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Background: The efficacy and safety of zolpidem in treating musician's dystonia are not well understood. Objective: We aimed to retrospectively investigate the efficacy and safety of zolpidem for musician's dystonia. Methods: We retrospectively reviewed medical records between January 2021 and December 2023 to identify patients with musician's dystonia who had been prescribed zolpidem. The Tubiana's musician's dystonia rating scale (range, 1-5; lower scores indicating greater severity) was used to evaluate musician's dystonia. Results: Fifteen patients were included in this study. The mean effective dose of zolpidem was 5.3±2.0 mg. The mean effective duration of zolpidem was 4.3±1.2 h. With zolpidem administration, the Tubiana's musician's dystonia rating scale score significantly improved from 2.2±1.0 to 4.3±0.8 (48.9% improvement, p<0.001). Two patients (13.3%) discontinued the drug owing to unsatisfactory results or sleepiness. Conclusion: This study suggests that zolpidem may be an alternative treatment option for musician's dystonia.
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Contemporary surgical management of dystonia includes neuromodulation via deep brain stimulation (DBS) or ablative techniques such as radiofrequency (RF) ablation. MRI-guided focused ultrasound (MRgFUS) is an emerging modality that uses high-intensity ultrasound to precisely ablate targets in the brain; this is incisionless, potentially avoiding the surgical risks of a burr hole and transcortical tract to reach the anatomical target. There is some evidence of efficacy in essential tremor and Parkinson's disease (PD), but, to date, there is no study aggregating the evidence of MRgFUS in dystonia. In this narrative review, we searched Medline, Embase, CINAHL, EBSCO, and ClinicalTrials.gov for primary studies and clinical trials on MRgFUS in the treatment of dystonia. Data were analyzed concerning dystonia phenotype, reported outcomes, and complications. PD-related dystonia was also included within the scope of the review. Using our search criteria, six articles on the use of MRgFUS in adult dystonia and three articles on the use of FUS in dystonia in PD were included. Four trials on the use of FUS in dystonia were also found on ClinicalTrials.gov, one of which was completed in December 2013. All included studies showed evidence of symptomatic improvement, mostly in focal hand dystonia; improvements were also found in dystonia-associated tremor, cervicobrachial dystonia, and dystonia-associated chronic neuropathic pain as well as PD-related dystonia. Reported complications included transient neurological deficits and persistent arm pain in one study. However, the evidence is limited to level-4 case series at present. MRgFUS is an emerging modality that appears to be safe and effective, particularly in focal hand dystonia, without major adverse effects. However, the quality of evidence is low at present, and long-term outcomes are unknown. High-quality prospective studies comparing MRgFUS to other surgical techniques will be useful in determining its role in the management of dystonia.
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OBJECTIVE: This study aimed to report the long-term results of thalamotomy in 23 patients with task-specific tremor. METHODS: Data of 23 patients with task-specific tremor who underwent ventralis intermedius nucleus and posterior part of ventro-oral nucleus thalamotomy at the Tokyo Women's Medical University Hospital between 2010 and 2022 were retrospectively analyzed. To evaluate neurological conditions, the severity of task-specific tremor was divided into 0 (no tremor), 1 (slightly tremulous), 2 (moderately tremulous), 3 (accomplishing tasks with great difficulty), and 4 (unable to complete tasks). We also used the subscores "handwriting" (0-4) and "spiral drawing" (0-4) of the Clinical Rating Scales for Tremor. Evaluation scales were presented as medians and interquartile ranges. RESULTS: The severities of task-specific tremor were 3.0 (3.0-4.0) preoperatively and 0.0 (0.0-0.0, p < 0.0001) at the last available evaluation. The writing and spiral drawing of the Clinical Rating Scales for Tremor significantly improved from 3.0 (3.0-4.0) and 3.0 (2.0-3.0) preoperatively, respectively, to 0.0 (0.0-0.0, p < 0.0001) and 0.0 (0.0-0.0, p < 0.0001) at the last available evaluation, respectively. The mean clinical follow-up period was 62.7 ± 26.0 months. Seven (30.4%) patients had focal hand dystonia, which newly developed on the ipsilateral side of the tremor at 2-45 months after the surgery. No serious complications were observed. INTERPRETATION: Thalamotomy significantly improves task-specific tremor with high long-term efficacy, and long-term follow-up is important because focal hand dystonia can develop postoperatively.
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Trastornos Distónicos , Radiocirugia , Temblor , Humanos , Femenino , Temblor/etiología , Temblor/cirugía , Estudios de Seguimiento , Estudios Retrospectivos , Resultado del Tratamiento , Radiocirugia/métodosRESUMEN
Background: The thalamic ventral intermediate nucleus (Vim) and globus pallidus internus are far apart and cannot be captured using a single electrode. Case Description: We describe our experience with a patient with dystonic tremors of the head and upper and lower extremities who showed symptomatic improvement after radiofrequency (RF) ablation using a parietal lobe approach with a single trajectory to capture the pallidothalamic tract and Vim. A 46-year-old man developed head tremors at 41 and a right-sided neck tilt three years later. Five years after the onset of the head tremors, tightness of the larynx during speech and tremors in both the upper and lower limbs also appeared. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score was 24, and the Fahn-Tolosa-Marin Tremor Rating Scale (FTM) score was 48. We captured the pallidothalamic tract and Vim along a single trajectory by locating the entry point in the inferior parietal lobule. One week after treatment, the TWSTRS and FTM scale scores were 9 (62.5%) and 30 (37.5%), respectively. No adverse events were observed. Conclusion: This case suggests that in dystonic tremors involving abnormalities of the basal ganglia-thalamo-cortical and cerebello-thalamo-cortical circuits, a single electrode can be used to approach both circuits through the parietal lobe approach.
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OBJECTIVE: In transcranial MR-guided focused ultrasound (TcMRgFUS), fiber tractography using diffusion tensor imaging (DTI) has been proposed as a direct method to identify the ventral intermediate nucleus (Vim), the ventral caudal nucleus (Vc), and the pyramidal tract (PT). However, the limitations of the DTI algorithm affect the accuracy of visualizing anatomical structures due to its low-quality fiber tractography, whereas the application of the generalized q-sampling imaging (GQI) algorithm enables the visualization of high-quality fiber tracts, offering detailed insights into the spatial distribution of motor cortex fibers. This retrospective study aimed to investigate the usefulness of high-precision fiber tractography using the GQI algorithm as a planning image in TcMRgFUS to achieve favorable clinical outcomes. METHODS: This study included 20 patients who underwent TcMRgFUS. The Clinical Rating Scale for Tremor (CRST) scores and MR images were evaluated pretreatment and at 24 hours and 3-6 months after treatment. Cases were classified based on the presence and adversity of adverse events (AEs): no AEs, mild AEs without additional treatment, and severe AEs requiring prolonged hospitalization. Fiber tractography of the Vim, Vc, and PT was visualized using the DTI and GQI algorithm. The overlapping volume between Vim fibers and the lesion was measured, and correlation analysis was performed. The relationship between AEs and the overlapping volume of the Vc and PT fibers within the lesions was examined. The cutoff value to achieve a favorable clinical outcome and avoid AEs was determined using receiver operating characteristic curve analysis. RESULTS: All patients showed improvement in tremors 24 hours after treatment, with 3 patients experiencing mild AEs and 1 patient experiencing severe AEs. At the 3- to 6-month follow-up, 5 patients experienced recurrence, and 2 patients had persistent mild AEs. Although fiber visualization in the motor cortex using the DTI algorithm was insufficient, the GQI algorithm enabled the visualization of significantly higher-quality fibers. A strong correlation was observed between the overlapping volume that intersects the lesion and Vim fibers and the degree of tremor improvement (r = 0.72). Higher overlapping volumes of Vc and PT within the lesion were associated with an increased likelihood of AEs (p < 0.05); the cutoff volume of Vim fibers within the lesion for a favorable clinical outcome was 401 mm3, while the volume of Vc and PT within the lesion to avoid AEs was 99 mm3. CONCLUSIONS: This pilot study suggests that incorporating the high-precision GQI algorithm for fiber tractography as a planning imaging technique for TcMRgFUS has the potential to enhance targeting precision and achieve favorable clinical outcomes.
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BACKGROUND: Musician's dystonia is a task-specific focal hand dystonia characterized by involuntary contraction of muscles while playing a musical instrument. Current treatment options are often insufficient. OBSERVATIONS: We present the effects of ventro-oral thalamic deep brain stimulation in a patient with musician's dystonia. The patient was a 67-year-old pianist with musician's dystonia who underwent deep brain stimulation with the ventralis oralis anterior and posterior nuclei of the thalamus as targets. The Tubiana and Chamagne rating scale was used to evaluate the effects of stimulation. The outcome was evaluated independently by four clinicians in a blinded manner at 3 months postoperatively. There was a distinct reduction of symptoms during stimulation. At 15 months postoperatively, the beneficial effect remained. No lasting side effects were observed. LESSONS: Further studies are warranted to evaluate the safety and long-term efficacy of this treatment modality.
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Background: Neurosurgical treatment for pistol shooting dystonia has not been studied. Case report: The patient was a 41-year-old woman who participated in the Olympic Games four times as a shooting player. Five months after the final Tokyo Olympic trials, she developed dystonia of the right index finger when shooting. Stereotactic thalamotomy was performed, and a complete resolution of dystonia was achieved. She garnered her personal best score and placed fifth in the Tokyo Olympics. Discussion: Thalamotomy along with deep brain stimulation can be a surgical modality for patients with task-specific dystonia who fail oral medications or botulinum toxin therapy.
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Distonía , Trastornos Distónicos , Femenino , Humanos , Adulto , Trastornos Distónicos/cirugía , Procedimientos NeuroquirúrgicosRESUMEN
A 62-year-old female experienced an extensive cerebral contusion in the left frontotemporal lobe due to an acute subdural hematoma at the age of 44 years. Six months after the injury, the patient developed epileptic seizures. The seizures were generalized with right cervical rotation and fencing posture. Despite prescriptions for four antiepileptic drugs, partial seizures occurred several times a month and focal to bilateral tonic-clonic seizures once every 2 months. Video-electroencephalography showed epileptic discharges in the left frontal lobe. The patient was subsequently referred to our department for palliative surgery. The patient underwent a left Forel-H-tomy. The prescription of antiepileptic drugs was not changed, and the patient was seizure free for 1 year. Forel-H-tomy, a surgical procedure for intractable epilepsy, was pioneered by Dennosuke Jinnai. Despite its previously reported remarkable efficacy, Forel-H-tomy has not been performed for several decades. Nevertheless, it remains a potential alternative treatment option for drug-resistant epilepsy.
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Epilepsia Refractaria , Epilepsia , Femenino , Humanos , Adulto , Persona de Mediana Edad , Epilepsia Refractaria/tratamiento farmacológico , Epilepsia Refractaria/cirugía , Convulsiones/tratamiento farmacológico , Convulsiones/cirugía , Epilepsia/tratamiento farmacológico , Anticonvulsivantes/uso terapéutico , Electroencefalografía/efectos adversosRESUMEN
Background: Essential tremor (ET) is a common involuntary movement disorder (IMD). Radiofrequency ablation (RFA) targeting the ventral intermediate nucleus (Vim) of the thalamus is a stereotactic neurosurgery performed in individuals with ET when pharmacotherapy is no longer effective. Though the reasons remain largely unclear, certain adverse events are known to appear post-RFA. These may be due to functional changes in the Vim, related to RFA-induced tremor reduction, or an adverse reaction to compensatory movement patterns used to perform movements in the presence of tremor symptoms. Objective: This study aimed to understand the characteristics of post-RFA symptoms in individuals with ET. Methods: In a longitudinal case-control study, we compared post-RFA symptoms between individuals with ET who underwent Vim-targeted RFA and those with IMD who underwent non-Vim-targeted RFA. Symptoms were compared preoperatively and 1-week and 1-month postoperatively. Quantitative assessments included center-of-pressure (COP) parameters, grip strength, Mini-Mental State Examination, two verbal fluency tests, and three types of physical performance assessments (upper extremity ability, balance ability, and gait ability). Results: Individuals with ET after RFA showed horizontal displacements of the COP to the treated side (the dominant side of the RFA target's hemisphere) at 1-week postoperatively compared to the preoperative period. The horizontal COP displacement was associated with balance dysfunction related to postural stability post-RFA. Other COP parameters did not significantly differ between the ET and IMD groups. Conclusion: COP displacement to the treated side may be due to a time lag in adjusting postural holding strategies to the long-standing lateral difference in tremor symptoms associated with tremor improvement after RFA.
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INTRODUCTION: Transcranial magnetic resonance-guided focused ultrasound surgery (TcMRgFUS) has the advantage of allowing immediate evaluation of therapeutic effects after each sonication and intraoperative magnetic resonance imaging (MRI) to visualize the lesion. When the image shows that the lesion has missed the planned target and the therapeutic effects are insufficient, the target of the subsequent ablation can be finely adjusted based on the image. The precision of this adjustment is determined by the image quality. However, the current intraoperative image quality with a 3.0T MRI system is insufficient for precisely detecting the lesion. Thus, we developed and validated a method for improving intraoperative image quality. METHODS: Because intraoperative image quality is affected by transmitter gain (TG), we acquired T2-weighted images (T2WIs) with two types of TG: the automatically adjusted TG (auto TG) and the manually adjusted TG (manual TG). To evaluate the character of images with 2 TGs, the actual flip angle (FA), the image uniformity, and the signal-to-noise ratio (SNR) were measured using a phantom. Then, to assess the quality of intraoperative images, T2WIs with both TGs were acquired during TcMRgFUS for 5 patients. The contrast-to-noise ratio (CNR) of the lesion was retrospectively estimated. RESULTS: The images of the phantom with the auto TG showed substantial variations between the preset and actual FAs (p < 0.01), whereas on the images with the manual TG, there were no variations between the two FAs (p > 0.05). The total image uniformity was considerably lower with the manual TG than with the auto TG (p < 0.01), indicating that the image's signal values with the manual TG were more uniform. The manual TG produced significantly higher SNRs than the auto TG (p < 0.01). In the clinical study, the lesions were clearly detected in intraoperative images with the manual TG, but they were difficult to identify in images with the auto TG. The CNR of lesions in images with manual TG was considerably higher than in images with auto TG (p < 0.01). CONCLUSION: Regarding intraoperative T2WIs using a 3.0T MRI system during TcMRgFUS, the manual TG method improved image quality and delineated the ablative lesion more clearly than the current method with auto TG.
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Imagen por Resonancia Magnética , Procedimientos Quirúrgicos Ultrasónicos , Humanos , Estudios Retrospectivos , Imagen por Resonancia Magnética/métodos , Fantasmas de Imagen , Procedimientos Quirúrgicos Ultrasónicos/métodos , Espectroscopía de Resonancia MagnéticaRESUMEN
Cervical dystonia, characterized by the involuntary contraction of cervical muscles, is the most common form of adult dystonia. In a patient with intractable cervical dystonia, we carried out a myotomy of the left obliquus capitis inferior and selective peripheral denervation (SPD) of the posterior branches of the C3-C6 spinal nerves based on preoperative 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT). The patient was a 65-year-old, right-handed man with an unremarkable medical history. His head rotated involuntarily to the left. Medication and botulinum toxin injections were ineffective, and surgical treatment was considered. 18F-FDG PET/CT imaging revealed FDG uptake in the left obliquus capitis inferior, right sternocleidomastoideus, and left splenius capitis. Myotomy of the left obliquus capitis inferior and SPD of the posterior branches of the C3-C6 spinal nerves was performed under general anesthesia. During the 6-month follow-up, the patient's Toronto Western Spasmodic Torticollis Rating Scale score improved from 35 to 9. This case shows that preoperative 18F-FDG PET/CT is effective in identifying dystonic muscles and determining the surgical strategy for cervical dystonia.
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A 30-year-old woman with tardive dystonia in the cervical region from long-term antipsychotic meds was treated with radiofrequency ablation of the right pallidothalamic tract in the fields of Forel. The patient showed improvement in both cervical dystonia and obsessive-compulsive disorder after the procedure, with 77.4% improvement in cervical dystonia and 86.7% improvement in obsessive-compulsive disorder. Although the treatment site in this case was intended to treat cervical dystonia, the lesion was located in the optimal stimulation network for both obsessive-compulsive disorder and cervical dystonia, suggesting that neuromodulation of this region could potentially treat both simultaneously.
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Globo Pálido , Trastorno Obsesivo Compulsivo , Subtálamo , Tortícolis , Trastorno Obsesivo Compulsivo/complicaciones , Trastorno Obsesivo Compulsivo/cirugía , Palidotomía/métodos , Globo Pálido/cirugía , Humanos , Femenino , Adulto , Subtálamo/cirugía , Antipsicóticos/efectos adversos , Ablación por Radiofrecuencia , Tortícolis/inducido químicamente , Tortícolis/complicaciones , Tortícolis/cirugía , Resultado del TratamientoRESUMEN
BACKGROUND: Gamma knife (GK) thalamotomy has been used as a treatment option for essential tremor (ET). Numerous studies on GK use in ET treatment have reported more varied responses and complication rates. METHODS: Data from 27 patients with ET who underwent GK thalamotomy were retrospectively analyzed. The Fahn-Tolosa-Marin Clinical Rating Scale for Tremor, handwriting, and spiral drawing were evaluated. Postoperative adverse events and magnetic resonance imaging findings were also evaluated. RESULTS: The mean age at GK thalamotomy was 78.1 ± 4.2 years. The mean follow-up period was 32.5 ± 19.4 months. The preoperative postural tremor, handwriting, and spiral drawing scores were 3.4 ± 0.6, 3.3 ± 1.0, and 3.2 ± 0.8, respectively, all of which showed significant improvements to 1.5 ± 1.2 (55.9% improvement, P < 0.001), 1.4 ± 1.1 (57.6% improvement, P < 0.001), and 1.6 ± 1.3 (50% improvement, P < 0.001), respectively, at the available final follow-up evaluations. Three patients presented with no improvement in tremor. Six patients presented with adverse effects, including complete hemiparesis, foot weakness, dysarthria, dysphagia, lip numbness, and finger numbness, at the final follow-up period. Two patients presented with serious complications, including complete hemiparesis due to massive widespread edema and chronic encapsulated expanding hematoma. One patient died of aspiration pneumonia following severe dysphagia secondary to chronic encapsulated expanding hematoma. CONCLUSIONS: GK thalamotomy is an efficient procedure for treating ET. Careful treatment planning is necessary to reduce complication rates. The prediction of radiation complications will increase the safety and effectiveness of GK treatment.
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Trastornos de Deglución , Temblor Esencial , Humanos , Anciano , Anciano de 80 o más Años , Temblor Esencial/cirugía , Temblor/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Estudios de Seguimiento , Hipoestesia , Tálamo/diagnóstico por imagen , Tálamo/cirugía , Tálamo/patología , Imagen por Resonancia Magnética , ParesiaRESUMEN
INTRODUCTION: With the advent of MR-guided focused ultrasound, the importance of the efficacy and safety of bilateral ventral intermediate (Vim) thalamotomy for essential tremor (ET) has increased. However, reports on bilateral Vim thalamotomy for ET remain scarce. METHODS: To review the results and complications of bilateral Vim thalamotomy for the treatment of ET in the upper extremities, we retrospectively analyzed the patients with ET who underwent bilateral Vim thalamotomy with radiofrequency (RF) thermal coagulation. As bilateral simultaneous thalamotomy can cause surgical complications, thalamotomy was performed in stages. The interval between the first and second thalamotomies was 21.3 ± 14.7 months. We evaluated the efficacy using the Clinical Rating Scale for Tremor (CRST) before and after the first and second treatments, respectively. We also evaluated the complications before and after the first and second treatments, respectively. Moreover, we assessed the adverse events. RESULTS: Seventeen patients were included in the study. The mean follow-up period following the second thalamotomy was 29.3 ± 15.0 months. The CRST part A + B scores were 34.9 ± 9.7, 20.8 ± 7.0, and 7.4 ± 6.8 before, following the first (40.4% improvement, p < 0.0001) and second thalamotomies (78.6% improvement, p < 0.0001), respectively. Nine patients presented with prolonged adverse events, including dysarthria, dysgeusia, dysphagia, tongue numbness, unsteady gait, and postural instability at the last available evaluation. All adverse events were mild and did not interfere with the patient's daily activities. DISCUSSION/CONCLUSIONS: Bilateral Vim thalamotomy with RF thermal coagulation was an effective treatment for ET in both upper extremities. Despite most possible complications being mild, additional studies with a larger sample size are required to ensure patient safety.
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Temblor Esencial , Humanos , Temblor Esencial/cirugía , Estudios Retrospectivos , Tálamo/cirugía , Resultado del Tratamiento , Temblor , Imagen por Resonancia Magnética/métodosRESUMEN
OBJECTIVE: The objective of this study was to evaluate, at 4 and 5 years posttreatment, the long-term safety and efficacy of unilateral MRI-guided focused ultrasound (MRgFUS) thalamotomy for medication-refractory essential tremor in a cohort of patients from a prospective, controlled, multicenter clinical trial. METHODS: Outcomes per the Clinical Rating Scale for Tremor (CRST), including postural tremor scores (CRST Part A), combined hand tremor/motor scores (CRST Parts A and B), and functional disability scores (CRST Part C), were measured by a qualified neurologist. The Quality of Life in Essential Tremor Questionnaire (QUEST) was used to assess quality of life. CRST and QUEST scores at 48 and 60 months post-MRgFUS were compared to those at baseline to assess treatment efficacy and durability. All adverse events (AEs) were reported. RESULTS: Forty-five and 40 patients completed the 4- and 5-year follow-ups, respectively. CRST scores for postural tremor (Part A) for the treated hand remained significantly improved by 73.3% and 73.1% from baseline at both 48 and 60 months posttreatment, respectively (both p < 0.0001). Combined hand tremor/motor scores (Parts A and B) also improved by 49.5% and 40.4% (p < 0.0001) at each respective time point. Functional disability scores (Part C) increased slightly over time but remained significantly improved through the 5 years (p < 0.0001). Similarly, QUEST scores remained significantly improved from baseline at year 4 (p < 0.0001) and year 5 (p < 0.0003). All previously reported AEs remained mild or moderate, and no new AEs were reported. CONCLUSIONS: Unilateral MRgFUS thalamotomy demonstrates sustained and significant tremor improvement at 5 years with an overall improvement in quality-of-life measures and without any progressive or delayed complications. Clinical trial registration no.: NCT01827904 (ClinicalTrials.gov).