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1.279 MICROELECTRODE RECORDINGS IN THE BASAL GANGLIA AND THALAMUS IN PATIENTS WITH DYSTONIA
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Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
abnormalities are very common in WD. However, very few detailed studies are available. In this study, we made an attempt to study the types of EOM abnormalities found in the neuro-psychiatric form of WD and their possible radiological associations. Methods: EOM were studied clinically in a total of 124 patients of WD, who were successively enrolled at the Movement Disorders Clinic of Bangur Institute of Neuroscience, Kolkata. Neuro-imaging was done whenever possible. A semi-structred proforma reviewed the various types of EOM anomalies, after proper informed consent. Results: EOM was involved in 81.4% of patients; the commonest abnormality being impaired horizontal saccades (74.19%). Vertical saccadic abnormality was present in 71.77%. Down-gaze restriction was present in 25.81% and up-gaze in 39.52%. Increased latency of saccades was found in 62.90%, slow saccades in 74.19%, impersistent horizontal saccades in 25%. Of the pursuits, horizontal pursuits were involved in 66.13% and vertical in 70.97% of patients. Convergence movements were involved in 53.23%. 1 patient had oculo-motor apraxia and 2 had eyelid apraxia. An association with MRI changes was found with downgaze abnormalities (cerebellum), vertical and horizontal pursuit abnormalities (mid-brain), hypometric horizontal saccades (cerebellum) and impersistence (cerebellum and with cortical atrophy). Conclusion: EOM abnormalities are significantly associated with WD. Saccadic abnormalities are commoner than pursuits. MRI association was found with some of the abnormalities, probably corresponding to the pathway of saccadic and pursuit control. 1.276 PROSODIC DEFICITS IN MIXED DYSARTHRIA (WILSON’S DISEASE) R. Kaipa1 , R.M. Thomas2 . 1 Communication Disorders, Canteburry, Christchurch, New Zealand; 2 Speech and Hearing, Dr. M V Shetty College of Speech and Hearing, Mangalore, India Background: Wilson’s disease (Hepatolenticular Degeneration) is an autosomal recessive genetic disorder in which copper accumulates in tissues and manifests as neurological disease. Prosodic deficits are common in speakers with Wilson’s disease. Wilson’s disease is a mixed dysarthria with overlapping features of Ataxic, Spastic and Hypokinetic Dysarthria. Aims: To characterize the prosodic impairment in a patient with Wilson’s disease based on the theoretical framework: pitch, loudness, duration, voice quality, and degree of reduction. Methodology: Speech data in the form of sentence reading, passage reading, and monologue were collected from the participant. Perceptual analysis was conducted on ten prosodic parameters to evaluate five dimensions of prosody, based on a theoretical framework: pitch, loudness, duration, voice quality, and degree of reduction. Outcomes and Results: The results showed that the most severely affected prosodic parameters were low pitch, strained voice, and inappropriate stress pattern, followed by monoloudness, monopitch and prolonged interval. The other parameters that were less affected were short phrases, inappropriate silences imprecise consonants. Conclusion: The prosodic profiling of the patient with Wilson’s disease was carried out perceptually based on the theoretical framework proposed by Pfitzinger (2006). This differs from traditional definitions of prosody by including the dimensions of voice quality and degree of reduction in articulation. Since it’s a single case study the results are preliminary in nature and should be interpreted with caution. It would be interesting to extend the use of this framework to investigate a larger sample of patients with Wilson’s disease of varying degrees (mild to severe).
1.277 WILSON’S DISEASE IN THE SOUTH OF BRAZIL: A 40 YEARS FOLLOW-UP STUDY H.A.G. Teive1 , R.S. De Bem2 , D. Muzillo2 , M.M. Deguti3 , R.P. Munhoz1 , E.R. Barbosa4 . 1 Neurology Service, Internal Medicine Department, 2 Gastroenterology Service, Internal Medicine Department, Hospital de Clinicas, Federal University of Paran´ a, Curitiba, 3 Hepatology and 4 Gastroenterology Department, Neurology Department, Hospital das Cl´ınicas, S˜ ao Paulo University, S˜ ao Paulo, Brazil Introduction: Wilson’s disease (WD) is an autosomal recessive inherited disorder of the copper metabolism leading to the accumulation of this metal in different organs and tissues. Hepatic and neurological symptoms are the main clinical features of the disease. Objective: The aim of study is to analyze the clinical presentation, epidemiological features, the disease course and the response to therapy in a historical cohort of 36 Brazilian WD patients. Patients and Methods: 36 patients with WD, diagnosed from 1971 to 2010, and followed in the south of Brazil were reviewed. Diagnosis of WD was based on clinical presentation, familial history, Kayser-Fleischer rings, low serum cerulosplasmin, increased 24 h urinary copper excretion, liver biopsy, genetic test and/or response to a D-penicillamine provocative test. Results: There were 16 (44.4%) male and 20 female (56.6%) patients, with mean age of 34.6±10.8 years, with high predominance of an exclusively European continental origin (74.5%). Mean age at the initial symptom presentation was 23.2±9.3 years. The time since the first clinical symptom and the definitive diagnosis was of 27.5±41.9 months. At presentation, 9 patients (25%) showed exclusively neuropsychiatric symptoms, 14 (38.9%) exclusively hepatic symptoms, 11 (30.6%) demonstrated both hepatic and neuropsychiatric features (mixed presentation) and 2 (5.5%) were asymptomatic. Treatment used included D-penicillamine (94.2%) and zinc acetate (5.8%). Conclusion: Our cohort of WD patients has the European continental origin as a unique characteristic, which differs from previous series described in Brazil. The follow-up and response to treatment was similiar to previous studies. 1.278 WILSON’S DISEASE: UPDATE ON INTEGRATED TRADITIONAL CHINESE MEDICINAL HERBS AND WESTERN MEDICINE IN CHINA X.-P. Wang, W. Li. Depart of Neurology, Shanghai First People’s hospital, Shanghai Jiao-Tong University, Shanghai, China Wilson’s disease (WD), or hepatolenticular degeneration, is an autosomal recessive disorder of copper metabolism caused by ATP7B gene mutation. As WD is an inherited disease of the nervous system which is medicable, early diagnosis and early and life-long treatment lead to better prognoses. The best treatment recommended for Wilson’s disease currently is to integrate traditional Chinese and Western medicines. A number of studies indicate that treatment which integrates traditional Chinese medicine and Western medicine can not only enforce the decopperization effect, but also improve liver function, intelligence and other factors. This article reviews in detail the advantages of Traditional Chinese medicine and Western medicine used together in the treatment of WD. 1.279 MICROELECTRODE RECORDINGS IN THE BASAL GANGLIA AND THALAMUS IN PATIENTS WITH DYSTONIA P. Zhuang1 , M. Hallett2 , Y. Zhang1 , J. Li1 , Y. Li1 . 1 Beijing Institute of Functional Neurosurgery, Xuanwu Hospital of Capital Medical University, Beijing, China; 2 Human Motor Control Section, NINDS, NIH, Bethesda, MD, USA Background: Dystonia is characterized by sustained muscle contractions, frequently causing twisting, leading to involuntary
Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
movement or abnormal postures. An alteration in neuronal activity in the basal ganglia and thalamus may underlie dystonia. Objective: We took advantage of a series of dystonia patients who were undergoing neurosurgery for treatment to investigate neuronal activity in relation to dystonia. Patients and Methods: Microelectrode recording was performed in the GPi, the STN and the Vop/Vim in patients with dystonia (primary: n = 20; secondary: n = 15) during surgery (Lesioning or DBS). EMG was simultaneously recorded in selected muscle groups. Single-unit was detected. Interspike intervals (ISI) histograms and coefficient of variation (CV) were used to evaluate the neuronal firing pattern. Cross correlations were performed to determine neuronal activity in relation to the EMG. Results: 81% exhibited altered neuronal activity including grouped discharges in GPi (n = 156) and STN (n = 87); long-lasting neuronal activity (n = 82) and rapid neuronal firing (n = 76) in Vop/Vim. The average firing rate was 38.5 Hz; 29.8 Hz and 33.5 Hz and the mean CV was 1.38; 1.68 and 1.34 for GPi, STN and Vop/Vim, respectively. There were dystonia-related neurons in GPi (n = 36), STN (n = 28) and Vop (n = 26) showing either a burst of activity or a pause in ongoing tonic activity. Conclusion: The alteration in neuronal activity in the basal ganglia and thalamus might underlie the dystonic movement. Dystoniarelated neuronal activity observed in the basal ganglia and thalamus indicates a critical role of their interactions affecting both indirect and direct pathways in the development of dystonia. 1.280 VARIOUS TARGETS FOR DEEP BRAIN STIMULATION FOR VARIOUS TYPES OF DYSTONIA T. Mandat1 , H. Koziara2 , T. Tykocki1 , B. Krolicki1 , B. Brodacki3 , T. Kmiec4 , D. Koziorowski5 , P. Nauman1 , R. Rola6 , W. Bonicki2 . 1 Neurosurgery, IPiN, 2 Neurosurgery, Maria Sklodowska-Curie Memorial Oncology Center, 3 Neurology, Military Medical Institute, 4 Department of Child Neurology, Memorial Children’s Health Institute, 5 Neurology, Health Sciences Division, Medical University of Warsaw, 6 Neurology, Institute of Psychiatry and Neurology, Warszawa, Poland Background and purpose: Generalized dystonia (GD) can be effectively treated with pallidal deep brain stimulation (GPi DBS), or subthalamic deep brain stimulation (STN DBS). The authors present group of patients with GD, treated with GPi or STN DBS. Materials and Methods: In 2005–2009 12 female and 8 male patients with GD were treated with GPi DBS or STN DBS. Mean age at implantation was 26±6. 2 patients were diagnosed with DYT-1 mutation. 7 patients were diagnosed with PANK-2 mutation. One patient with previous bilateral pallidotomies and 6 patients with diagnosed PANK-2 mutation were qualified to STN DBS. Rest of the group was qualified to GPi DBS. Clinical status of the patients was evaluated with dystonia scales. Follow up was conducted 6 months after implantation. Results: Whole group of patients reported subjective improvement following surgery that was confirmed with tailored scales. Mean improvement was evaluated to 46%. More significant improvement was noted in the GPi group than in the STN group (51% vs 38%) The best results were achieved in the DYT-1 group (89%). Conclusions: Deep brain stimulation is effective and safe method of GD treatment. Decision which surgical target is optimal should be undertaken individually depending on clinical history, phenotype and etiology of GD. 1.281 REVERSIBLE PISA SYNDROME IN A PATIENT WITH PARKINSON’S DISEASE ON PRAMIPEXOLE 2 1 ´ Sanchez-Ferro E. De Pablo-Fernandez1 , A. ´ . Neurology, King’s 2 College Hospital, London, UK; Neurology, 12 de Octubre University Hospital, Madrid, Spain Introduction: Pisa syndrome is a dystonic lateral deviation of the trunk first reported in patients treated with neuroleptics. It has
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rarely been described in patients receiving other drugs or with neurodegenerative diseases and it has been recently associated with dopaminergic therapy in Parkinson’s disease patients. Objective: To report the development of Pisa syndrome in a patient with Parkinson’s disease on pramipexole and highlight the reversibility of the lateral trunk deviation after the early drug withdrawal. Methods: Case report. Results: A 76-year-old woman presented to our clinic 2 years ago with right-predominant motor slowness, rigidity and bradykinesia. She was diagnosed of Parkinson’s disease according to the Brain Bank criteria and was started on rasagiline after the diagnosis was made. Pramipexole was added 1 year later due to worsening of symptoms. Her relatives complained of lateral trunk deviation to the right developed 4 months ago. This abnormal posture was unnoticed by the patient and It was more evident when walking. On examination she presented a dystonic trunk deviation to the right in keeping with the diagnosis of Pisa syndrome. Pramipexole was switched to levodopa-carbidopa therapy and symptoms resolved in 4 weeks. Conclusion: Pisa syndrome may appear in patients with Parkinson’s disease as a complication of dopaminergic therapy. The early recognition of symptoms and rapid withdrawal of the priming drug may lead to the resolution of symptoms. 1.282 EFFECTIVENESS OF BOTULINUM TOXIN TYPE A IN TREATMENT OF “PISA SYNDROME”: CASE REPORT D. Sichinava, D. Sichinava, L. Astakhova, A. Ambartsumyan. Department of Neurology, General Hospital, Krasnodar, Russia Aims: To demonstrate a clinical case of Effectiveness of BTX type A in treatment of “Pizza Syndrom”. Methods: Case report: Patient S. (Male, 28 years old) with psychiatric disease was referred to the General Hospital with complaints of involuntary flexion of the body and head to right side and rotation of the trunk. The patient had been treated with peroral neuroleptics for 7 years. These symptoms had developed after 3 months of treatment with prolong-released neuroleptics. He was diagnosed as Secondary generalized-tardive dystonia: “Pisa Syndrome”. The patient was treated with BTX type A. Results: The significant effect was seen and the patient had dramatic improvement of his dystonia. “Pisa syndrome” disappeared after 1 month of injection BTX type A. The patient has been under control for more than 6 months. There were no dystonic symptoms renewal. Conclusions: BTX type A is very effective in treatment of “Pisa Syndrome” and could be recommended for treatment of tardive dystonia. 1.283 GPI AND STN DBS FOR GENERAL DYSTONIA T. Mandat1,2 , B. Brodacki3 , H. Koziara1 , W. Bonicki2 , T. Kmiec4 , D. Koziorowski5 , P. Nauman1 . 1 Neurosurgery, IPiN, 2 Neurosurgery, Maria Sklodowska-Curie Memorial Oncology Center, 3 Neurology, Military Medical Institute, 4 Department of Child Neurology, Memorial Children’s Health Institute, 5 Neurology, Health Sciences Division, Medical University of Warsaw, Warszawa, Poland Background and purpose: Deep brain stimulation (DBS) is an accepted tool of general dystonia (GD). Main anatomical target for DBS in GD remain pallidum (GPi). The authors present history of GD treatment with GPi DBS, where lack of effective control of abnormal movements forced to use additionally subthalamic nucleus (STN) DBS. Case presentation: First abnormal movements appeared at age 33 in 2008. Firstly dystonic movements included right upper limb and subsequently right lower limb dystonia, torticollis and dysphagia
Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
abnormalities are very common in WD. However, very few detailed studies are available. In this study, we made an attempt to study the types of EOM abnormalities found in the neuro-psychiatric form of WD and their possible radiological associations. Methods: EOM were studied clinically in a total of 124 patients of WD, who were successively enrolled at the Movement Disorders Clinic of Bangur Institute of Neuroscience, Kolkata. Neuro-imaging was done whenever possible. A semi-structred proforma reviewed the various types of EOM anomalies, after proper informed consent. Results: EOM was involved in 81.4% of patients; the commonest abnormality being impaired horizontal saccades (74.19%). Vertical saccadic abnormality was present in 71.77%. Down-gaze restriction was present in 25.81% and up-gaze in 39.52%. Increased latency of saccades was found in 62.90%, slow saccades in 74.19%, impersistent horizontal saccades in 25%. Of the pursuits, horizontal pursuits were involved in 66.13% and vertical in 70.97% of patients. Convergence movements were involved in 53.23%. 1 patient had oculo-motor apraxia and 2 had eyelid apraxia. An association with MRI changes was found with downgaze abnormalities (cerebellum), vertical and horizontal pursuit abnormalities (mid-brain), hypometric horizontal saccades (cerebellum) and impersistence (cerebellum and with cortical atrophy). Conclusion: EOM abnormalities are significantly associated with WD. Saccadic abnormalities are commoner than pursuits. MRI association was found with some of the abnormalities, probably corresponding to the pathway of saccadic and pursuit control. 1.276 PROSODIC DEFICITS IN MIXED DYSARTHRIA (WILSON’S DISEASE) R. Kaipa1 , R.M. Thomas2 . 1 Communication Disorders, Canteburry, Christchurch, New Zealand; 2 Speech and Hearing, Dr. M V Shetty College of Speech and Hearing, Mangalore, India Background: Wilson’s disease (Hepatolenticular Degeneration) is an autosomal recessive genetic disorder in which copper accumulates in tissues and manifests as neurological disease. Prosodic deficits are common in speakers with Wilson’s disease. Wilson’s disease is a mixed dysarthria with overlapping features of Ataxic, Spastic and Hypokinetic Dysarthria. Aims: To characterize the prosodic impairment in a patient with Wilson’s disease based on the theoretical framework: pitch, loudness, duration, voice quality, and degree of reduction. Methodology: Speech data in the form of sentence reading, passage reading, and monologue were collected from the participant. Perceptual analysis was conducted on ten prosodic parameters to evaluate five dimensions of prosody, based on a theoretical framework: pitch, loudness, duration, voice quality, and degree of reduction. Outcomes and Results: The results showed that the most severely affected prosodic parameters were low pitch, strained voice, and inappropriate stress pattern, followed by monoloudness, monopitch and prolonged interval. The other parameters that were less affected were short phrases, inappropriate silences imprecise consonants. Conclusion: The prosodic profiling of the patient with Wilson’s disease was carried out perceptually based on the theoretical framework proposed by Pfitzinger (2006). This differs from traditional definitions of prosody by including the dimensions of voice quality and degree of reduction in articulation. Since it’s a single case study the results are preliminary in nature and should be interpreted with caution. It would be interesting to extend the use of this framework to investigate a larger sample of patients with Wilson’s disease of varying degrees (mild to severe).
1.277 WILSON’S DISEASE IN THE SOUTH OF BRAZIL: A 40 YEARS FOLLOW-UP STUDY H.A.G. Teive1 , R.S. De Bem2 , D. Muzillo2 , M.M. Deguti3 , R.P. Munhoz1 , E.R. Barbosa4 . 1 Neurology Service, Internal Medicine Department, 2 Gastroenterology Service, Internal Medicine Department, Hospital de Clinicas, Federal University of Paran´ a, Curitiba, 3 Hepatology and 4 Gastroenterology Department, Neurology Department, Hospital das Cl´ınicas, S˜ ao Paulo University, S˜ ao Paulo, Brazil Introduction: Wilson’s disease (WD) is an autosomal recessive inherited disorder of the copper metabolism leading to the accumulation of this metal in different organs and tissues. Hepatic and neurological symptoms are the main clinical features of the disease. Objective: The aim of study is to analyze the clinical presentation, epidemiological features, the disease course and the response to therapy in a historical cohort of 36 Brazilian WD patients. Patients and Methods: 36 patients with WD, diagnosed from 1971 to 2010, and followed in the south of Brazil were reviewed. Diagnosis of WD was based on clinical presentation, familial history, Kayser-Fleischer rings, low serum cerulosplasmin, increased 24 h urinary copper excretion, liver biopsy, genetic test and/or response to a D-penicillamine provocative test. Results: There were 16 (44.4%) male and 20 female (56.6%) patients, with mean age of 34.6±10.8 years, with high predominance of an exclusively European continental origin (74.5%). Mean age at the initial symptom presentation was 23.2±9.3 years. The time since the first clinical symptom and the definitive diagnosis was of 27.5±41.9 months. At presentation, 9 patients (25%) showed exclusively neuropsychiatric symptoms, 14 (38.9%) exclusively hepatic symptoms, 11 (30.6%) demonstrated both hepatic and neuropsychiatric features (mixed presentation) and 2 (5.5%) were asymptomatic. Treatment used included D-penicillamine (94.2%) and zinc acetate (5.8%). Conclusion: Our cohort of WD patients has the European continental origin as a unique characteristic, which differs from previous series described in Brazil. The follow-up and response to treatment was similiar to previous studies. 1.278 WILSON’S DISEASE: UPDATE ON INTEGRATED TRADITIONAL CHINESE MEDICINAL HERBS AND WESTERN MEDICINE IN CHINA X.-P. Wang, W. Li. Depart of Neurology, Shanghai First People’s hospital, Shanghai Jiao-Tong University, Shanghai, China Wilson’s disease (WD), or hepatolenticular degeneration, is an autosomal recessive disorder of copper metabolism caused by ATP7B gene mutation. As WD is an inherited disease of the nervous system which is medicable, early diagnosis and early and life-long treatment lead to better prognoses. The best treatment recommended for Wilson’s disease currently is to integrate traditional Chinese and Western medicines. A number of studies indicate that treatment which integrates traditional Chinese medicine and Western medicine can not only enforce the decopperization effect, but also improve liver function, intelligence and other factors. This article reviews in detail the advantages of Traditional Chinese medicine and Western medicine used together in the treatment of WD. 1.279 MICROELECTRODE RECORDINGS IN THE BASAL GANGLIA AND THALAMUS IN PATIENTS WITH DYSTONIA P. Zhuang1 , M. Hallett2 , Y. Zhang1 , J. Li1 , Y. Li1 . 1 Beijing Institute of Functional Neurosurgery, Xuanwu Hospital of Capital Medical University, Beijing, China; 2 Human Motor Control Section, NINDS, NIH, Bethesda, MD, USA Background: Dystonia is characterized by sustained muscle contractions, frequently causing twisting, leading to involuntary
Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
movement or abnormal postures. An alteration in neuronal activity in the basal ganglia and thalamus may underlie dystonia. Objective: We took advantage of a series of dystonia patients who were undergoing neurosurgery for treatment to investigate neuronal activity in relation to dystonia. Patients and Methods: Microelectrode recording was performed in the GPi, the STN and the Vop/Vim in patients with dystonia (primary: n = 20; secondary: n = 15) during surgery (Lesioning or DBS). EMG was simultaneously recorded in selected muscle groups. Single-unit was detected. Interspike intervals (ISI) histograms and coefficient of variation (CV) were used to evaluate the neuronal firing pattern. Cross correlations were performed to determine neuronal activity in relation to the EMG. Results: 81% exhibited altered neuronal activity including grouped discharges in GPi (n = 156) and STN (n = 87); long-lasting neuronal activity (n = 82) and rapid neuronal firing (n = 76) in Vop/Vim. The average firing rate was 38.5 Hz; 29.8 Hz and 33.5 Hz and the mean CV was 1.38; 1.68 and 1.34 for GPi, STN and Vop/Vim, respectively. There were dystonia-related neurons in GPi (n = 36), STN (n = 28) and Vop (n = 26) showing either a burst of activity or a pause in ongoing tonic activity. Conclusion: The alteration in neuronal activity in the basal ganglia and thalamus might underlie the dystonic movement. Dystoniarelated neuronal activity observed in the basal ganglia and thalamus indicates a critical role of their interactions affecting both indirect and direct pathways in the development of dystonia. 1.280 VARIOUS TARGETS FOR DEEP BRAIN STIMULATION FOR VARIOUS TYPES OF DYSTONIA T. Mandat1 , H. Koziara2 , T. Tykocki1 , B. Krolicki1 , B. Brodacki3 , T. Kmiec4 , D. Koziorowski5 , P. Nauman1 , R. Rola6 , W. Bonicki2 . 1 Neurosurgery, IPiN, 2 Neurosurgery, Maria Sklodowska-Curie Memorial Oncology Center, 3 Neurology, Military Medical Institute, 4 Department of Child Neurology, Memorial Children’s Health Institute, 5 Neurology, Health Sciences Division, Medical University of Warsaw, 6 Neurology, Institute of Psychiatry and Neurology, Warszawa, Poland Background and purpose: Generalized dystonia (GD) can be effectively treated with pallidal deep brain stimulation (GPi DBS), or subthalamic deep brain stimulation (STN DBS). The authors present group of patients with GD, treated with GPi or STN DBS. Materials and Methods: In 2005–2009 12 female and 8 male patients with GD were treated with GPi DBS or STN DBS. Mean age at implantation was 26±6. 2 patients were diagnosed with DYT-1 mutation. 7 patients were diagnosed with PANK-2 mutation. One patient with previous bilateral pallidotomies and 6 patients with diagnosed PANK-2 mutation were qualified to STN DBS. Rest of the group was qualified to GPi DBS. Clinical status of the patients was evaluated with dystonia scales. Follow up was conducted 6 months after implantation. Results: Whole group of patients reported subjective improvement following surgery that was confirmed with tailored scales. Mean improvement was evaluated to 46%. More significant improvement was noted in the GPi group than in the STN group (51% vs 38%) The best results were achieved in the DYT-1 group (89%). Conclusions: Deep brain stimulation is effective and safe method of GD treatment. Decision which surgical target is optimal should be undertaken individually depending on clinical history, phenotype and etiology of GD. 1.281 REVERSIBLE PISA SYNDROME IN A PATIENT WITH PARKINSON’S DISEASE ON PRAMIPEXOLE 2 1 ´ Sanchez-Ferro E. De Pablo-Fernandez1 , A. ´ . Neurology, King’s 2 College Hospital, London, UK; Neurology, 12 de Octubre University Hospital, Madrid, Spain Introduction: Pisa syndrome is a dystonic lateral deviation of the trunk first reported in patients treated with neuroleptics. It has
S67
rarely been described in patients receiving other drugs or with neurodegenerative diseases and it has been recently associated with dopaminergic therapy in Parkinson’s disease patients. Objective: To report the development of Pisa syndrome in a patient with Parkinson’s disease on pramipexole and highlight the reversibility of the lateral trunk deviation after the early drug withdrawal. Methods: Case report. Results: A 76-year-old woman presented to our clinic 2 years ago with right-predominant motor slowness, rigidity and bradykinesia. She was diagnosed of Parkinson’s disease according to the Brain Bank criteria and was started on rasagiline after the diagnosis was made. Pramipexole was added 1 year later due to worsening of symptoms. Her relatives complained of lateral trunk deviation to the right developed 4 months ago. This abnormal posture was unnoticed by the patient and It was more evident when walking. On examination she presented a dystonic trunk deviation to the right in keeping with the diagnosis of Pisa syndrome. Pramipexole was switched to levodopa-carbidopa therapy and symptoms resolved in 4 weeks. Conclusion: Pisa syndrome may appear in patients with Parkinson’s disease as a complication of dopaminergic therapy. The early recognition of symptoms and rapid withdrawal of the priming drug may lead to the resolution of symptoms. 1.282 EFFECTIVENESS OF BOTULINUM TOXIN TYPE A IN TREATMENT OF “PISA SYNDROME”: CASE REPORT D. Sichinava, D. Sichinava, L. Astakhova, A. Ambartsumyan. Department of Neurology, General Hospital, Krasnodar, Russia Aims: To demonstrate a clinical case of Effectiveness of BTX type A in treatment of “Pizza Syndrom”. Methods: Case report: Patient S. (Male, 28 years old) with psychiatric disease was referred to the General Hospital with complaints of involuntary flexion of the body and head to right side and rotation of the trunk. The patient had been treated with peroral neuroleptics for 7 years. These symptoms had developed after 3 months of treatment with prolong-released neuroleptics. He was diagnosed as Secondary generalized-tardive dystonia: “Pisa Syndrome”. The patient was treated with BTX type A. Results: The significant effect was seen and the patient had dramatic improvement of his dystonia. “Pisa syndrome” disappeared after 1 month of injection BTX type A. The patient has been under control for more than 6 months. There were no dystonic symptoms renewal. Conclusions: BTX type A is very effective in treatment of “Pisa Syndrome” and could be recommended for treatment of tardive dystonia. 1.283 GPI AND STN DBS FOR GENERAL DYSTONIA T. Mandat1,2 , B. Brodacki3 , H. Koziara1 , W. Bonicki2 , T. Kmiec4 , D. Koziorowski5 , P. Nauman1 . 1 Neurosurgery, IPiN, 2 Neurosurgery, Maria Sklodowska-Curie Memorial Oncology Center, 3 Neurology, Military Medical Institute, 4 Department of Child Neurology, Memorial Children’s Health Institute, 5 Neurology, Health Sciences Division, Medical University of Warsaw, Warszawa, Poland Background and purpose: Deep brain stimulation (DBS) is an accepted tool of general dystonia (GD). Main anatomical target for DBS in GD remain pallidum (GPi). The authors present history of GD treatment with GPi DBS, where lack of effective control of abnormal movements forced to use additionally subthalamic nucleus (STN) DBS. Case presentation: First abnormal movements appeared at age 33 in 2008. Firstly dystonic movements included right upper limb and subsequently right lower limb dystonia, torticollis and dysphagia