P2.043 Deep brain stimulation of globus pallidus versus subthalamic nucleus for alleviating of dystonia symptoms among NBIA patients

P2.043 Deep brain stimulation of globus pallidus versus subthalamic nucleus for alleviating of dystonia symptoms among NBIA patients

Poster presentations / Parkinsonism and Related Disorders 15S2 (2009) S29–S199 PD patients tested for PINK1 and to 79 PD patients tested for Parkin m...

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Poster presentations / Parkinsonism and Related Disorders 15S2 (2009) S29–S199

PD patients tested for PINK1 and to 79 PD patients tested for Parkin mutations. UPDRS scores, medication dosages and adverse events were assessed at the clinical evaluations of the DBS patients preand postoperative. Results: We found a high frequency of patients who carried genetic mutation and underwent DBS surgery compared to the general PD population: 3/57 (5.7%) DBS patients and 6/626 (1%) total PD patients were positive for the p.G2019S - LRRK2 mutation, 4/57 (7%) DBS and 6/79 (7.6%) total PD cases had Parkin mutations and 1/57 (1.7%) DBS and 3/131 total (2.3%) patients were PINK1 mutation carrier. The clinical outcome, improvement of motor scores and the frequency of adverse events were similar in patients with mutation compared to patients without any known genetic cause. Conclusions: Patients with a genetic background represented a high frequency (14%) in our DBS population and they are likely to benefit from DBS at least as well as patients with no known genetic cause. P2.040 Gerotechnological aspects of DBS patient controllers: utility and futility I. Kaiser1 , I. Kryspin-Exner2 , F. Alesch1 . 1 Department of Neurosurgery, Medical University of Vienna, 2 Institute of Clinical Psychology, Faculty of Psychology, University of Vienna, Vienna, Austria Introduction: Deep brain stimulation (DBS) is an effective treatment for patients with movement disorders. Within the scope of DBS therapy, patients typically obtain a hand-held patient controller to monitor battery capacity and adjust stimulation parameters according to their present somatic state. While some patients find the controller easy to navigate, the majority of patients are overstrained with this technical device. Most patients with DBS are aged older than 60 years – a cohort that generally lacks confidence to use technical systems and has special needs in learning new technologies. Objective: To give an overview of ergonomic aspects of different therapy controllers with a special focus on gerotechnological issues that may arise for elderly patients. Methods: The present study compares design and usability aspects (size, display, number and color of buttons, sound, menu depth, patient interaction) of patient controllers currently commercially available: Medtronic Access® Review Controller (Soletra), Medtronic Access® Controller (Kinetra), St. Jude QuikLink™ Controller (Libra), Medtronic Activa® Patient Controller (Activa PC/RC). Conclusion: Low acceptance of DBS patient controllers causes many avoidable consultations and decreases patients’ autonomy. In general, older adults are willing to learn new technologies, but encounter certain difficulties in getting familiar with technical products. Usability problems may be related to design factors or training issues. The present study forms the initial point for future studies that include the patient’s perspective and points out possible usability flaws of DBS controllers to create new impulses for industrial design and to increase the use and acceptance in patients. P2.041 Beyond motor control: does deep brain stimulation of the subthalamic nucleus alter reward processing in patients with Parkinson’s disease? U. Lueken1 , M. Wolz2 , J. Koy3 , A. Storch2 , O. Riedel1 , H.-U. Wittchen1 , M. Dshemuchadse4 , T. Goschke4 . 1 Institute of Clinical Psychology and Psychotherapy, 2 Department of Neurology, Carl Gustav Carus University Hospital, 3 Department of Neurosurgery, Carl Gustav Carus University Hospital, 4 Department of Psychology, Technische Universit¨ at Dresden, Dresden, Germany Background and Aims: Although being an effective treatment for advanced Parkinson’s disease (PD), deep brain stimulation (DBS) of the subthalamic nucleus (STN) can cause cognitive and affective

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dysfunctions that may manifest as neuropsychiatric disturbances (e.g. pathological gambling or substance abuse). Based on findings evidencing the importance of the STN for fast-forward inhibitory control we aimed to characterize its functional profile for different aspects of reward processing. Methods: Within a postoperative design, patients were tested under two conditions (stimulation on vs. off). An assessment protocol was used encompassing affective symptoms, executive functions, and paradigms tapping on reward processing, including an inter-temporal choice task and the Iowa Gambling Task (IGT). From this ongoing study, descriptive data from a preliminary sample of n = 7 PD patients are presented. Results: Patients reported higher levels of state anxiety and negative mood when the electrodes were switched off. Executive performance appeared to be comparable for on and off states. Although results on reward processing were highly variable, patients tended to show differential discounting rates in the intertemporal choice task and gains realized in the IGT. Discussion: Preliminary results imply that acute DBS of the STN may affect reward processing. Moreover, data are in accordance with animal data suggesting that the STN may differentially affect impulsivity and reward evaluation (i.e. impulsive action vs. impulsive choice). However, due to the high inter-individual variability and limited sample size data should be interpreted with caution. Larger sample sizes are needed to analyze sources of influence that could explain additional variance. P2.042 Bilateral malfunction of cerebral electrodes at generalized dystonia patient T. Mandat1 , H. Koziara1 , T. Tykocki1 , W. Bonicki2 , P. Nauman1 . 1 IPiN, 2 Neurosurgery, CO-I, Warszawa, Poland The risk of connector damage among dystonic patients because of enormous neck movement is high. Location of cerebral electrodes minimizes the risk of damage of this part of hardware. The authors present the history of patient treated with pallidal bilateral deep brain stimulation because of generalized dystonia. Great improvement was noticed after implantation. Six months after implantation clinical status of the patient deteriorated. During evaluation of the system open circuit was identified at the right side. The patient was qualified for replacement of the connector. One week after, the clinical status of the patient progressed to deteriorate. During evaluation, open circuit was identified bilaterally. Neuroimaging studies did not reveal location of the damage. Replacement of the right connector did not cause improvement. Cerebral electrodes were replaced bilaterally. The integrity of system was verified. Clinical status of the patient improved rapidly. P2.043 Deep brain stimulation of globus pallidus versus subthalamic nucleus for alleviating of dystonia symptoms among NBIA patients T. Mandat1 , T. Kmiec2 , H. Koziara1 , T. Tykocki1 , M. Tutaj3 , R. Rola4 , W. Libionka5 , M. Bilska2 , E. Jurkiewicz6 , M. Hartig2 , H. Prokish7 , W. Bonicki8 , P. Nauman1 . 1 Neurosurgery, IPiN, 2 Neurology, CZD, Warszawa, 3 Neurology, UJ, Krakow, 4 I Neurology, IPiN, Warszawa, 5 Neurosurgery, UJ, Krakow, 6 Radiology, CZD, Warszawa, Poland; 7 Human Genetics, HC, Munich, Germany; 8 Neurosurgery, CO-I, Warszawa, Poland Treatment of Neurodegeneration with Brain Iron Accumulation (NBIA) is difficult and frequently ineffective. The authors present a group of patients with clinically and radiologically diagnosed NBIA with genetically confirmed PANK2 mutation, treated with deep brain stimulation. Materials and Methods: Five patients with confirmed PANK2 mutation (NBIA-PKAN) were treated with deep brain stimulation

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Poster presentations / Parkinsonism and Related Disorders 15S2 (2009) S29–S199

in 2008. Age of the patients varied from 8 to 24 years. The clinical condition of the patients was evaluated with various scales. At three cases the permanent electrodes were implanted to the subthalamic nuclei, and at two other cases the electrodes were implanted to the globus pallidus. The surgical procedure was undertaken under general anesthesia. The target was identified with direct and indirect method. Intrasurgical macrostimulation and microrecording were used for neurophysiological evaluation of the target. Postsurgical local field potentials were recorded in three STN cases. Results: Neither neurological deterioration nor surgical complication were noted among the group. Caregivers of the patients noted subjective improvement of the clinical state of the subjects that was confirmed with tailored scales. Conclusions: Usage of deep brain stimulation for PKAN patients carries minimal risk, effectively improves neurological status and the quality. Indication of best target point for permanent electrodes placement remains open and requires further observation on larger group of patients. P2.044 Bilateral GPi DBS for OFF dystonias and ON dyskinesias in juvenile PD Z. Mari1 , R. Von Coelln1 , P. Dash1 , L. Marsh2 , F. Lenz3 . 1 Neurology, 2 Psychiatry, 3 Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA Data supporting preference of DBS target choice in juvenile PD are lacking. In particular, correlation between disabling symptoms and choice of target (between STN and GPi) represents an underinvestigated area. Our patient was originally diagnosed with DRD at 10 years of age. He had dramatic response to levodopa, which helped him transition from being wheelchair-bound to near-normal functioning. The diagnosis was subsequently revised to juvenile PD as he developed progressively worsening signs of parkinsonism, requiring increasing quantities of levodopa. The patient is from the Middle East and no genetic etiology for his juvenile PD was identified. When he was first seen in our Center at 25 years of age, he was greatly disabled from grade 4 ON dyskinesias and severe OFF dystonias. Presurgically, the patient had virtually no ON time without dyskinesias, despite taking levodopa no less frequently than hourly during waking hours, up to 3.5 grams a day. Bilateral GPi DBS electrodes were implanted, with moderate microlesioning benefit. There was no levodopa dose reduction. Multiple programming sessions allowed stable and sustained 70–90% reduction of OFF dystonias and complete resolution of dyskinesias. The patient now spends most of the day ON without dyskinesias. Many experts consider GPi a better suited target when the most disabling symptoms include dyskinesias and dystonias. While prospective studies are needed to address site selection as a function of most disabling symptoms, our case supports the notion that GPi stimulation can work exceptionally well in reducing ON dyskinesias and OFF dystonias in juvenile PD. P2.045 Dopamine dysregulation syndrome (DDS): worse after bilateral STN DBS? Z. Mari1 , R. Von Coelln1 , J. Savitt1 , F. Lenz2 , L. Marsh3 . 1 Neurology, 2 Neurosurgery, 3 Psychiatry, Johns Hopkins University School of Medicine, Baltimore, MD, USA Levodopa daily intake is reduced after bilateral STN DBS implants in most PD patients. Therefore, most levodopa-related adverse effects, including DDS, have been reported to improve after STN DBS. However, behavior may worsen after STN DBS in some cases, including compulsive/impulsive symptoms. Data on predictors of postsurgical behavioral complications are lacking.

We present a 57-year-old PD patient with bipolar disease diagnosed before his PD was diagnosed. He had long-standing history of severe impulsive and compulsive behaviors before surgery. He also had DDS almost immediately following the commencement of levodopa therapy. Based on his narrow levodopa therapeutic window, severe motor fluctuations with wearing off in about 2 hours, dyskinesias, and no cognitive or ambulatory contraindications, he was referred for bilateral STN-DBS implants. We concluded that his pathological behavior was in great part due to excessive levodopa intake, which we expected to reduce significantly after surgery. From the motor standpoint the surgery was greatly successful with marked stimulation-related improvement of the cardinal and disabling motor symptoms of PD. Unfortunately, his self-(over)medicating behavior/DDS did not improve and both dyskinesias and impulsive/ compulsive behaviors continued. In fact, complications in both private and professional life appeared to worsen, as a result of these behaviors, including loss of job, divorce, and increased rate of psychiatry ER visits and arrests after surgery. While prospective studies are needed to help identify predictors of post-DBS behavioral complications, our experience suggests that history of psychopathology predating PD and/or particularly severe impulsive/compulsive behavior should be carefully reviewed during pre-DBS evaluations. P2.046 Neuropsychological outcome after unilateral STN DBS: a comparison of English-speakers and Spanish-speakers I.B. Marion1 , H.L. Katzen2 , C.E. Myerson1 , K. Rodriguez2 , B.V. Gallo2 , B.E. Levin1,2 . 1 Psychology, 2 Neurology, University of Miami, Miami, FL, USA Objective: Hispanics are the fastest growing minority in the US, and there is evidence that they may have a higher prevalence of Parkinson’s disease (PD) than non-Hispanic Whites, Asians and African-Americans. To date, there is no information on cognitive outcome following deep brain stimulation (DBS) among Hispanics. The current study compared primary English-speakers and primary Spanish-speakers on a battery of neuropsychological measures, preand post-DBS. Methods: Twenty-eight PD patients (15 English-speakers and 13 Spanish-speakers) underwent comprehensive cognitive testing at baseline and one-year following DBS. All patients underwent unilateral subthalamic nucleus (STN) DBS and were tested in their primary language. Results: English-speakers and Spanish-speakers differed significantly on education, F(1,27) = 8.87, p < 0.01, and dopamine agonist use, c 2 (1, N = 28) = 7.05, p < 0.01. Spanish-speakers were older, had later disease onset, and greater baseline cognitive difficulties, but these differences were not statistically significant. ANCOVA revealed that, compared to English-speakers, Spanishspeakers exhibited significantly greater decline on tests of phonemic fluency, F(4,23) = 6.56, p = 0.02, confrontation naming, F(1,27) = 5.17, p = 0.03, visuospatial orientation, F(1,27) = 5.30, p = 0.03, visuospatial integration, F(1,27) = 7.21, p = 0.01, and working memory, F(1,27) = 7.95, p = 0.01, even after controlling for significant baseline differences. Conclusion: Compared to English-speakers, Spanish-speakers showed greater decline on select neurocognitive tasks of language, visuospatial and executive function. These results suggest that Spanish-speakers may be at more risk for cognitive decline after DBS, due to greater vulnerability at baseline.