- Email: [email protected]
1.259 RISPERIDONE VERSUS BEHAVIOUR THERAPY IN THE TREATMENT OF TIC DISORDERS – A RANDOMIZED SINGLE-BLINDED TRIAL
Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
Results: There are no large controlled clinical trials to guide hemiballismus therapy. However there are several agents that have been used to suppress these abnormal movements. One of which being dopamine receptor blocking agents. This case report demonstrated that catecholamine-depleting agent (tetrabenazine) is very effective in controlling hemiballismus. It has shown much better safety profile when long term therapy is needed. Conclusion: Tetrabenazine is the first drug approved by the FDA for the treatment of chorea in Huntington’s disease. Tetrabenazine appears to be an effective agent to manage other hyperkinetic disorders, such as hemiballisms associated with subthalamic lesions. 1.257 A CROSSOVER STUDY OF A TRADITIONAL ASIAN HERBAL MEDICINE FOR HUNTINGTON’S DISEASE K. Iwasaki1,2 , A. Kikuchi3 , A. Takeda3 , T. Satoh4 , J.-I. Sawada5 , H. Konno6 , T. Takahashi3 , S. Takayama2 , M. Tobita7 , N. Yaegashi2 . 1 Center for Traditional Asian Medicine, National Nishitaga Hospital, 2 Department of Traditional Asian Medicine, School of medicine, Tohoku University, 3 Department of Neurology, Tohoku University School of medicine, 4 Department of Neurology, Nagamachi Hospital, Sendai, 5 Department of Neurology, Osaka General Medical Center, Osaka, 6 Department of Neurology, Nishitaga National Hospital, Sendai, 7 Department of Neurology, Yonezawa National Hospital, Yonezawa, Japan Objective: A two-way crossover study of a traditional Asian herbal medicine yokukansan (YKS, Yi-gan San in Chinese) was conducted for Huntington’s disease (HD). Methods: YKS is a powdered extract of Atractylodis lanceae rhizoma, Poria, Cnidii rhizoma, Angelicae radix, Bupleuri radix, Glycyrrhizae radix, and Uncariae uncis cum ramulus. Thirteen HD patients were randomly assigned for one of two treatment sequences. Six patients were treated with YKS for 12 weeks then observed next 12 weeks without YKS (group A, 4 males and 2 female, mean age 47.5 and mean Unified Huntington’s Disease Rating Scale (UHDRS) 48.5±25.3). Other 7 patients were treated in reverse order (group B, 3 males and 4 females, mean age 47.3, and mean UHDRS 74.7±23.1). For each variable including total UHDRS, Maximal Chorea and Maximal Dystonia subscales, differences between the scores in the treated and untreated periods were calculated by analysis of variance (ANOVA). Results: Total UHDRS (p = 0.067) and Maximal Chorea (p = 0.055) showed a trend for reduction by YKS treatment when the data were adjusted for each baseline level. Total UHDRS (p = 0.089) and Maximal Dystonia (p = 0.073) also showed a trend for reduction with no adjustment. Extrapyramidal symptoms or changes of cognition or ADL were not observed.
Figure: UHDRS change with YKS treatment.
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Conclusions: Our study suggested that YKS treatment may reduce the involuntary movements in HD patients but the sample size was not enough to show the statistical significance. 1.258 EFFECT OF CAFFEIC ACID, ROFECOXIB AND THEIR COMBINATION AGAINST INTRASTRIATAL QUINOLINIC ACID INDUCED OXIDATIVE, MITOCHONDRIAL AND HISTOLOGICAL ALTERATIONS J. Mishra, H. Kalonia, A. Kumar. Institute of Pharmaceutical Sciences, Panjab University, Chandigarh, India Oxidative stress has long been implicated in the neurotoxic effects of glutamate acting through N-methyl-D-aspartate (NMDA) receptors. Therefore, present study has been designed to explore the effect of rofecoxib and caffeic acid on the involvement of oxidative stress, mitochondrial dysfunction and neuronal linked with NMDA receptor-mediated excitotoxicity. Caffeic acid, is a wellknown antioxidant flavanoid, implicate anti-inflammatory and immunomodulatory like actions. The present study is an attempt to investigate the antioxidant-like effect of caffeic acid and rofecoxib and their combination against QA-induced oxidative damage, mitochondrial dysfunction and histological alterations. Intrastriatal injection of quinolinic acid (300 nmol) significantly increased oxidative stress (raised lipid peroxidation, nitrite concentration, depleted SOD and catalase), altered mitochondrial complex enzyme activities and histological alteration in the ex vivo striatum. Caffeic acid (5 and 10 mg/kg, p.o.) and rofecoxib (10 and 20 mg/kg, p.o.) treatment for 21 days significantly attenuated oxidative damage and impairment in mitochondrial activities of complex enzymes in the ex vivo striatum. Further, combination of sub effective doses of rofecoxib (10 mg/kg, p.o.) and caffeic acid (5 mg/kg, p.o.) potentiated their protective effect which was significant as compared to their effect per se. The present study suggests the therapeutic effect of caffeic acid and rofecoxib combination against QA-induced ex vivo oxidative damage, mitochondrial and histological alterations in rats. 1.259 RISPERIDONE VERSUS BEHAVIOUR THERAPY IN THE TREATMENT OF TIC DISORDERS – A RANDOMIZED SINGLE-BLINDED TRIAL S. de Bruijn1 , C. Verdellen2 , D. Cath3 , M. Verbraak2 , A. Wertenbroek1 , J. van de Griendt2 , J. Rath1 , T. van Woerkom1 , Dutch Tourette Study Group. 1 Haga Teaching Hospital, The Hague, 2 HSK Group, Arnhem, 3 Academic Anxiety Centre Altrecht, Utrecht, The Netherlands Today there are no comparative studies between D2-blocking agents (antipsychotics) and exposure response prevention (ERP) in patients with Tourette syndrome (TS) or chronic tic disorders (CTD). In this randomised, single-blinded, open trial, 80 patients with TS or CTD will be randomized for medical treatment (Risperidone 2–6 mg. per day) or behaviour therapy (ERP during 12 weeks). After a baseline period of 2 weeks (week −2 to 0) and after 6 and 12 weeks of treatment, response will be measured using the Yale Global Tic Severity Scale (YGTSS). Follow-up data will be obtained at 3 and 9 months after end of treatment. Included are patients with DSM-IV criteria for TS or CTD, and both children and adults are included (range 6 to 65 years of age). Exclusion criteria are severe major depression, severe autism, psychosis, addiction, mental deficiency, cardiovascular disease, family history of QT prolongation, bradycardia, other medication known to prolong QT interval, and inability to read/speak Dutch, and a drug holiday for specific tic medication (antipsychotics) for at least four weeks prior to entering the study. Prior treatment is not an exclusion criterium. Primary outcome measure is tic severity measured by the YGTSS at week 12, directly post treatment. Secondary outcome measures include tic frequency rated at home (videotape tic counts and by partner/parent), quality of life and severity of premonitory urges. Patient characteristics and comorbidity are used to gain insight
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in predictors of response. Dropout rates and side effects will be compared in both conditions. 1.260 PROTECTIVE EFFECT OF ROFECOXIB AND NIMESULIDE AGAINST INTRA-STRIATAL QUINOLINIC ACID INDUCED BEHAVIORAL, OXIDATIVE STRESS AND MITOCHONDRIAL DYSFUNCTIONS IN RATS H. Kalonia, A. Kumar. University Institute of Pharmaceutical Sciences, Panjab University, Chandigarh, India Role of cyclooxygenase (COX) enzyme has been well documented in both physiological and pathological conditions. COX-1 and COX-2 converts arachidonic acid into prostaglandins. Non selective inhibition of COXs produces undesirable effects, whereas selective COX-2 inhibition produces protective effects in various inflammatory diseases. Recently, cyclooxygenase (COX) inhibitors have been implicated as a neuroprotectant in the treatment of various neurodegenerative diseases. Quinolinic acid is an endogenous excitotoxin that causes neurotoxicity in diverse areas of the brain and produces motor dysfunction. Present study is an attempt to investigate the possible role of COX inhibitors (selective COX-2 inhibitor and preferential COX-2 inhibitors) against quinolinic acid induced behavioral, oxidative stress and mitochondrial enzyme complex alterations in rats. Intra striatal administration of quinolinic acid (300 nmol) caused significant reduction in body weight (9%), motor in-coordination, oxidative damage [increased MDA (100%), nitrite cocentration (195%), depleted SOD (71%), catalase levels (70%)] and alteration in mitochondrial enzyme complex activity (decreased complex I (50%), II (50%) and IV (62%)) as compared to sham operated animals. Chronic treatment with rofecoxib (10 and 20 mg/kg, p.o.) and nimesulide (10 and 20 mg/kg, p.o.) significantly attenuated quinolinic acid-induced behavioral and biochemical alterations as compared to quinolinic acid 300 nmol treated group. Further, rofecoxib (10, 20 mg/kg) and nimesulide (20 mg/kg) significantly restored mitochondrial enzyme complex activities in striatum as compared to quinolinic acid 300 nmol treated group. Present study highlights the therapeutic potential of cyclooxygenase inhibitors against quinolinic acid induced neurotoxicity. 1.261 THAP1/DYT6 SEQUENCE VARIANTS IN NON-DYT1 EARLY ONSET PRIMARY DYSTONIA IN CHINA AND THEIR EFFECTS ON RNA EXPRESSION F.B. Cheng1,2 , L.J. Ozelius3 , X.H. Wan1 , J.C. Feng2 , L.Y. Ma1 , L. Wang1 , Y.M. Yang1 . 1 Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 2 Neurology, The First Affiliated Hospital of Jilin University, Changchun, China; 3 Genetics and Genomic Sciences and Neurology, Mount Sinai School of Medicine, New York, NY, USA Background: Mutations in the THAP1 gene were recently identified as the cause of DYT6 primary dystonia. More than 40 mutations in this gene have been described in different populations. However, no previous report has identified sequence variations that affect the transcript process of the THAP1 gene. In addition, the mutation frequency in Chinese early-onset primary dystonia has not been well characterized. Materials and Methods: One hundred and two unrelated patients with non-DYT1 early-onset primary dystonia (age at onset <26 years), family members of participants with mutations, and 200 neurological normal controls were screened for THAP1 gene mutations. The effects of the identified mutations on RNA expression were analyzed using semi-quantitative real-time PCR. Results: Seven sequence variants (c.63_66del TTTC, c.161 G>T, c.224A>T, c.267G>A, c.339 T>C, c.449A>C, and c.539T>C) were identified in this group of patients (6.9%). In this cohort, 15 subjects (seven unrelated patients and eight family members) were detected
to have THAP1 sequence variants. Among these 15 subjects, 11 were manifested (penetrance of DYT6 was 73.3%) and seven presented with craniocervical involvement (63.6%). However, one patient manifested paroxysmal headshake, and one presented with essential hand tremor. Semi-quantitative real-time PCR indicated that a novel silent mutation (c.267G>A) decreased the expression of THAP1 in human lymphocytes. Conlusions: Our findings indicated that THAP1 sequence variants are not common in non-DYT1 early-onset primary dystonia in China and that the clinical manifestation may vary. One silent mutation (c.267G>A) was shown to affect THAP1 expression. 1.262 CASE PRESENTATION: THE COEXISTENCE OF PSYCHOLOGICAL AND SOMATIC FACTORS IN A PATIENT WITH PAROXYSMAL DYSKINESIA V. Sajin1 , I. Moldovanu2 , C. Guranda2 . 1 Neurology, State Medial and Pharmaeutical University ‘Niolae Testemitanu’, 2 Institute of Neurology and Neurosurgery, Chisinau, Moldova Paroxysmal dyskinesia (PD) are characterized by sudden attacks of involuntary movements with different movement combinations and intact consciousness. The etiopathology of this disease is not completely understood, therefore the evaluation of the psychogenic impact on this disease could be useful in differential diagnosis, management and treatment of PDs. We examined a 63-years old patient with 30 years of frequent dyskinesic and dystonic attacks. He corresponded to the usual description of the paroxismal kinesigenic dyskinesia (Bhatia, 1999; Jankovic and Demirkiran, 2002; van Rootselaar et al., 2009, etc.). He has been examined and treated in many hospitals, without any significant result. We undertook a complete clinical and neurological examination, standard laboratory tests, EEG, MRI. The patient also completed Somatoform Disease Questionnaire-20 items and we performed the Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR). The general and neurological exams were normal, as well as the laboratory tests. Cerebral MRI detected the slight frontal lobe atrophy and brachidolicocephaly. On EEG there were only some theta waves after the attacks. The patient corresponded to the criteria of the the conversion disorder of the DSM-IV-TR. The majority of his crises decrease after the placebo injection of 4 ml solution NaCl 0.9%. During the medical consilium, the patient suddenly had a transient spastic gait, aphonia and limb weakness which disappeared after the injection of the solution NaCl 0.9%. The PD is a very polymorphic disease and it should be differentiated from the pure conversion disorder and the mixed form, when the management needs more psychological techniques. 1.263 FUNCTIONAL OUTCOME OF DEFEROXAMINE PRIMED HUMAN ADIPOSE DERIVED MESENCHYMAL STEM CELL TRANSPLANTS IN A RAT MODEL OF HUNTINGTON’S DISEASE M.A. Edalatmanesh1 , A.R. Bahrami2 , H. Naderi2 , E. Abbasi3 , Z. Alizadeh3 , H. Nikfarjam4 . 1 Department of Physiology, Islamic Azad University, Science & Research Branch, Fars, 2 Cell & Molecular Research Center, Iranian Academic Center for Education, Culture and Research (ACER), 3 School of Medicine, Islamic Azad University, Mashhad Branch, 4 Department of Biology, Ferdowsi University of Mashhad, Mashhad, Iran Introduction: One of the biggest handicaps in stem cell transplantation is improper migration and homing of administrated cells. The chemokine receptor, CXCR4, which may play an important role in cell homing and deferoxamine could be overexpress CXCR4 in mesenchymal stem cells. We examined whether in vitro priming of human adipose derived mesenchymal stem cells (hADMSCs) with defroxamine before transplantation into the Quinolinic acid (QA) lesioned rat model of Huntington disease altered in vivo hADMSCs
Results: There are no large controlled clinical trials to guide hemiballismus therapy. However there are several agents that have been used to suppress these abnormal movements. One of which being dopamine receptor blocking agents. This case report demonstrated that catecholamine-depleting agent (tetrabenazine) is very effective in controlling hemiballismus. It has shown much better safety profile when long term therapy is needed. Conclusion: Tetrabenazine is the first drug approved by the FDA for the treatment of chorea in Huntington’s disease. Tetrabenazine appears to be an effective agent to manage other hyperkinetic disorders, such as hemiballisms associated with subthalamic lesions. 1.257 A CROSSOVER STUDY OF A TRADITIONAL ASIAN HERBAL MEDICINE FOR HUNTINGTON’S DISEASE K. Iwasaki1,2 , A. Kikuchi3 , A. Takeda3 , T. Satoh4 , J.-I. Sawada5 , H. Konno6 , T. Takahashi3 , S. Takayama2 , M. Tobita7 , N. Yaegashi2 . 1 Center for Traditional Asian Medicine, National Nishitaga Hospital, 2 Department of Traditional Asian Medicine, School of medicine, Tohoku University, 3 Department of Neurology, Tohoku University School of medicine, 4 Department of Neurology, Nagamachi Hospital, Sendai, 5 Department of Neurology, Osaka General Medical Center, Osaka, 6 Department of Neurology, Nishitaga National Hospital, Sendai, 7 Department of Neurology, Yonezawa National Hospital, Yonezawa, Japan Objective: A two-way crossover study of a traditional Asian herbal medicine yokukansan (YKS, Yi-gan San in Chinese) was conducted for Huntington’s disease (HD). Methods: YKS is a powdered extract of Atractylodis lanceae rhizoma, Poria, Cnidii rhizoma, Angelicae radix, Bupleuri radix, Glycyrrhizae radix, and Uncariae uncis cum ramulus. Thirteen HD patients were randomly assigned for one of two treatment sequences. Six patients were treated with YKS for 12 weeks then observed next 12 weeks without YKS (group A, 4 males and 2 female, mean age 47.5 and mean Unified Huntington’s Disease Rating Scale (UHDRS) 48.5±25.3). Other 7 patients were treated in reverse order (group B, 3 males and 4 females, mean age 47.3, and mean UHDRS 74.7±23.1). For each variable including total UHDRS, Maximal Chorea and Maximal Dystonia subscales, differences between the scores in the treated and untreated periods were calculated by analysis of variance (ANOVA). Results: Total UHDRS (p = 0.067) and Maximal Chorea (p = 0.055) showed a trend for reduction by YKS treatment when the data were adjusted for each baseline level. Total UHDRS (p = 0.089) and Maximal Dystonia (p = 0.073) also showed a trend for reduction with no adjustment. Extrapyramidal symptoms or changes of cognition or ADL were not observed.
Figure: UHDRS change with YKS treatment.
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Conclusions: Our study suggested that YKS treatment may reduce the involuntary movements in HD patients but the sample size was not enough to show the statistical significance. 1.258 EFFECT OF CAFFEIC ACID, ROFECOXIB AND THEIR COMBINATION AGAINST INTRASTRIATAL QUINOLINIC ACID INDUCED OXIDATIVE, MITOCHONDRIAL AND HISTOLOGICAL ALTERATIONS J. Mishra, H. Kalonia, A. Kumar. Institute of Pharmaceutical Sciences, Panjab University, Chandigarh, India Oxidative stress has long been implicated in the neurotoxic effects of glutamate acting through N-methyl-D-aspartate (NMDA) receptors. Therefore, present study has been designed to explore the effect of rofecoxib and caffeic acid on the involvement of oxidative stress, mitochondrial dysfunction and neuronal linked with NMDA receptor-mediated excitotoxicity. Caffeic acid, is a wellknown antioxidant flavanoid, implicate anti-inflammatory and immunomodulatory like actions. The present study is an attempt to investigate the antioxidant-like effect of caffeic acid and rofecoxib and their combination against QA-induced oxidative damage, mitochondrial dysfunction and histological alterations. Intrastriatal injection of quinolinic acid (300 nmol) significantly increased oxidative stress (raised lipid peroxidation, nitrite concentration, depleted SOD and catalase), altered mitochondrial complex enzyme activities and histological alteration in the ex vivo striatum. Caffeic acid (5 and 10 mg/kg, p.o.) and rofecoxib (10 and 20 mg/kg, p.o.) treatment for 21 days significantly attenuated oxidative damage and impairment in mitochondrial activities of complex enzymes in the ex vivo striatum. Further, combination of sub effective doses of rofecoxib (10 mg/kg, p.o.) and caffeic acid (5 mg/kg, p.o.) potentiated their protective effect which was significant as compared to their effect per se. The present study suggests the therapeutic effect of caffeic acid and rofecoxib combination against QA-induced ex vivo oxidative damage, mitochondrial and histological alterations in rats. 1.259 RISPERIDONE VERSUS BEHAVIOUR THERAPY IN THE TREATMENT OF TIC DISORDERS – A RANDOMIZED SINGLE-BLINDED TRIAL S. de Bruijn1 , C. Verdellen2 , D. Cath3 , M. Verbraak2 , A. Wertenbroek1 , J. van de Griendt2 , J. Rath1 , T. van Woerkom1 , Dutch Tourette Study Group. 1 Haga Teaching Hospital, The Hague, 2 HSK Group, Arnhem, 3 Academic Anxiety Centre Altrecht, Utrecht, The Netherlands Today there are no comparative studies between D2-blocking agents (antipsychotics) and exposure response prevention (ERP) in patients with Tourette syndrome (TS) or chronic tic disorders (CTD). In this randomised, single-blinded, open trial, 80 patients with TS or CTD will be randomized for medical treatment (Risperidone 2–6 mg. per day) or behaviour therapy (ERP during 12 weeks). After a baseline period of 2 weeks (week −2 to 0) and after 6 and 12 weeks of treatment, response will be measured using the Yale Global Tic Severity Scale (YGTSS). Follow-up data will be obtained at 3 and 9 months after end of treatment. Included are patients with DSM-IV criteria for TS or CTD, and both children and adults are included (range 6 to 65 years of age). Exclusion criteria are severe major depression, severe autism, psychosis, addiction, mental deficiency, cardiovascular disease, family history of QT prolongation, bradycardia, other medication known to prolong QT interval, and inability to read/speak Dutch, and a drug holiday for specific tic medication (antipsychotics) for at least four weeks prior to entering the study. Prior treatment is not an exclusion criterium. Primary outcome measure is tic severity measured by the YGTSS at week 12, directly post treatment. Secondary outcome measures include tic frequency rated at home (videotape tic counts and by partner/parent), quality of life and severity of premonitory urges. Patient characteristics and comorbidity are used to gain insight
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in predictors of response. Dropout rates and side effects will be compared in both conditions. 1.260 PROTECTIVE EFFECT OF ROFECOXIB AND NIMESULIDE AGAINST INTRA-STRIATAL QUINOLINIC ACID INDUCED BEHAVIORAL, OXIDATIVE STRESS AND MITOCHONDRIAL DYSFUNCTIONS IN RATS H. Kalonia, A. Kumar. University Institute of Pharmaceutical Sciences, Panjab University, Chandigarh, India Role of cyclooxygenase (COX) enzyme has been well documented in both physiological and pathological conditions. COX-1 and COX-2 converts arachidonic acid into prostaglandins. Non selective inhibition of COXs produces undesirable effects, whereas selective COX-2 inhibition produces protective effects in various inflammatory diseases. Recently, cyclooxygenase (COX) inhibitors have been implicated as a neuroprotectant in the treatment of various neurodegenerative diseases. Quinolinic acid is an endogenous excitotoxin that causes neurotoxicity in diverse areas of the brain and produces motor dysfunction. Present study is an attempt to investigate the possible role of COX inhibitors (selective COX-2 inhibitor and preferential COX-2 inhibitors) against quinolinic acid induced behavioral, oxidative stress and mitochondrial enzyme complex alterations in rats. Intra striatal administration of quinolinic acid (300 nmol) caused significant reduction in body weight (9%), motor in-coordination, oxidative damage [increased MDA (100%), nitrite cocentration (195%), depleted SOD (71%), catalase levels (70%)] and alteration in mitochondrial enzyme complex activity (decreased complex I (50%), II (50%) and IV (62%)) as compared to sham operated animals. Chronic treatment with rofecoxib (10 and 20 mg/kg, p.o.) and nimesulide (10 and 20 mg/kg, p.o.) significantly attenuated quinolinic acid-induced behavioral and biochemical alterations as compared to quinolinic acid 300 nmol treated group. Further, rofecoxib (10, 20 mg/kg) and nimesulide (20 mg/kg) significantly restored mitochondrial enzyme complex activities in striatum as compared to quinolinic acid 300 nmol treated group. Present study highlights the therapeutic potential of cyclooxygenase inhibitors against quinolinic acid induced neurotoxicity. 1.261 THAP1/DYT6 SEQUENCE VARIANTS IN NON-DYT1 EARLY ONSET PRIMARY DYSTONIA IN CHINA AND THEIR EFFECTS ON RNA EXPRESSION F.B. Cheng1,2 , L.J. Ozelius3 , X.H. Wan1 , J.C. Feng2 , L.Y. Ma1 , L. Wang1 , Y.M. Yang1 . 1 Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 2 Neurology, The First Affiliated Hospital of Jilin University, Changchun, China; 3 Genetics and Genomic Sciences and Neurology, Mount Sinai School of Medicine, New York, NY, USA Background: Mutations in the THAP1 gene were recently identified as the cause of DYT6 primary dystonia. More than 40 mutations in this gene have been described in different populations. However, no previous report has identified sequence variations that affect the transcript process of the THAP1 gene. In addition, the mutation frequency in Chinese early-onset primary dystonia has not been well characterized. Materials and Methods: One hundred and two unrelated patients with non-DYT1 early-onset primary dystonia (age at onset <26 years), family members of participants with mutations, and 200 neurological normal controls were screened for THAP1 gene mutations. The effects of the identified mutations on RNA expression were analyzed using semi-quantitative real-time PCR. Results: Seven sequence variants (c.63_66del TTTC, c.161 G>T, c.224A>T, c.267G>A, c.339 T>C, c.449A>C, and c.539T>C) were identified in this group of patients (6.9%). In this cohort, 15 subjects (seven unrelated patients and eight family members) were detected
to have THAP1 sequence variants. Among these 15 subjects, 11 were manifested (penetrance of DYT6 was 73.3%) and seven presented with craniocervical involvement (63.6%). However, one patient manifested paroxysmal headshake, and one presented with essential hand tremor. Semi-quantitative real-time PCR indicated that a novel silent mutation (c.267G>A) decreased the expression of THAP1 in human lymphocytes. Conlusions: Our findings indicated that THAP1 sequence variants are not common in non-DYT1 early-onset primary dystonia in China and that the clinical manifestation may vary. One silent mutation (c.267G>A) was shown to affect THAP1 expression. 1.262 CASE PRESENTATION: THE COEXISTENCE OF PSYCHOLOGICAL AND SOMATIC FACTORS IN A PATIENT WITH PAROXYSMAL DYSKINESIA V. Sajin1 , I. Moldovanu2 , C. Guranda2 . 1 Neurology, State Medial and Pharmaeutical University ‘Niolae Testemitanu’, 2 Institute of Neurology and Neurosurgery, Chisinau, Moldova Paroxysmal dyskinesia (PD) are characterized by sudden attacks of involuntary movements with different movement combinations and intact consciousness. The etiopathology of this disease is not completely understood, therefore the evaluation of the psychogenic impact on this disease could be useful in differential diagnosis, management and treatment of PDs. We examined a 63-years old patient with 30 years of frequent dyskinesic and dystonic attacks. He corresponded to the usual description of the paroxismal kinesigenic dyskinesia (Bhatia, 1999; Jankovic and Demirkiran, 2002; van Rootselaar et al., 2009, etc.). He has been examined and treated in many hospitals, without any significant result. We undertook a complete clinical and neurological examination, standard laboratory tests, EEG, MRI. The patient also completed Somatoform Disease Questionnaire-20 items and we performed the Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR). The general and neurological exams were normal, as well as the laboratory tests. Cerebral MRI detected the slight frontal lobe atrophy and brachidolicocephaly. On EEG there were only some theta waves after the attacks. The patient corresponded to the criteria of the the conversion disorder of the DSM-IV-TR. The majority of his crises decrease after the placebo injection of 4 ml solution NaCl 0.9%. During the medical consilium, the patient suddenly had a transient spastic gait, aphonia and limb weakness which disappeared after the injection of the solution NaCl 0.9%. The PD is a very polymorphic disease and it should be differentiated from the pure conversion disorder and the mixed form, when the management needs more psychological techniques. 1.263 FUNCTIONAL OUTCOME OF DEFEROXAMINE PRIMED HUMAN ADIPOSE DERIVED MESENCHYMAL STEM CELL TRANSPLANTS IN A RAT MODEL OF HUNTINGTON’S DISEASE M.A. Edalatmanesh1 , A.R. Bahrami2 , H. Naderi2 , E. Abbasi3 , Z. Alizadeh3 , H. Nikfarjam4 . 1 Department of Physiology, Islamic Azad University, Science & Research Branch, Fars, 2 Cell & Molecular Research Center, Iranian Academic Center for Education, Culture and Research (ACER), 3 School of Medicine, Islamic Azad University, Mashhad Branch, 4 Department of Biology, Ferdowsi University of Mashhad, Mashhad, Iran Introduction: One of the biggest handicaps in stem cell transplantation is improper migration and homing of administrated cells. The chemokine receptor, CXCR4, which may play an important role in cell homing and deferoxamine could be overexpress CXCR4 in mesenchymal stem cells. We examined whether in vitro priming of human adipose derived mesenchymal stem cells (hADMSCs) with defroxamine before transplantation into the Quinolinic acid (QA) lesioned rat model of Huntington disease altered in vivo hADMSCs