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OPL043 Multifocal motor neuropathy: an observational study of the clinical, electrophysiological and self-reported disability outcomes in 54 patients
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Tuesday, November 8, 2005
households), hemorrhagic in 25.6% (1.7% of 2,038 households), and unknown in 16.5%. Their stroke was managed in general hospitals in 54.9',/0, oriental medicine hospital in 19.5%, local clinic in 6.0',/0, and local oriental medicine clinic in 9.0%. The outcome was excellent in 24.1',/o, lnildly to moderately disabled in 34.6"/0, and bed-ridden in 5.3%. And 36.1% of the stroke victims died. 12.5% of them experienced stroke recurrence. Stroke itself was the most common cause o f death (83.3%) and 4.2% of stroke deaths were suicide. Conclusion: We conclude that the prevalence rate of stroke in Korean households is quite comparable to that observed in other countries. Further studies are necessary to elucidate the effectiveness o f the stroke care system in Korea. OPL042 Global Stroke Initiative in Honduras: Report tloii1 the First 4 Years of the Program Marco T Medina 1, Roberto Padilla 1, Amflcar Zelaya 1, Betty Avila-Alvarado 2, Arnold Thompson 3, Reyna M Durbn a, Julia N Bailey 4. 1Neurology Training Program, National Autonomous
University of Honduras, Tegucigalpa, Honduras; 2Internal Medicine Training Program, National Autonomous University of Honduras; SSchool of Medical Sciences, National Autonomous University of Honduras; 4David Geffen School of Medicine, Neurospsyehiatrie Institute, Universityof California at Los Angeles, United States ofAmerica Background: Stroke is a public health problem in Honduras. Our purpose was to apply the methodology of the Global Stroke Initiative as a pilot program in Honduras. Methods: We applied the WHO Stepwise Stroke approach evaluating hospital stroke events, community fatal and non-fatal events, and prevalence of risk factors for stroke. Between 2001 and 2005 we developed surveillance sites, a hospital stroke registry (Hospital Escuela, Tegucigalpa City) and two population-based registries at Colonia Kem~edy (Tegucigalpa) and the Salamfi rural County. Results: Urban population-based study (11,573 inhabitants in 2001, 2003 and 2004): stroke prevalence was 5.7/1000. In a case-control study (2004) high blood pressure's OR was 8.27 (i~ -- 0.039, 95% C I - 0.87-78.01). The door-to-door survey detemrined a prevalence of high blood pressure of 24.8%, 56"/0 of cases were not under treatment or adequate control. Stroke incidence was 66/100, 000. Most strokes were ischernic. Rural population-based study (15,396 inhabitants in 2004): Prevalence was 3.5/1000, subtypes are under study. Hospitalbased study (182 consecutive cases during 2003): Case-fatality rate at 1 month was 24%. Stroke subtypes were: ischemfic (61%), hemorrhagic (124"/o) and non-detemlined (115"/o). Risk factors were high blood pressure (55%), snicking (32%), alcoholisnl (29%), and diabetes (9%). Conclusions: Both urban and rural prevalence rates of stroke were similar to those from developed and Latin American countries. Ischemic stroke was more frequent, but hemorrhagic strokes are very common. High blood pressure and lifestyle variations may explain etiology. A stroke care system in Honduras should reduce the kigh case-fatality. Support and funds by: The Education Committee of the World Federation of Neurology and the Horowitz Foundation.
Motor Neuropathy & Motor Neurone Disease OPL043 Multifocal Motor Neuropathy: an observational study of the clinical, eleetrophysiological and self-reported disability outcomes in 54 patients Slee, M L3, Selvan, A 2, and Donaghy, M ~. 1Department of Clinical
Neurology" and Neurophysiology2, Radelffe Infirmary, Oxford, UK; 2Department of Neurophysiology, Radehffe Infirmary, Oxford, U~" SDepartment of Neurology, Austin Health, Melbourne, Australia
Oral Platform Abstracts Methods: The clinical and electrophysiological data of 54 patients with muhifocal motor neuropathy were studied from a tertiary centre. 30 patients participated in a telephone interview assessing disability. Results: 37 (69%) patients were male. Average symptom duration prior to diagnosis was 6 years. 36 (68"/0) patients had distal upper limb onset, however a substantial proportion (26"/0) had purely lower limb onset. The clinical features of marked weakness in non-wasted muscles and differential weakness across a common motor nerve terminal branch were recorded in over 50% of patients. 16 (30%) had elevated anti-GM-1 ganglioside titres. 12 patients (22%) demonstrated fasciculation, muscle hypertrophy, myokyJlfia or neuromyotonia. Detailed neurophysiologic assessment was available for 48 patients who were stratified into three groups. Group I: patients with definite or probable conduction block (AAEM criteria). Group II: patients with conduction block according to new criteria (Gosh & Donaghy in press) and Group III: patients with no conduction block. Conduction block was detected in only 62.5"/0 (30/48) of patients using either conventional or new criteria. 18 patients had no conduction block but most demonstrated other features of demyelination. 26 (49%) patients were successfully IVIG treated. 10% were IVIG trial mlresponsive and 40% of patients did not require treatment. New criteria identified 11 patients who would otherwise not qualify for conduction block according to standard A A E M criteria. Of these, 6 were treated successfully with IVIG. In the questiomtaire group (IN - 31) the significant majority of treated patients demonstrated stability or improvement in disability rating scores across time, however some deteriorated despite treatment. OPL045 Impaired nlitochondrial anti-oxidant defenee in SODl-related i'alililJ.al amyotrophic lateral sclerosis (FALS)
Wood-Allum, C ~, Barber, S ~, Kirby, f , Heath, pa, Holden, H l, Beaujemx, T a, Allen, S a, Alexson, S2, Ince, p3, Shaw, pa. 1Academic
Neurology Unit, Shefj)'eld University Medical School, Sheffield, UK," 2Department of Medical Laboratory Sciences and Technology, Karolinska Instituter, Sweden; SAcademic Pathology Unit, Sheffield University Medical School, Sheffield, UK Background: Whilst the majority of amyotrophic lateral sclerosis (ALS) occurs sporadically, .~2% is due to nmtations to CuZn superoxide disnmtase (SOD1). It is still unclear how nlutant SOD1 injures motor neurons but increasing evidence suggests that mitochondrial dysfunction may be important in disease pathogenesis. Methods: 2D-SDS PAGE, MALDI-TOF MS and online database searching were used to identify changes in rnitochondrial protein expression brought about by nlutant SOD1 in NSC34 cells. Confirmatory Western blotting was performed in NSC34 cells and key changes also confirmed in SOD 1 transgenic mouse spinal cord. Q-PCR of laser-capture microdissected spinal motor neurons was then used to compare the m R N A levels of key proteins in sporadic ALS and SODl-related FALS cases compared to controls. Results: The expression of 29 proteins changed in a mutant SOD1specific manner. These included anti-oxidant enzymes, apoptotic effectors, and electron transport chain components. Peroxiredoxin 3 (Pr x 3), a tlfioredoxin-dependent hydroperoxidase, was dowll-regulated in mutant SODl-expressing cells and in SODI transgenic mouse spinal cord. Q-PCR for Pr×3 further suggested down-regulation in SALS and SODl-related FALS cases compared to controls. Data from our pharmacological manipulation o f lnitochondrial anti-oxidant defence will also be presented. Conehisions: Given the evidence for oxidative stress in ALS, it is interesting that Pr×3, an anti-oxidant rnitochondrial matrix protein is down-regulated in the presence of mutant SOD1 in NSC34 cells, in SODI transgenic mice and in patients. Changes in mitochondrial anti-oxidant defence may play a role in the death of motor neurones ill SOD 1-related FALS and its modulation may offer therapeutic opportunities.
Tuesday, November 8, 2005
households), hemorrhagic in 25.6% (1.7% of 2,038 households), and unknown in 16.5%. Their stroke was managed in general hospitals in 54.9',/0, oriental medicine hospital in 19.5%, local clinic in 6.0',/0, and local oriental medicine clinic in 9.0%. The outcome was excellent in 24.1',/o, lnildly to moderately disabled in 34.6"/0, and bed-ridden in 5.3%. And 36.1% of the stroke victims died. 12.5% of them experienced stroke recurrence. Stroke itself was the most common cause o f death (83.3%) and 4.2% of stroke deaths were suicide. Conclusion: We conclude that the prevalence rate of stroke in Korean households is quite comparable to that observed in other countries. Further studies are necessary to elucidate the effectiveness o f the stroke care system in Korea. OPL042 Global Stroke Initiative in Honduras: Report tloii1 the First 4 Years of the Program Marco T Medina 1, Roberto Padilla 1, Amflcar Zelaya 1, Betty Avila-Alvarado 2, Arnold Thompson 3, Reyna M Durbn a, Julia N Bailey 4. 1Neurology Training Program, National Autonomous
University of Honduras, Tegucigalpa, Honduras; 2Internal Medicine Training Program, National Autonomous University of Honduras; SSchool of Medical Sciences, National Autonomous University of Honduras; 4David Geffen School of Medicine, Neurospsyehiatrie Institute, Universityof California at Los Angeles, United States ofAmerica Background: Stroke is a public health problem in Honduras. Our purpose was to apply the methodology of the Global Stroke Initiative as a pilot program in Honduras. Methods: We applied the WHO Stepwise Stroke approach evaluating hospital stroke events, community fatal and non-fatal events, and prevalence of risk factors for stroke. Between 2001 and 2005 we developed surveillance sites, a hospital stroke registry (Hospital Escuela, Tegucigalpa City) and two population-based registries at Colonia Kem~edy (Tegucigalpa) and the Salamfi rural County. Results: Urban population-based study (11,573 inhabitants in 2001, 2003 and 2004): stroke prevalence was 5.7/1000. In a case-control study (2004) high blood pressure's OR was 8.27 (i~ -- 0.039, 95% C I - 0.87-78.01). The door-to-door survey detemrined a prevalence of high blood pressure of 24.8%, 56"/0 of cases were not under treatment or adequate control. Stroke incidence was 66/100, 000. Most strokes were ischernic. Rural population-based study (15,396 inhabitants in 2004): Prevalence was 3.5/1000, subtypes are under study. Hospitalbased study (182 consecutive cases during 2003): Case-fatality rate at 1 month was 24%. Stroke subtypes were: ischemfic (61%), hemorrhagic (124"/o) and non-detemlined (115"/o). Risk factors were high blood pressure (55%), snicking (32%), alcoholisnl (29%), and diabetes (9%). Conclusions: Both urban and rural prevalence rates of stroke were similar to those from developed and Latin American countries. Ischemic stroke was more frequent, but hemorrhagic strokes are very common. High blood pressure and lifestyle variations may explain etiology. A stroke care system in Honduras should reduce the kigh case-fatality. Support and funds by: The Education Committee of the World Federation of Neurology and the Horowitz Foundation.
Motor Neuropathy & Motor Neurone Disease OPL043 Multifocal Motor Neuropathy: an observational study of the clinical, eleetrophysiological and self-reported disability outcomes in 54 patients Slee, M L3, Selvan, A 2, and Donaghy, M ~. 1Department of Clinical
Neurology" and Neurophysiology2, Radelffe Infirmary, Oxford, UK; 2Department of Neurophysiology, Radehffe Infirmary, Oxford, U~" SDepartment of Neurology, Austin Health, Melbourne, Australia
Oral Platform Abstracts Methods: The clinical and electrophysiological data of 54 patients with muhifocal motor neuropathy were studied from a tertiary centre. 30 patients participated in a telephone interview assessing disability. Results: 37 (69%) patients were male. Average symptom duration prior to diagnosis was 6 years. 36 (68"/0) patients had distal upper limb onset, however a substantial proportion (26"/0) had purely lower limb onset. The clinical features of marked weakness in non-wasted muscles and differential weakness across a common motor nerve terminal branch were recorded in over 50% of patients. 16 (30%) had elevated anti-GM-1 ganglioside titres. 12 patients (22%) demonstrated fasciculation, muscle hypertrophy, myokyJlfia or neuromyotonia. Detailed neurophysiologic assessment was available for 48 patients who were stratified into three groups. Group I: patients with definite or probable conduction block (AAEM criteria). Group II: patients with conduction block according to new criteria (Gosh & Donaghy in press) and Group III: patients with no conduction block. Conduction block was detected in only 62.5"/0 (30/48) of patients using either conventional or new criteria. 18 patients had no conduction block but most demonstrated other features of demyelination. 26 (49%) patients were successfully IVIG treated. 10% were IVIG trial mlresponsive and 40% of patients did not require treatment. New criteria identified 11 patients who would otherwise not qualify for conduction block according to standard A A E M criteria. Of these, 6 were treated successfully with IVIG. In the questiomtaire group (IN - 31) the significant majority of treated patients demonstrated stability or improvement in disability rating scores across time, however some deteriorated despite treatment. OPL045 Impaired nlitochondrial anti-oxidant defenee in SODl-related i'alililJ.al amyotrophic lateral sclerosis (FALS)
Wood-Allum, C ~, Barber, S ~, Kirby, f , Heath, pa, Holden, H l, Beaujemx, T a, Allen, S a, Alexson, S2, Ince, p3, Shaw, pa. 1Academic
Neurology Unit, Shefj)'eld University Medical School, Sheffield, UK," 2Department of Medical Laboratory Sciences and Technology, Karolinska Instituter, Sweden; SAcademic Pathology Unit, Sheffield University Medical School, Sheffield, UK Background: Whilst the majority of amyotrophic lateral sclerosis (ALS) occurs sporadically, .~2% is due to nmtations to CuZn superoxide disnmtase (SOD1). It is still unclear how nlutant SOD1 injures motor neurons but increasing evidence suggests that mitochondrial dysfunction may be important in disease pathogenesis. Methods: 2D-SDS PAGE, MALDI-TOF MS and online database searching were used to identify changes in rnitochondrial protein expression brought about by nlutant SOD1 in NSC34 cells. Confirmatory Western blotting was performed in NSC34 cells and key changes also confirmed in SOD 1 transgenic mouse spinal cord. Q-PCR of laser-capture microdissected spinal motor neurons was then used to compare the m R N A levels of key proteins in sporadic ALS and SODl-related FALS cases compared to controls. Results: The expression of 29 proteins changed in a mutant SOD1specific manner. These included anti-oxidant enzymes, apoptotic effectors, and electron transport chain components. Peroxiredoxin 3 (Pr x 3), a tlfioredoxin-dependent hydroperoxidase, was dowll-regulated in mutant SODl-expressing cells and in SODI transgenic mouse spinal cord. Q-PCR for Pr×3 further suggested down-regulation in SALS and SODl-related FALS cases compared to controls. Data from our pharmacological manipulation o f lnitochondrial anti-oxidant defence will also be presented. Conehisions: Given the evidence for oxidative stress in ALS, it is interesting that Pr×3, an anti-oxidant rnitochondrial matrix protein is down-regulated in the presence of mutant SOD1 in NSC34 cells, in SODI transgenic mice and in patients. Changes in mitochondrial anti-oxidant defence may play a role in the death of motor neurones ill SOD 1-related FALS and its modulation may offer therapeutic opportunities.