Cervical Dystonia Research Articles

Social Cognition Deficits in Cervical Dystonia, but not Blepharospasm: Focal Dystonias Diverge.

Movement Disorders Clinical PracticeAccepted Articles LETTERS: NEW OBSERVATION Social Cognition Deficits in Cervical Dystonia, but not Blepharospasm: Focal Dystonias Diverge Jeanne Feuerstein MD, Corresponding Author Jeanne Feuerstein MD [email protected] orcid.org/0000-0002-7701-3120 Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USA Department of Neurology, Rocky Mountain Regional VA Medical Center, Aurora, Colorado, USA Correspondence to: Jeanne Feuerstein, MD, Address: 12631 E. 17 Ave Room 5508, Aurora CO 80045 | Mail Stop B185, Phone: (720) 848-2080, E-mail: [email protected]Search for more papers by this authorBrooke Heffernan MD, Brooke Heffernan MD Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USASearch for more papers by this authorSamantha K. Holden MD, MS, Samantha K. Holden MD, MS Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USASearch for more papers by this authorStefan Sillau PhD, Stefan Sillau PhD Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USASearch for more papers by this authorBrian D. Berman MD, MS, Brian D. Berman MD, MS orcid.org/0000-0002-0602-9942 Department of Neurology, Virginia Commonwealth University, Richmond, Virginia, USASearch for more papers by this author Jeanne Feuerstein MD, Corresponding Author Jeanne Feuerstein MD [email protected] orcid.org/0000-0002-7701-3120 Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USA Department of Neurology, Rocky Mountain Regional VA Medical Center, Aurora, Colorado, USA Correspondence to: Jeanne Feuerstein, MD, Address: 12631 E. 17 Ave Room 5508, Aurora CO 80045 | Mail Stop B185, Phone: (720) 848-2080, E-mail: [email protected]Search for more papers by this authorBrooke Heffernan MD, Brooke Heffernan MD Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USASearch for more papers by this authorSamantha K. Holden MD, MS, Samantha K. Holden MD, MS Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USASearch for more papers by this authorStefan Sillau PhD, Stefan Sillau PhD Department of Neurology, University of Colorado School of Medicine, Aurora, Colorado, USASearch for more papers by this authorBrian D. Berman MD, MS, Brian D. Berman MD, MS orcid.org/0000-0002-0602-9942 Department of Neurology, Virginia Commonwealth University, Richmond, Virginia, USASearch for more papers by this author First published: 07 June 2023 https://doi.org/10.1002/mdc3.13808 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1002/mdc3.13808. AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future. RelatedInformation

Improving the Efficacy of Botulinum Toxin for Cervical Dystonia: A Scoping Review.

Cervical dstonia (CD) is a chronic disorder with a significant detrimental impact on quality of life, requiring long-term treatment. Intramuscular injections of botulinum neurotoxin (BoNT) every 12 to 16 weeks have become the first-line option for CD. Despite the remarkable efficacy of BoNT as a treatment for CD, a significantly high proportion of patients report poor outcomes and discontinue the treatment. The reasons that drive sub-optimal response or treatment failure in a proportion of patients include but are not limited to inappropriate muscle targets and/or BoNT dosing, improper method of injections, subjective feeling of inefficacy, and the formation of neutralizing antibodies against the neurotoxin. The current review aims to complement published research focusing on the identification of the factors that might explain the failure of BoNT treatment in CD, highlighting possible solutions to improve its outcomes. Thus, the use of the new phenomenological classification of cervical dystonia known as COL-CAP might improve the identification of the muscle targets, but more sensitive information might come from the use of kinematic or scintigraphic techniques and the use of electromyographic or ultrasound guidance might ensure the accuracy of the injections. Suggestions are made for the development of a patient-centered model for the management of cervical dystonia and to emphasize that unmet needs in the field are to increase awareness about the non-motor spectrum of CD, which might influence the perception of the efficacy from BoNT injections, and the development of dedicated rehabilitation programs for CD that might enhance its effectiveness.

Anatomical categorization of isolated non-focal dystonia: novel and existing patterns using a data-driven approach.

According to expert consensus, dystonia can be classified as focal, segmental, multifocal, and generalized, based on the affected body distribution. To provide an empirical and data-driven approach to categorizing these distributions, we used a data-driven clustering approach to compare frequency and co-occurrence rates of non-focal dystonia in pre-defined body regions using the Dystonia Coalition (DC) dataset. We analyzed 1,618 participants with isolated non-focal dystonia from the DC database. The analytic approach included construction of frequency tables, variable-wise analysis using hierarchical clustering and independent component analysis (ICA), and case-wise consensus hierarchical clustering to describe associations and clusters for dystonia affecting any combination of eighteen pre-defined body regions. Variable-wise hierarchical clustering demonstrated closest relationships between bilateral upper legs (distance = 0.40), upper and lower face (distance = 0.45), bilateral hands (distance = 0.53), and bilateral feet (distance = 0.53). ICA demonstrated clear grouping for the a) bilateral hands, b) neck, and c) upper and lower face. Case-wise consensus hierarchical clustering at k = 9 identified 3 major clusters. Major clusters consisted primarily of a) cervical dystonia with nearby regions, b) bilateral hand dystonia, and c) cranial dystonia. Our data-driven approach in a large dataset of isolated non-focal dystonia reinforces common segmental patterns in cranial and cervical regions. We observed unexpectedly strong associations between bilateral upper or lower limbs, which suggests that symmetric multifocal patterns may represent a previously underrecognized dystonia subtype.

Reversible Bilateral Basal Ganglia and Brainstem Lesions in Neuropsychiatric Systemic Lupus Erythematosus: A Case Report

<p>The pathophysiology behind neuropsychiatric SLE (NPSLE) remains poorly understood and its clinical and radiological manifestations are highly varied. In this report, we present a complex case of an adolescent female patient with a three-week history of systemic symptoms (fever, nausea, vomiting, weight-loss, polyarticular joint pain), progressive motor weakness, tremor, and altered mental status. Physical examination was significant for oromandibular and cervical dystonia rigidity, and general weakness with imposed right-sided hemiparesis. A head MRI demonstrated bilateral hyperintense lesions of the basal ganglia and brainstem, SS without restricted diffusion. The patient was diagnosed with NPSLE, lupus nephritis, electrolyte imbalance, severe hypoalbuminemia, lupus cardiomyopathy, autoimmune hemolytic anemia, pulmonary tuberculosis, and sepsis. The patient was given treatment in the ICU with pulse dose corticosteroids, intravenous antibiotics, intravenous immunoglobulins (IVIg), and supportive treatment with correction of hematologic and electrolyte abnormalities. Her condition improved rapidly. Full alertness was regained, and symptoms of oromandibular dystonia, tremor, and weakness diminished significantly. A follow-up MRI three weeks later revealed complete disappearance of lesions, which we attribute to resolution of the inflammatory process in the brain.</p>

P.023 The Pain in Dystonia Scale (PIDS): development and validation

Background: Pain in a common symptom in adult-onset idiopathic dystonia (AOID). An appropriate tool to understand this symptom is needed to improve AOID patients’ care. We developed a rating instrument for pain in AOID and validated it in cervical dystonia (CD). Methods: Development and validation of the Pain in Dystonia Scale (PIDS) in three phases: 1. International experts and participants generated and evaluated the preliminary items for content validity; 2. The PIDS was drafted and revised, followed by cognitive interviews to ensure suitability for self-administration; and 3. the clinimetric properties of the final PIDS were assessed in 85 participants. Results: PIDS evaluates pain severity (by body part), functional impact and external modulating factors. It showed high test-retest reliability the total score (0.9, p<0.001), intraclass correlation coefficients higher than 0.7 for all items and high internal consistency (Cronbach’s alpha 0.9). Convergent validity analysis revealed a strong correlation between the PIDS severity score and the TWSTRS pain subscale (0.8, p<0.001), the brief pain inventory short form (0.7, p<0.001) and impact of pain on daily functioning (0.7, p<0.001). Conclusions: The PIDS is the first specific questionnaire developed to evaluate pain in patients with AOID with high-level clinimetric properties in people with CD.

The Pain in Dystonia Scale (PIDS)-Development and Validation in Cervical Dystonia.

A better understanding of pain in adult-onset idiopathic dystonia (AOID) is needed to implement effective therapeutic strategies. To develop a new rating instrument for pain in AOID and validate it in cervical dystonia (CD). Development and validation of the Pain in Dystonia Scale (PIDS) comprised three phases. In phase 1, international experts and participants with AOID generated and evaluated the preliminary items for content validity. In phase 2, the PIDS was drafted and revised by the experts, followed by cognitive interviews to ensure self-administration suitability. In phase 3, the PIDS psychometric properties were assessed in 85 participants with CD and retested in 40 participants. The final version of PIDS evaluates pain severity (by body-part), functional impact, and external modulating factors. Test-retest reliability showed a high-correlation coefficient for the total score (0.9, P < 0.001), and intraclass correlation coefficients were 0.7 or higher for all items in all body-parts subscores. The overall PIDS severity score showed high internal consistency (Cronbach's α, 0.9). Convergent validity analysis revealed a strong correlation between the PIDS severity score and the Toronto Western Spasmodic Torticollis Rating Scale pain subscale (0.8, P < 0.001) and the Brief Pain Inventory-short form items related to pain at time of the assessment (0.7, P < 0.001) and impact of pain on daily functioning (0.7, P < 0.001). The PIDS is the first specific questionnaire developed to evaluate pain in all patients with AOID, here, demonstrating high-level psychometric properties in people with CD. Future work will validate PIDS in other forms of AOID. © 2023 International Parkinson and Movement Disorder Society.

Grey matter correlates of dystonic soft signs in essential tremor

BackgroundQuestionable signs of dystonia are a common finding in patients with essential tremor (ET). Brain structural alterations in ET patients plus dystonic soft signs (ET + ds) in comparison to ET patients without dystonic soft signs (ET-ds) or patients with tremor associated with manifest dystonia (TAWD) have not been examined yet. Therefore, our study aims to explore alterations of brain grey matter in patients with ET + ds. MethodsA total of 68 elderly patients with ET-ds (n = 32), ET + ds (n = 20) or idiopathic cervical dystonia with dystonia associated action tremor of the upper limbs (TAWD, n = 16) and 42 age-matched healthy controls underwent a clinical and electrophysiological assessment and 3T MRI. For grey matter alterations T1 MRI images were analysed by voxel-based morphometry. Additionally, regression analyses with clinical parameters (tremor frequency, severity and disease duration) were performed. ResultsVBM showed a significant increase of grey matter in the right lentiform nucleus in ET + ds and TAWD compared to HC and ET-ds. Further, an increase of cortical grey matter in the middle frontal gyrus in ET + ds was shown. The hypertrophy of the lentiform nucleus in ET + ds was correlated with disease severity and duration. ConclusionPatients with ET + ds showed grey matter brain structural alterations similar to TAWD. Our findings suggest an involvement of the basal ganglia-cortical loop in ET + ds which may indicate a pathophysiological similarity with TAWD rather than ET.

Pooled Safety Analysis of IncobotulinumtoxinA in the Treatment of Neurological Disorders in Adults

The pooled incidences of treatment-emergent adverse events (TEAEs) were examined by indication using the integrated clinical database of Merz-sponsored, placebo-controlled, or repeat-dose studies of incobotulinumtoxinA in adults with cervical dystonia, blepharospasm, limb spasticity, sialorrhea, or essential tremor of the upper limb. Overall incidences of TEAEs, serious TEAEs, TEAEs leading to discontinuation, fatal TEAEs, TEAEs of special interest (TEAESIs; indicating possible toxin spread), and treatment-related (TR) events were determined for incobotulinumtoxinA and placebo after a single injection and for repeated dose cycles of incobotulinumtoxinA. The most frequent events after a single dose of incobotulinumtoxinA are summarized. After a single cycle, incidences of overall TEAEs were similar between incobotulinumtoxinA and the placebo in most indications, although between-indication differences were observed. Few TEAEs led to incobotulinumtoxinA discontinuation; there were no fatal TEAEs with incobotulinumtoxinA. In general, repeated cycles did not increase the incidence of any event. The most frequent TR-TEAEs were indication-dependent, including dysphagia for indications affecting the head or neck. The TR-TEAESIs across all indications were most commonly muscular weakness, dysphagia and dry mouth. Overall, the results of this pooled analysis support and extend the favorable safety and tolerability profile of incobotulinumtoxinA for the treatment of adult neurological disorders established by individual clinical studies.

Pain Reduction in Cervical Dystonia Following Treatment with IncobotulinumtoxinA: A Pooled Analysis.

This analysis pooled pain severity data from four phase 3 and 4 studies of incobotulinumtoxinA (incoBoNT-A) for the treatment of cervical dystonia (CD) in adults. CD-related pain severity was assessed at baseline, each injection visit, and 4 weeks after each injection of incoBoNT-A using the Toronto Western Spasmodic Torticollis Rating Scale pain severity subscale or a pain visual analog scale. Both were analyzed using a score range of 0-10 and pain was categorized as mild, moderate, or severe. Data for 678 patients with pain at baseline were assessed and sensitivity analyses evaluated pain responses in the subgroup not taking concomitant pain medication (n = 384 at baseline). At Week 4 after the first injection, there was a mean change of -1.25 (standard deviation 2.04) points from baseline pain severity (p < 0.0001), with 48.1% showing ≥ 30% pain reduction from baseline, 34.4% showing ≥50% pain reduction from baseline, and 10.3% becoming pain free. Pain responses were sustained over five injection cycles with a trend to incremental improvements with each successive cycle. Pain responses in the subgroup not taking concomitant pain medication demonstrated the lack of confounding effects of pain medications. These results confirmed the pain relief benefits of long-term treatment with incoBoNT-A.

Fixel-Based Analysis Reveals Whole-Brain White Matter Abnormalities in Cervical Dystonia.

Cervical dystonia (CD) is a form of isolated focal dystonia typically associated to abnormal head, neck, and shoulder movements and postures. The complexity of the clinical presentation limits the investigation of its pathophysiological mechanisms, and the neural networks associated to specific motor manifestations are still the object of debate. We investigated the morphometric properties of white matter fibers in CD and explored the networks associated with motor symptoms, while regressing out nonmotor scores. Nineteen patients affected by CD and 21 healthy controls underwent diffusion-weighted magnetic resonance imaging. We performed fixel-based analysis, a novel method evaluating fiber orientation within specific fiber bundles, and compared fiber morphometric properties between groups. Moreover, we correlated fiber morphometry with the severity of motor symptoms in patients. Compared to controls, patients exhibited decreased white matter fibers in the right striatum. Motor symptom severity negatively correlated with white matter fibers passing through inferior parietal areas and the head representation area of the motor cortex. Abnormal white matter integrity at the basal ganglia level may affect several functional networks involved, for instance, in motor preparation and execution, visuomotor coordination, and multimodal integration. This may result in progressive maladaptive plasticity, culminating in overt symptoms of dystonia. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Botulinum toxin type A infiltration in spasticity and cervical dystonia. Muscle morphology: an overlooked factor

IntroductionBotulinum toxin type A is used to treat spasticity and dystonia. However, its relationship with muscle morphology has not been studied. The action mechanism of botulinum toxin is based on the inhibition of acetylcholine release. Therefore, larger doses of toxin would be needed to treat larger muscles. This study aims to establish whether there is a discrepancy between muscle morphology and the botulinum toxin doses administered. MethodsWe dissected, and subsequently measured and weighed, muscles from the upper and lower limbs and the head of a fresh cadaver. We consulted the summary of product characteristics for botulinum toxin type A to establish the recommended doses for each muscle and calculated the number of units infiltrated per gramme of muscle. ResultsDifferent muscles present considerable morphological variability, and the doses of botulinum toxin administered to each muscle are very similar. We observed great variability in the amount of botulinum toxin administered per gramme of muscle, ranging from 0.3 U/g in the biceps femoris to 14.6 U/g in the scalene muscles. The mean dose was 2.55 U/g. The doses administered for nearly all lower limb muscles were below this value. ConclusionsThere are significant differences in morphology between the muscles of the lower limbs, upper limbs, and head, but similar doses of botulinum toxin are administered to each muscle. These differences result in great variability in the number of units of botulinum toxin administered per gramme of muscle.

Improvement in Cervical Dystonia – Outcomes of a Prospective, Multicenter, International Registry of Deep Brain Stimulation (DBS) (P11-11.002)

<h3>Objective:</h3> To report real-world outcomes of Dystonia patients implanted with a Multiple Independent Current Control (MICC)-based Directional DBS system. <h3>Background:</h3> Management of dystonia using Deep Brain Stimulation (DBS) is a well-established therapeutic approach. However, optimal DBS target sites in patients with cervical (focal) versus generalized dystonia are thought to diverge and be specific for particular connections (Horn 2022). DBS devices equipped with capabilities such as directionality and Multiple Independent Current Control (MICC) may offer potential for improved clinical outcomes. Here, we report a sub-analysis of patients with cervical dystonia only or dystonia with cervical involvement from an on-going, multicenter registry. <h3>Design/Methods:</h3> In this prospective, on-label, multi-center, international registry (ClinicalTrials.gov Identifier: NCT02686125) of dystonia, enrolled subjects received MICC-based directional DBS systems (Vercise, Boston Scientific). Subjects were followed up to 3 years (post-implant). Several study assessments evaluating their dystonia symptoms (e.g., TWSTRS), quality of life (GIC) and overall satisfaction were administered. Adverse Events were also collected. <h3>Results:</h3> A total of 43-patients (mean age 56.9-years, 58% females) with focal (cervical) dystonia only and 83-patients (mean 41.95-years, 61% females) with generalized or segmental dystonia with cervical involvement who underwent pallidal DBS were prospectively evaluated. Both groups reported significant improvement in overall TWSTRS scores – however the extent varied. In the cervical only cohort, a 19.9-point improvement was noted at 6-months (n=25) and sustained up to 1-year (23.2-point improvement, n=20). In those with generalized or segmental dystonia (with cervical involvement), a 9.7-point. and 7.3-improvement in overall TWSTRS scores was noted at 6- (n = 50) and 12-months (n = 38), respectively. <h3>Conclusions:</h3> This registry represents the first comprehensive, large-scale collection of real-world outcomes associated with dystonia patients implanted with a directional DBS system capable of MICC. Preliminary results demonstrate significant improvement in patients with cervical dystonia (either alone or with other regions involved) following DBS. <b>Disclosure:</b> Dr. Albanese has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Boston Scientific. Dr. Albanese has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Frontiers Publishing Group. Dr. Albanese has a non-compensated relationship as a President-Elect with International Association on Parkinsonism and Related Disorders that is relevant to AAN interests or activities. Roshini Jain has received personal compensation for serving as an employee of Boston Scientific. Roshini Jain has received stock or an ownership interest from Boston Scientific. Lilly Chen has received personal compensation for serving as an employee of Boston Scientific . Joachim Krauss has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Inomed. Joachim Krauss has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for aleva. Joachim Krauss has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Boston.

Cervical Dystonia in a Headache Population: General Medical Education (GME) Training to Standardize Screening (P11-12.005)

<h3>Objective:</h3> To highlight the importance of GME training on screening for cervical dystonia in patients presenting for headaches or cervicalgia. <h3>Background:</h3> Cervical dystonia (CD) is a condition of involuntary overactivation of the cervical musculature resulting in abnormal head posture. Notably, comorbid headache in CD is present in up to 75% of patients. Although the prevalence of CD is reported to be quite low, underdiagnosis or misdiagnosis is common. This is likely due to the challenging physical diagnosis often requiring a movement disorder specialist, and the lack of focused training in neurology residency programs. Although CD can improve with Botulinum toxin (BT), greater than 50% of patients remain untreated. <h3>Design/Methods:</h3> From 2017–2020, an IRB-reviewed Quality Improvement Project was initiated by a Movement Disorder Neurologist in a GME-Residency program. Chart review was performed on all patients presenting for headache, migraine, or cervicalgia, and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was performed to assess for comorbid cervical dystonia. <h3>Results:</h3> During this timeframe, 6455 patients presented for headache, migraine, cervical dystonia, or cervicalgia. 306 patients were diagnosed with both cervicalgia and headache. Of those patients, 5% were ultimately diagnosed with cervical dystonia. All diagnoses of cervical dystonia were made by a Movement Disorder Neurologist or resident trained in this screening approach. All patients diagnosed with cervical dystonia had chronic headaches, and most had daily headaches. Of patients diagnosed with cervical dystonia, 86% were started on treatment with BT, with a 91% success rate. <h3>Conclusions:</h3> Patients with cervical dystonia commonly present to Neurology for headache or migraine, as comorbid pain is frequent. Underdiagnosis of dystonia may lead to suboptimal treatment choices. Formalized training of residents to use TWSTRS on patients with headache and cervicalgia may enhance diagnosis and improve treatment outcomes. <b>Disclosure:</b> Dr. Inoue has nothing to disclose. Dr. Dalton has nothing to disclose. Dr. Hack has nothing to disclose. Dr. Baker has nothing to disclose.

Characteristics and Treatment Response to OnabotulinumtoxinA of Patients From CD-PROBE With Anterocollis and Retrocollis (P12-11.005)

<h3>Objective:</h3> To describes baseline characteristics and treatment response of these cervical dystonia (CD) subtypes. <h3>Background:</h3> CD-PROBE included patients with anterocollis (AC) and retrocollis (RC) treated with onabotulinumtoxinA (onabotA). <h3>Design/Methods:</h3> CD PROBE was an observational, prospective study designed to identify real-world outcomes in patients with CD after onabotA treatment; patients from 82 US sites with CD diagnosis who were candidates for onabotA therapy, new to treatment, or not previously treated in a clinical trial in ≥16 weeks, and completed 3 onabotA treatments. Treatment benefit was measured using the Patient’s Global Impression of Change (PGI-C), Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Cervical Dystonia Impact Profile (CDIP-58), Clinician’s Global Impression of Change (CGI-C), and physician assessments of CD severity. <h3>Results:</h3> Patients with AC (n=59) and RC (n=55) were older and more disabled, with a shorter time to diagnosis and treatment, than patients with laterocollis (LC) or torticollis (TC). From PGI-C responses, 58.4% of RC vs 47.8% of AC patients reported being much/very much improved by onabotA post-injection 3 (final visit). CGI-C responses similarly improved over this timeframe; 82.6% of RC and 60.9% of AC patients reporting much/very much improved. The mean total TWSTRS score decreased for AC and RC patients from injection 1 (46 [AC]; 40 [RC]) to final visit (36 [AC]; 32 [RC]); CDIP-58 scores also decreased over time. The proportion of patients with physician-assessed severe CD decreased from injection 1 (28.8% [AC]; 21.8% [RC]) to final visit (21.7% [AC]; 4.3% [RC]); most patients shifted to lower symptom severity. OnabotA dose generally increased per visit, with AC patients having lowest doses (153.5–195.4U) and RC patients the highest (184.0–213.4U). Adverse event of dysphagia reported in 4 AC and 7 RC patients. No new safety signals were identified. <h3>Conclusions:</h3> CD PROBE data indicates real-world treatment with onabotA relieves CD symptoms in patients with anterocollis and retrocollis. <b>Disclosure:</b> Dr. Dashtipour has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Amneal, Abbvie, Acorda, Acadia, Ipsen, Supernus, Sunovion, Neurocrine, Teva. Dr. Dashtipour has received personal compensation in the range of $100,000-$499,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amneal, Abbvie, Acadia, Supernus. Dr. Dashtipour has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for Amneal, Acorda, Abbvie, Ipsen, Teva, Neurocrine, SupernusPD, sunovion . Dr. Jankovic has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Revance. Dr. Jankovic has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Revance, Allergan. The institution of Dr. Jankovic has received research support from Baylor College of Medicine. Dr. Jankovic has received research support from Abbvie. The institution of Dr. Jankovic has received research support from Abbvie. Dr. Charles has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Supernus. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Revance. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merz. Dr. Charles has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Newronika. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ipsen. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Iota. Dr. Charles has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Abbott. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AbbVie. Dr. Charles has stock in Arena Therapeutics. The institution of Dr. Charles has received research support from Pharma 2 B. The institution of Dr. Charles has received research support from Intec. The institution of Dr. Charles has received research support from Merz. The institution of Dr. Charles has received research support from Novartis. The institution of Dr. Charles has received research support from Aeon. The institution of Dr. Charles has received research support from Impax. Dr. Mehta has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Adamas Pharma. Dr. Mehta has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Abbott. Dr. Mehta has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CNS Ratings. Marc Schwartz has nothing to disclose. Dr. Sadeghi has received personal compensation for serving as an employee of AbbVie. Dr. Sadeghi has stock in Abbvie/Abbott.

A comprehensive cognitive profile of patients with cervical dystonia (P5-12.006)

<h3>Objective:</h3> To conduct a comprehensive analysis of cognition in cervical dystonia (CD). <h3>Background:</h3> Cervical dystonia is the most prevalent phenotype of the adult onset idiopathic focal dystonias (AOIFD), a group of hyperkinetic disorders. Patients with AOIFD have been shown to have varying abnormalities in cognition. This non-motor feature is underrecognised and may influence QoL. Studies into cognition in CD have been limited by inconsistent psychological assessment tools making generalisations difficult. We selected wide ranging neuropsychological tests to create a comprehensive cognitive profile. <h3>Design/Methods:</h3> 13 adult patients (5 male, 8 female) with idiopathic focal CD completed an extensive neuropsychological battery. Participants were screened for presence of mood disorders (HADS). All patients were receiving 3 monthly botulinum toxin treatment and were not on any medications that could influence cognition. Cognitive domains assessed: premorbid functioning, general intellectual functioning, language, information processing speed, attention, memory, executive function and social cognition using reliable and valid standardized neuropsychological assessments. <h3>Results:</h3> Mean age was 60-years. Mean HADS-A= 6 and mean HADS-D=5. CD patients showed clear evidence of cognitive dysfunction compared to normative data. The most significant impairments were noted in social cognitive measures [emotional prosody to emotional face, FAB (mean Z score: −1.75); naming emotional prosody, FAB (mean Z score: −0.81)], executive function [SWMBE468, CANTAB (mean Z score: −0.73)] and, memory [LM2, WMS (mean Z score: −0.82)]. Moderate impairments were seen in information processing speed [WAIS coding (mean Z score: −0.51)], language [Boston Naming Test (mean Z score: −0.51)] and attention and working memory [RVPA, CANTAB (mean Z score: −0.61)]. Patients performed better than controls in another aspect of social cognition [conflicting emotional prosody, FAB (Z score: 1.67)]. <h3>Conclusions:</h3> There is mounting evidence of cognitive deficits in CD, implicating cognitive pathways in disease pathogenesis. This non-motor aspect may influence QoL and patient satisfaction. Early recognition and neuropsychological input could improve outcomes. <b>Disclosure:</b> Ms. Diepman has nothing to disclose. Dr. Rafee has nothing to disclose. Ruth Monaghan has nothing to disclose. Miss McCormack has nothing to disclose. Dr. Hutchinson has nothing to disclose. Dr. O’Keeffe has nothing to disclose.

SYNCHRONIZE: Real-World Retrospective Review of Safety in Patients Treated With OnabotulinumtoxinA for More Than 1 Therapeutic Indication: An Epidemiologic Perspective (P12-11.006)

SYNCHRONIZE: Real-World Retrospective Review of Safety in Patients Treated With OnabotulinumtoxinA for More Than 1 Therapeutic Indication: An Epidemiologic Perspective (P12-11.006)

Deep Brain Stimulation of the Subthalamic Nucleus in a Patient With X-Linked Dystonia-Parkinsonism. (P11-11.001)

<h3>Objective:</h3> N/A <h3>Background:</h3> X-linked dystonia-parkinsonism (XDP) is one of the genetic causes of generalized dystonia. Both dystonia and parkinsonism components of the disease are frequently resistant to pharmacologic treatment. Deep brain stimulation (DBS) of the globus pallidus pars interna (GPi) is an effective treatment modality, especially for dystonic manifestations of XDP. The subthalamic nucleus (STN) is a known DBS target in Parkinson disease but it is less frequently used for treatment of dystonia. In our case report we describe a patient with XDP who underwent bilateral STN DBS with notable improvement in parkinsonism and dystonia. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> Our patient is a right-handed Filipino man from the island of Panay. He developed bilateral parkinsonism, blepharospasm, oromandibular and cervical dystonia at the age of 51. Diagnosis of XDP was confirmed with genetic testing. Marked improvement of Unified Parkinson Disease Rating Scale (UPDRS) motor score in response to levodopa administration was demonstrated (65 off and 36 on). His pre-DBS Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was 27.5. Bilateral GPi were initially selected as a target for DBS lead placement based on previously published data. Surgical target was subsequently changed to bilateral STN due to distortion in GPi anatomy likely secondary to long-standing disease. Medtronic Percept PC neurostimulator with Sensight DBS leads were used. There was no surgical complications. Patient had an immediate improvement in tremor, bradykinesia and rigidity after his first programming visit; antidystonic effects of stimulation became evident over the next few weeks. There was substantial improvement in limb and oromandibular dystonia and mild effect on cervical dystonia and blepharospasm. His speech clarity became better as well. His motor UPDRS and BFMDRS decreased to 21 and 19, respectively. Gait, posture, ability to perform activities of daily living significantly improved. <h3>Conclusions:</h3> The subthalamic nucleus may be considered an alternative deep brain stimulation target in patients with X-linked dystonia-parkinsonism. <b>Disclosure:</b> Dr. Redko has nothing to disclose. Brian Kopell has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medtronic. Brian Kopell has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Abbott Neuromodulation. Brian Kopell has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Enspire DBS. Dr. Tse has nothing to disclose.

Efficacy and Safety of DaxibotulinumtoxinA for Injection Over Successive Treatments in Adults With Cervical Dystonia in the Phase 3 ASPEN-1 and ASPEN-OLS Trials (P11-11.006)

Efficacy and Safety of DaxibotulinumtoxinA for Injection Over Successive Treatments in Adults With Cervical Dystonia in the Phase 3 ASPEN-1 and ASPEN-OLS Trials (P11-11.006)

Long-term Safety of Repeat Treatments of DaxibotulinumtoxinA for Injection in Adults With Isolated Cervical Dystonia in Phase 3, Open-label, Multicenter ASPEN-OLS Trial (P11-11.003)

Long-term Safety of Repeat Treatments of DaxibotulinumtoxinA for Injection in Adults With Isolated Cervical Dystonia in Phase 3, Open-label, Multicenter ASPEN-OLS Trial (P11-11.003)

“Klüver-Bucy syndrome as a complication of COVID-19” (P1-12.001)

<h3>Objective:</h3> COVID-19 infection is known to cause various complications, including neuropsychiatric ones. Klüver-Bucy syndrome (KBS) is described following some infections, among other causes, and represents a rare constellation of symptoms. <h3>Background:</h3> Majority of COVID-19 associated psychiatric cases described include depression and anxiety in an outpatient, and agitation and confusion in the ICU setting. There are no reports of constellation of symptoms consistent with partial Klüver-Bucy syndrome, which often has infectious etiology. We report a case of 46 y/o Male with past medical history Cerebral Palsy with baseline left-sided hemiparesis, mild dysarthria, two concussions, cervical dystonia, bipolar disorder who developed intermittent episodes of speaking foreign language (Spanish), desire to consume previously rejected foods, staring spells, hypersexuality, change in his regular hobbies:artistic tendencies instead of previously preferred mathematical problem solving after having a prolonged COVID-19 infection with neuropathic pain in the feet and visual field defects on initial presentation. The patient was successfully treated with Carbamazepine. <h3>Design/Methods:</h3> Patient was monitored and treated in our neurology clinic, after a discharge from the hospital for his new onset acute neuropsychiatric symptoms. <h3>Results:</h3> Diagnostic workup included EEG/Video EEG/Brain MRI with and without contrast/Serum and CSF analysis, and has shown nonspecific white matter lesions on T2/FLAIR sequences of the brain MRI, paucicellularity on CSF flow cytometry with essentially absent B-lineage cells, increase in CD4/CD8 ratio, and decreased CD3%, CD8% and CD8 on a serum lymphocytic panel. SARS-COV-2 PCR remained positive for almost two months <h3>Conclusions:</h3> COVID-19 has been associated with various short and long-term sequelae. Psychiatric complications are not infrequent, but are underreported. In our patient’s case, he presented with symptoms of hypersexuality, dietary change, change in hobbies, vision disturbances, staring spells and episodes of clapping and speaking foreign language. We suggest, that this is the first case of a partial Klüver-Bucy syndrome after COVID-19 with the resolution of symptoms on carbamazepine. <b>Disclosure:</b> Dr. Dargah-Zada has nothing to disclose.