI findings. We also aimed to evaluate the accuracy of these new criteria for lesions dissemination in space (DIS) and time (DIT). Patients and methods: 44 patients presenting with CIS included in the study, and followed up for at least 2 years. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of MRI dissemination-in-space criteria were calculated. Results: Overall conversion rate was 86.4%. Out of 44 patients, 38 converted to CDMS a mean of 10.1 months after onset of first clinical event. Swanton's modified criteria showed a sensitivity of 84%, a specificity of 23%, PPV is 88%, NPV is 25%, with an accuracy of 77%. Barkoff–Tintore criteria showed a sensitivity of 81%, a specificity of 50%, PPV is 91%, NPV is 30% with an accuracy of 77%. The McDonald 2010 modified criteria a sensitivity of 47.3%, a specificity of 83.3%, PPV is 94.7%, and NPV is 20%, an accuracy of 52.2%.