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The clinical, electrophysiological and morphological peculiarities of sensory CIDP
e396
Abstracts / Journal of the Neurological Sciences 333 (2013) e358–e421
transferrin levels are predictive of developing abnormally high cerebral iron deposition as measured by MRI. doi:10.1016/j.jns.2013.07.1432
Abstract — WCN 2013 No: 1689 Topic: 6 — MS & Demyelinating Diseases The benefits of visual evoked potential examination in the diagnosis and prognosis of multiple sclerosis M.K. Onara, M. Terzib, S. Senb, S. Cambazb. aNeurology, Samsun, Turkey; bOndokuz Mayis University, Samsun, Turkey Background: Visual evoked potentials (VEPs) have been used in the diagnosis of multiple sclerosis (MS). But, in the revised form of McDonald criteria it was not included. Objective: We wanted to investigate the relation between the VEPs and the clinical types and the prognosis of MS. Patients and methods: In 354 MS patients VEPs were evaluated. The relation between VEP results and neurologic findings at onset of the disease, IgG index, oligoclonal band positivity, EDSS scores at onset, 1st year, 5th year, 10th year and transition from clinical isolated syndrome to MS was determined. The patients were grouped according to their initial neurological findings such as optic neuritis, supratentorial involvement, brain-stem–cerebellar involvement and spinal involvement. The VEP findings were classified as monoocular, bilateral pathology and no pathology. Results: VEP abnormalities were more frequent in patients in optic neuritis group as expected. VEP abnormalities were less in the spinal group. In patients with bilateral VEP abnormality EDSS scores at onset, 1st, 5th and 10th years were higher than the patients with monoocular abnormality and no abnormality. No relation was found between the VEP abnormalities and the clinical types of the disease and transition from clinical isolated syndrome to MS. Conclusions: We suggest that VEP examination can predict the evolution of disability in MS patients. doi:10.1016/j.jns.2013.07.1433
Abstract — WCN 2013 No: 1775 Topic: 6 — MS & Demyelinating Diseases Characteristics of headaches in cases diagnosed with neuro-behcet's disease D. Selcukia, A. Kisabaya, T. Pırıldarb. aNeurology, Manisa, Turkey; b Celal Bayar University, Manisa, Turkey Introduction and aims: Behcet's disease is a vascular-inflammatory chronic disease with an obscure pathogenesis. Primary neurological involvement is in the form of non-structural headache (migrainous HA), subclinical NBS, cerebral dural sinus vein thrombosis (extra axial), MSS involvement (intraaxial), neuro-psycho-behcet and peripheral nervous involvements. Method: In this study, patients applying to the outpatient Behcet clinic between 2000 and 2013 were examined retrospectively. Headache severity was evaluated using VAS scales. The types of headaches of the patients diagnosed with headaches in Behcet's disease were made in compliance with the 2011 diagnosis criteria of the International Headache Society. Localization of headaches, the existence of aura, frequency, severity, duration of attacks, neurological examinations, accompanying diseases, clinic, methods of imaging, treatment, and treatments for prophylaxis in the acute and chronic period of the patients who applied to the clinic were reviewed.
Conclusion: Different from Behcet's syndrome, in the 50% of the cases it was found in compliance with intracranial increase in pressure. Lumbar function opening pressures changed between 250 and 350 mm of water. In the imaging examinations (cranial MRG, MRA) the existence of venous thrombosis (43%), and the ratio of parenchymal involvement (50%) were rather high. Corticosteroid infusion is given to neuro-behcet patients during attacks and immunosuppressant treatment added following this. Nearly 90% of the cases benefited from the treatment given. Discussion: Headaches are the most common neurological complaint of cases diagnosed with neuro-behcet. Cases were given appropriate treatment for their clinical findings and symptoms and nearly all of them showed full or partial remission. doi:10.1016/j.jns.2013.07.1434
Abstract — WCN 2013 No: 1761 Topic: 6 — MS & Demyelinating Diseases Characteristics of headaches in cases diagnosed with behcet's disease A. Kisabay, D. Selcuki, T. Pırıldar. Neurology, Celal Bayar University, Manisa, Turkey Introduction and aim: Behcet's disease (BD) is a chronic disease which progresses with the involvement of many organs and systems. It is a vascular-inflammatory disease with an obscure pathogenesis. Characteristics of headaches in patients diagnosed with Behcet's disease, their differences from other types of headaches, methods of imaging and treatment were planned to be assessed in accordance with literature. Method: In this study, patients applying to the outpatient Behcet clinic between 2000 and 2013 were examined retrospectively. The types of headaches of the patients diagnosed with headaches in Behcet's disease were made in compliance with the 2011 diagnosis criteria of the International Headache Society. Localization of headaches, the existence of aura, frequency, severity, duration of attacks, neurological examinations, accompanying diseases, clinic, methods of imaging, treatment, and treatments for prophylaxis in the acute and chronic period of the patients who applied to the clinic were inspected. Conclusion: It was found out that 24 of the 42 (57%) patients applying to the clinic had headaches. Approximately half of these cases had tensiontype headaches (33%) and the other half (58%) had migraine-type headache. It was remarkable that most of the cases complained that their headaches were more severe and frequent during or before attacks. Discussion: Characteristics and circumstances that need special attention were reemphasized for the treatment of these cases which were assessed with the existence of autoimmunity along with headache types. doi:10.1016/j.jns.2013.07.1435
Abstract — WCN 2013 No: 1765 Topic: 6 — MS & Demyelinating Diseases The clinical, electrophysiological and morphological peculiarities of sensory CIDP E. Gavriliuca, J.-M. Légerb, V. Lisnica, V. Nemtana, L. Munteanua. a State Medical and Pharmaceutical University ‘Nicolae Testemitanu’, Chisinau, Moldova; bReference Center for Neuromuscular Diseases, Hôpital Pitié-Salpêtrière, Paris, France Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired disorder of peripheral sensory and motor nerves. In 5–6% of patients with CIDP only sensory symptoms are present.
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
e397
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
Abstracts / Journal of the Neurological Sciences 333 (2013) e358–e421
transferrin levels are predictive of developing abnormally high cerebral iron deposition as measured by MRI. doi:10.1016/j.jns.2013.07.1432
Abstract — WCN 2013 No: 1689 Topic: 6 — MS & Demyelinating Diseases The benefits of visual evoked potential examination in the diagnosis and prognosis of multiple sclerosis M.K. Onara, M. Terzib, S. Senb, S. Cambazb. aNeurology, Samsun, Turkey; bOndokuz Mayis University, Samsun, Turkey Background: Visual evoked potentials (VEPs) have been used in the diagnosis of multiple sclerosis (MS). But, in the revised form of McDonald criteria it was not included. Objective: We wanted to investigate the relation between the VEPs and the clinical types and the prognosis of MS. Patients and methods: In 354 MS patients VEPs were evaluated. The relation between VEP results and neurologic findings at onset of the disease, IgG index, oligoclonal band positivity, EDSS scores at onset, 1st year, 5th year, 10th year and transition from clinical isolated syndrome to MS was determined. The patients were grouped according to their initial neurological findings such as optic neuritis, supratentorial involvement, brain-stem–cerebellar involvement and spinal involvement. The VEP findings were classified as monoocular, bilateral pathology and no pathology. Results: VEP abnormalities were more frequent in patients in optic neuritis group as expected. VEP abnormalities were less in the spinal group. In patients with bilateral VEP abnormality EDSS scores at onset, 1st, 5th and 10th years were higher than the patients with monoocular abnormality and no abnormality. No relation was found between the VEP abnormalities and the clinical types of the disease and transition from clinical isolated syndrome to MS. Conclusions: We suggest that VEP examination can predict the evolution of disability in MS patients. doi:10.1016/j.jns.2013.07.1433
Abstract — WCN 2013 No: 1775 Topic: 6 — MS & Demyelinating Diseases Characteristics of headaches in cases diagnosed with neuro-behcet's disease D. Selcukia, A. Kisabaya, T. Pırıldarb. aNeurology, Manisa, Turkey; b Celal Bayar University, Manisa, Turkey Introduction and aims: Behcet's disease is a vascular-inflammatory chronic disease with an obscure pathogenesis. Primary neurological involvement is in the form of non-structural headache (migrainous HA), subclinical NBS, cerebral dural sinus vein thrombosis (extra axial), MSS involvement (intraaxial), neuro-psycho-behcet and peripheral nervous involvements. Method: In this study, patients applying to the outpatient Behcet clinic between 2000 and 2013 were examined retrospectively. Headache severity was evaluated using VAS scales. The types of headaches of the patients diagnosed with headaches in Behcet's disease were made in compliance with the 2011 diagnosis criteria of the International Headache Society. Localization of headaches, the existence of aura, frequency, severity, duration of attacks, neurological examinations, accompanying diseases, clinic, methods of imaging, treatment, and treatments for prophylaxis in the acute and chronic period of the patients who applied to the clinic were reviewed.
Conclusion: Different from Behcet's syndrome, in the 50% of the cases it was found in compliance with intracranial increase in pressure. Lumbar function opening pressures changed between 250 and 350 mm of water. In the imaging examinations (cranial MRG, MRA) the existence of venous thrombosis (43%), and the ratio of parenchymal involvement (50%) were rather high. Corticosteroid infusion is given to neuro-behcet patients during attacks and immunosuppressant treatment added following this. Nearly 90% of the cases benefited from the treatment given. Discussion: Headaches are the most common neurological complaint of cases diagnosed with neuro-behcet. Cases were given appropriate treatment for their clinical findings and symptoms and nearly all of them showed full or partial remission. doi:10.1016/j.jns.2013.07.1434
Abstract — WCN 2013 No: 1761 Topic: 6 — MS & Demyelinating Diseases Characteristics of headaches in cases diagnosed with behcet's disease A. Kisabay, D. Selcuki, T. Pırıldar. Neurology, Celal Bayar University, Manisa, Turkey Introduction and aim: Behcet's disease (BD) is a chronic disease which progresses with the involvement of many organs and systems. It is a vascular-inflammatory disease with an obscure pathogenesis. Characteristics of headaches in patients diagnosed with Behcet's disease, their differences from other types of headaches, methods of imaging and treatment were planned to be assessed in accordance with literature. Method: In this study, patients applying to the outpatient Behcet clinic between 2000 and 2013 were examined retrospectively. The types of headaches of the patients diagnosed with headaches in Behcet's disease were made in compliance with the 2011 diagnosis criteria of the International Headache Society. Localization of headaches, the existence of aura, frequency, severity, duration of attacks, neurological examinations, accompanying diseases, clinic, methods of imaging, treatment, and treatments for prophylaxis in the acute and chronic period of the patients who applied to the clinic were inspected. Conclusion: It was found out that 24 of the 42 (57%) patients applying to the clinic had headaches. Approximately half of these cases had tensiontype headaches (33%) and the other half (58%) had migraine-type headache. It was remarkable that most of the cases complained that their headaches were more severe and frequent during or before attacks. Discussion: Characteristics and circumstances that need special attention were reemphasized for the treatment of these cases which were assessed with the existence of autoimmunity along with headache types. doi:10.1016/j.jns.2013.07.1435
Abstract — WCN 2013 No: 1765 Topic: 6 — MS & Demyelinating Diseases The clinical, electrophysiological and morphological peculiarities of sensory CIDP E. Gavriliuca, J.-M. Légerb, V. Lisnica, V. Nemtana, L. Munteanua. a State Medical and Pharmaceutical University ‘Nicolae Testemitanu’, Chisinau, Moldova; bReference Center for Neuromuscular Diseases, Hôpital Pitié-Salpêtrière, Paris, France Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired disorder of peripheral sensory and motor nerves. In 5–6% of patients with CIDP only sensory symptoms are present.
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
e397
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