0987 A Young-onset Parkinson's disease — Sri Lankan profile
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Poster Abstracts Wednesday, November 9, 2005 $351 was measured by high performance liquid chromatography. In 15 minutes after treatment with L-DOPA, the relative fluorescence units in CDCFH decreased (p < 0.03) and plasma L-DOPA started to increase, indicating that L-DOPA lowered the intracellular ROS of circulating neutrophils. 0987 A Young-onset Parkinson's disease - Sri Lankan prof'de Wijeinanne, S 1, Alibhoy, A,T, 2, Fenmndo, N3,Gamage, R, 4. ~Senior Registrar, National Hospital, Colombo, Sri Lanka; 2Resident Neurologist, National Hospital Colombo, Sri Lanka; 3Senior ,Registrar, National Hospital, Colombo, Sri Lanka; 4Consultant Neurologist National Hospital, Colombo, Sri Lanka Background: Parkinson's disease is an uncommon disease in the young. Young-onset Parkinson's disease (YOPD) refers to patients whose onset is at age 21 to 40 years. We describe the clinical profile of 40 patients with Young-onset Parkinson's disease in Sri Lanka with a view to identifying differences in their profile when compared to the West. Method: Study was conducted on patients with Parkinson's disease in the above age group attending the Movement disorder clinic at the National Hospital of Sri Lanka. Result: The study was conducted on 40 patients (126 men and 14 women). The mean duration of illness was 11.4 years. The most common presenting manifestations were tremor (153%) and dystonia (20%). The majority 95% had developed bilateral disease by 5 years. Freezing was noted in 46"/0 and it was the predominant disability in 20"/0. Falls were less common (25"/0). Dementia, depression and psychosis were not seen. No significant medical or consistent environmental factor could be identified. Dyskinesia was seen in 53%, on an average 8 years from the disease onset and 6 years after starting levodopa. There was only one patient with a family lffstory. Conclusion: The clinical profile of our patients is similar to those in the West, with the high inddence of dystonia as a presenting feature. However the percentage affected by dyskinesia was nearly 50% less in our patients probably because of the smaller doses of levodopa that have been used in these patients. 0988 Paxkinson's disease (PD): MRI and radiographic imaging (DAT-scan and SPECT) findings correlating to autonomic and cognitive dysfunction M. Arnaoutoglou l, G.P. Spanos 2, G. Tsaleras a, A. Arnaoutoglou a, N. Vlaikidis a, V. Kosta a, G. Tiblalexi :, F. Sedadghat ~, S.I. Baloyannis 1. 11"~ Department of Neurology, AHEPA Hospital, Aristotle University of Thessaloniki, Greece; ZDepartment of,Radiology, AHEPA Hospital Aristotle University of Thessaloniki, Greece; 3Department of Nuclear Medicine, AHEPA Hospital Aristotle University of Thessaloniki, Greece Background: Neuroimaging of parkinsonean patients combined with clinical evaluation is currently used in differential diagnosis of PD from other extrapyranridal disorders (Parkinson-plus syndromes). Objective: The purpose of this study was the evaluation of neuroima- ging findings and their correlation to non-motor symptoms of PD. Methods: We evaluated 45 patients with PD, recording autonomic and cognitive dysfunction. All patients underwent brain MRI were evaluated SPECT and DAT-scan evaluating cerebral and brainstem atrophy, basal ganglia signal intensity changes, decrease of cerebral blood flow and dopamine uptake. Results: Cognitive and autonomic, deficits were more conspicuous in the advanced stages of PD. Cognitive dysfunction was correlated with cerebral atrophy although decrease of cortical blood flow was also present in Parkinson's disease patients with normal cognition and mild to moderate motor dysfunction. Autonomic disorders were mostly correlated with abnormal DAT/scan and basal ganglia signal intensity changes. Condusiom Neuroimaging techniques in PD are valuable not only as diagnostic tools but they also provide important data in the evaluation of disease progression. 0989 Alpha-l-Antichymotrypsin-AA genotype is a risk factor and modulating factor for Multiple System Atrophy Asai, H 1, Furiya, y1 Hirano, M 1, Kurumatani, N 2, Ueno, S 1. 1Department of Neurology', Nora Medical University, Japan; 2Department of Hygiene, Nora Medical University, Japan Background: Multiple system atrophy (MSA) is a sporadic., progressive neurodegenerative disease of undetermined cause that is characterized clinically by a combination of cerebellar, pyranffdal, extrapyranffdal, and autonomic disorders. The pathogenesis of tiffs disease and its risk factors remain unknown. MSA shares similar clinical features with Parkinson's disease (PD). Both diseases have recently been classified as synudeinopathies, suggesting involvement of a common pathogenetic mechanism. An alpha-l-antichymotrypsin (ACT) polymorplffsm in the signal peptide (-15 Ala to Thr) gives rise to three genotypes: ACT-AA, AT, and TT. Recent studies have demonstrated the ACT-AA genotype was reported to be associated with an increased risk oFPD. Method: We investigated ACT genotyp es in 105 patients with multiple system atrophy (MSA) and age-matched controls. The ACT concen- tration in cerebrospinal fluid (CSF) was measured by inmmnoturbi- dimetry in 32 out of 105 patients. Result: The frequency of ACT-AA genotype was significantly higher in the patients with MSA (20.0%) than in the controls (10.5%). The onset of MSA was significantly earlier and the disease progressed significantly faster in the patients with ACT-AA genotype than in those with the other genotypes. The ACT concentration in CSF was higher in the patients with ACT-AA (10.31 ± 0.03 mg/dl) than in those of ACT-TT (0.23 ± 0.01 mg/dl, p < 0.05). Conclusion: To our knowledge, this is the first study to show that the ACI'-AA genotype is a risk factor and modulating factor for MSA. Furthermore our findings suggest the involvement of ACT-relating inflanunatory process in the pathogenesis of MSA. 0990 Thl/Th2 Balance of Peripheral Immune System in Paxkinson Disease Baba, Y~, Kuroiwa, A ~, Tsuboi, ya, Yamada, T a. Departments of Neurologj and Microbiology and ImmunologV7, Fukuoka Universi(v School of Medicine, Fukuoka, Japan Background: Parkinson disease (PD) is a neurodegenerative dis- order pathologically characterized by dopaminergic cell loss in the substantia nigra and expression of Lewy bodies in the brain. Immunological alteration predominantly expressed CD8 T cells has been considered as a pathogenesis affecting the formation of PD pathology. Decreased CD4+/CD8 + T cells ratio in peripheral nimmne system has also been demonstrated. The similarity of immunologic phenotype in the central nervous and periphery sug- gests that systemic immunological alteration may be involved with development of PD. Mettloa: We assessed Thl/Th2 balance in the peripheral blood samples of 30 PD patients (17 males) and 30 normal control subjects (115 males) without any history of immunologic diseases. Thl/Th2 balance was evaluated based on the ratio of IFN-gamma and IL-4. Results: Twenty-four (80%) patients with PD and 11 (37%) control patients demonstrated an increased IFN-ganuna/IL-4 ratio. The distribution was statistically significant (p < 0.01). Historical investi- gation indicated that PD patients with increased IFN-ganmm/IL-4
Transcript of 0987 A Young-onset Parkinson's disease — Sri Lankan profile
Poster Abstracts Wednesday, November 9, 2005 $351
was measured by high performance liquid chromatography. In 15 minutes after treatment with L-DOPA, the relative fluorescence units in CDCFH decreased (p < 0.03) and plasma L-DOPA started to increase, indicating that L-DOPA lowered the intracellular ROS of circulating neutrophils.
0987 A Young-onset Parkinson's disease - Sri Lankan prof'de
Wijeinanne, S 1, Alibhoy, A,T, 2, Fenmndo, N3,Gamage, R, 4. ~Senior Registrar, National Hospital, Colombo, Sri Lanka; 2Resident Neurologist, National Hospital Colombo, Sri Lanka; 3Senior ,Registrar, National Hospital, Colombo, Sri Lanka; 4Consultant Neurologist National Hospital, Colombo, Sri Lanka
Background: Parkinson's disease is an uncommon disease in the young. Young-onset Parkinson's disease (YOPD) refers to patients whose onset is at age 21 to 40 years. We describe the clinical profile of 40 patients with Young-onset Parkinson's disease in Sri Lanka with a view to identifying differences in their profile when compared to the West. Method: Study was conducted on patients with Parkinson's disease in the above age group attending the Movement disorder clinic at the National Hospital of Sri Lanka. Result: The study was conducted on 40 patients (126 men and 14 women). The mean duration of illness was 11.4 years. The most common presenting manifestations were tremor (153%) and dystonia (20%). The majority 95% had developed bilateral disease by 5 years. Freezing was noted in 46"/0 and it was the predominant disability in 20"/0. Falls were less common (25"/0). Dementia, depression and psychosis were not seen. No significant medical or consistent environmental factor could be identified. Dyskinesia was seen in 53%, on an average 8 years from the disease onset and 6 years after starting levodopa. There was only one patient with a family lffstory. Conclusion: The clinical profile of our patients is similar to those in the West, with the high inddence of dystonia as a presenting feature. However the percentage affected by dyskinesia was nearly 50% less in our patients probably because of the smaller doses of levodopa that have been used in these patients.
0988 Paxkinson's disease (PD): MRI and radiographic imaging (DAT-scan and SPECT) findings correlating to autonomic and cognitive dysfunction
M. Arnaoutoglou l, G.P. Spanos 2, G. Tsaleras a, A. Arnaoutoglou a, N. Vlaikidis a, V. Kosta a, G. Tiblalexi :, F. Sedadghat ~, S.I. Baloyannis 1. 11"~ Department of Neurology, AHEPA Hospital, Aristotle University of Thessaloniki, Greece; ZDepartment of,Radiology, AHEPA Hospital Aristotle University of Thessaloniki, Greece; 3Department of Nuclear Medicine, AHEPA Hospital Aristotle University of Thessaloniki, Greece
Background: Neuroimaging of parkinsonean patients combined with clinical evaluation is currently used in differential diagnosis of PD from other extrapyranridal disorders (Parkinson-plus syndromes). Objective: The purpose of this study was the evaluation of neuroima- ging findings and their correlation to non-motor symptoms of PD. Methods: We evaluated 45 patients with PD, recording autonomic and cognitive dysfunction. All patients underwent brain MRI were evaluated SPECT and DAT-scan evaluating cerebral and brainstem atrophy, basal ganglia signal intensity changes, decrease of cerebral blood flow and dopamine uptake. Results: Cognitive and autonomic, deficits were more conspicuous in the advanced stages of PD. Cognitive dysfunction was correlated with cerebral atrophy although decrease of cortical blood flow was also present in Parkinson's disease patients with normal cognition and mild to moderate motor dysfunction. Autonomic disorders were mostly
correlated with abnormal DAT/scan and basal ganglia signal intensity changes. Condusiom Neuroimaging techniques in PD are valuable not only as diagnostic tools but they also provide important data in the evaluation of disease progression.
0989 Alpha-l-Antichymotrypsin-AA genotype is a risk factor and modulating factor for Multiple System Atrophy
Asai, H 1, Furiya, y 1 Hirano, M 1, Kurumatani, N 2, Ueno, S 1. 1Department of Neurology', Nora Medical University, Japan; 2Department of Hygiene, Nora Medical University, Japan
Background: Multiple system atrophy (MSA) is a sporadic., progressive neurodegenerative disease of undetermined cause that is characterized clinically by a combination of cerebellar, pyranffdal, extrapyranffdal, and autonomic disorders. The pathogenesis of tiffs disease and its risk factors remain unknown. MSA shares similar clinical features with Parkinson's disease (PD). Both diseases have recently been classified as synudeinopathies, suggesting involvement of a common pathogenetic mechanism.
An alpha-l-antichymotrypsin (ACT) polymorplffsm in the signal peptide (-15 Ala to Thr) gives rise to three genotypes: ACT-AA, AT, and TT. Recent studies have demonstrated the ACT-AA genotype was reported to be associated with an increased risk oFPD. Method: We investigated ACT genotyp es in 105 patients with multiple system atrophy (MSA) and age-matched controls. The ACT concen- tration in cerebrospinal fluid (CSF) was measured by inmmnoturbi- dimetry in 32 out of 105 patients. Result: The frequency of ACT-AA genotype was significantly higher in the patients with MSA (20.0%) than in the controls (10.5%). The onset of MSA was significantly earlier and the disease progressed significantly faster in the patients with ACT-AA genotype than in those with the other genotypes. The ACT concentration in CSF was higher in the patients with ACT-AA (10.31 ± 0.03 mg/dl) than in those of ACT-TT (0.23 ± 0.01 mg/dl, p < 0.05). Conclusion: To our knowledge, this is the first study to show that the ACI ' -AA genotype is a risk factor and modulating factor for MSA. Furthermore our findings suggest the involvement of ACT-relating inflanunatory process in the pathogenesis of MSA.
0990 Thl/Th2 Balance of Peripheral Immune System in Paxkinson Disease
Baba, Y~, Kuroiwa, A ~, Tsuboi, ya, Yamada, T a . Departments of Neurologj and Microbiology and ImmunologV 7, Fukuoka Universi(v School of Medicine, Fukuoka, Japan
Background: Parkinson disease (PD) is a neurodegenerative dis- order pathologically characterized by dopaminergic cell loss in the substantia nigra and expression of Lewy bodies in the brain. Immunological alteration predominantly expressed CD8 T cells has been considered as a pathogenesis affecting the formation of PD pathology. Decreased CD4+/CD8 + T cells ratio in peripheral nimmne system has also been demonstrated. The similarity of immunologic phenotype in the central nervous and periphery sug- gests that systemic immunological alteration may be involved with development of PD. Mettloa: We assessed Thl /Th2 balance in the peripheral blood samples of 30 PD patients (17 males) and 30 normal control subjects (115 males) without any history of immunologic diseases. Thl /Th2 balance was evaluated based on the ratio of IFN-gamma and IL-4. Results: Twenty-four (80%) patients with PD and 11 (37%) control patients demonstrated an increased IFN-ganuna/IL-4 ratio. The distribution was statistically significant (p < 0.01). Historical investi- gation indicated that PD patients with increased IFN-ganmm/IL-4
