Efficacy and safety of zolpidem for musician's dystonia.

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.

Long-term follow-up of task-specific tremor after thalamotomy: a retrospective observational study.

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.

Radiofrequency ablation of the pallidothalamic tract and ventral intermediate nucleus for dystonic tremor through the parietal approach.

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.

Abolition of seizures following Forel-H-tomy for drug-resistant epilepsy: A case report.

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.

Pistol Shooting Dystonia Treated with Thalamotomy.

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.

Myotomy and Selective Peripheral Denervation Based on 18F-FDG PET/CT in Intractable Cervical Dystonia: A Case Report.

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.

Improvement of obsessive-compulsive disorder after pallidothalamic tractotomy for cervical dystonia.

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.

Gamma Knife Thalamotomy for Essential Tremor: A Retrospective Analysis.

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.

Bilateral Radiofrequency Ventral Intermediate Thalamotomy for Essential Tremor.

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.

Loss-of-function mutations in SGCE found in Japanese patients with myoclonus-dystonia.

SGCE myoclonus-dystonia is a monogenic form of dystonia with an autosomal dominant mode of inheritance that co-occurs with a myoclonic jerk. In this study, we present 12 Japanese patients from nine families with this disease. Targeted next-generation sequencing covering major causative genes for monogenic dystonias identified nine distinct SGCE mutations from each of the families: three nonsense, two frameshift, two missense, one in-frame 15 bp deletion, and one splice donor site mutations, of which four were previously unreported. One missense mutation (c.662G>T, p.Gly221Val) was located at the 3' end of exon 5 (NM_001099400), which was predicted to cause aberrant splicing according to in silico predictions. Minigene assays performed together with the c.825+1G>C mutation demonstrated complete skipping of exon 5 and 6, respectively, in their transcripts. The other missense (c.1345A>G, p.Met449Val) and 15 bp deletion (c.168_182del, p.Phe58_Leu62del) mutations showed a significant reduction in cell membrane expression via HiBiT bioluminescence assay. Therefore, we concluded that all the detected mutations were disease-causing. Unlike the other detected mutations, p.Met449Val affects only isoform 3 (NP_001092870 encoded by NM_001099400) among the variously known isoforms of SGCE. This isoform is brain-specific and is mostly expressed in the cerebellum, which supports recent studies showing that cerebellar dysfunction is a key element in the pathophysiology of SGCE myoclonus-dystonia.

Long-Term Results of Gamma Knife Radiosurgery for Trigeminal Neuralgia.

OBJECTIVE: Although the short- to medium-term efficacy of Gamma Knife therapy for drug-resistant essential trigeminal neuralgia has been reported, long-term evaluations are limited. We evaluated patient data obtained at least 10 years post-treatment and examined the significance of this treatment using new end points. METHODS: Among 249 consecutive patients with essential trigeminal neuralgia who were treated with Gamma Knife radiosurgery (retrogasserian target/4-mm single isocenter/90 Gy at 100%) at our institution between January 2003 and October 2011, 103 patients who were followed up for at least 10 years (mean, 174 [120-219] months) after treatment and whose data were amenable to accurate evaluation, were included in this retrospective study. Herein, we used the Barrow Neurological Institute (BNI) pain intensity scale as a clinical evaluation method for pain and the BNI numbness scale to evaluate complications (namely facial dysesthesia). RESULTS: The initial and final follow-up pain attack cessation (BNI pain intensity score I-IIIa) rate was 82.5% (85 of 103) and 58.2% (60 of 103), respectively. Furthermore, sensory impairment (BNI numbness score ≥ II) at the last follow-up was observed in 24.3% (25 of 103) of the cases, while very bothersome status (BNI numbness score IV) was observed in 2.9% of the cases. CONCLUSIONS: Gamma Knife radiosurgery for essential trigeminal neuralgia showed good therapeutic effects during long-term follow-up. Serious complications of significant concern in the short- to mid-term follow-up, resolved spontaneously. Therefore, the indications for treatment should be expanded to include patients who strongly desire Gamma Knife therapy.

Complex regional pain syndrome after mRNA-based COVID-19 vaccination.

We describe the case of a 17-year-old woman diagnosed with complex regional pain syndrome (CRPS) at a pain clinic after the second dose of the COVID-19 vaccine. She was referred to our department for surgical treatment of movement disorder seven months after the second inoculation. Baclofen (50 µg), administered intrathecally, improved the involuntary movements of her right hand. After administration of zolpidem (5 mg), involuntary movements of the right index finger almost disappeared. However, neither zolpidem nor intrathecal baclofen improved the limited range of motion of the first joint of the left-hand finger. Despite various reports on CRPS development after vaccination, only one case post COVID-19 vaccination has been reported. Therefore, healthcare providers should keep in mind that CRPS can appear after the COVID-19 vaccination.

Successful treatment of focal hand dystonia after cervical whiplash injury by thalamotomy.

Background: Posttraumatic focal hand dystonia after cervical whiplash injury is rare. Moreover, the treatment of peripheral posttraumatic dystonia is usually ineffective. Herein, we report a case of successful thalamotomy for a patient with focal hand dystonia after cervical whiplash injury. Case Description: A 39-year-old woman was hit from behind by a car; subsequently, she felt strange in the right hand and was diagnosed with whiplash injury. A month later, she developed a persistent abnormal posture of the right hand. Brain imaging showed no lesions, and cervical magnetic resonance imaging showed stenosis but no spinal cord signal changes. Posttraumatic dystonia was diagnosed, for which the patient underwent left ventro-oral (Vo) thalamotomy. One year after the first surgery, the patient underwent left Vo and ventral intermediate nucleus (Vim) thalamotomy due to recurrence of dystonia. Nine years after the second surgery, the patient continues to be able to maintain her normal physical routine. Conclusion: Vo-Vim thalamotomy may be a feasible and effective treatment for focal hand dystonia after cervical whiplash injury.

Postoperative 18F-FDG-PET/CT documents efficacy of selective peripheral denervation for treating cervical dystonia.

Background: Cervical dystonia, characterized by involuntary contraction of the cervical muscles, is the most common form of adult dystonia. We compared the preoperative versus postoperative 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) scans to confirm the efficacy of selective peripheral denervation (SPD) for treating cervical dystonia. Case Description: A 38-year-old male with the right-sided cervical dystonia underwent a left pallidothalamic tractotomy. However, the involuntary neck movement persisted and correlated with the 18F-FDG-PET/CT imaging that showed persistent FDG uptake in the right obliquus capitis inferior muscle. A subsequent SPD resulted in resolution of the dystonia that correlated with lack of further 18F-FDG-PET/CT uptake in the right obliquus capitis inferior muscle. Conclusion: The postoperative 18F-FDG-PET/CT documented the efficacy of an SPD in resolving a patient's cervical dystonia.

Efficacy and Safety of Zolpidem for Focal Dystonia After Neurosurgical Treatments: A Retrospective Cohort Study.

Although there are several reports of the significant efficacy of zolpidem for treating dystonia, zolpidem is still considered an anecdotal treatment. Here, we evaluated the efficacy and safety of zolpidem for treating residual dystonia in patients who previously received various neurosurgical treatments majorly including deep brain stimulation and radiofrequency ablation. We retrospectively reviewed medical records from January 2021 to September 2021 to identify patients with dystonia who had been prescribed zolpidem after undergoing neurosurgery. Twenty patients were enrolled in this study, including those with blepharospasm (two), tongue dystonia (four), mouth dystonia (one), spasmodic dysphonia (two), cervical dystonia (six), focal hand dystonia (three), hemidystonia (two), blepharospasm with cervical dystonia (one), and mouth dystonia with cervical dystonia (one). Single doses of zolpidem ranged between 2.5 and 10 mg, while daily dosages ranged from 10 to 30 mg. The zolpidem dose prescribed was 5-10 mg, with single and daily doses of 7 ± 2.9 and 14.5 ± 6.0 mg, respectively. With zolpidem administration, the participants' Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale score significantly improved from 8.1 ± 6.7 to 3.7 ± 2.5 (50.6% improvement, p < 0.0001). Improvements in arm dystonia, blepharospasm, and spasmodic dysphonia were observed using the Arm Dystonia Disability Scale, Jankovic Rating Scale, and Voice Handicap Index, respectively. No improvements were observed in cervical dystonia on the Toronto Western Spasmodic Torticollis Rating Scale. Drowsiness, including three cases each of mild and moderate drowsiness, was the most frequent adverse effect (30%), which persisted for 2-3 h. Transient amnesia and rapid eye movement sleep behavior disorder occurred in two patients and one patient, respectively. Although our findings suggest that zolpidem can be a valuable treatment option for patients with residual dystonia after neurosurgical treatments, the beneficial effects for cervical dystonia were limited.

Unilateral pallidothalamic tractotomy at Forel's field H1 for cervical dystonia.

BACKGROUND: Neurosurgical ablation of Forel's field H1 for cervical dystonia, which is currently abandoned, was formerly used in the 1960s-1970s. Regardless of the lack of neuroimaging modalities and objective evaluation scales, the reported efficacy was significant. Although recent studies have reappraised the ablation of the pallidothalamic tract at Forel's field H1 for Parkinson's disease, the efficacy for cervical dystonia has not been investigated well. METHODS: Data of 35 patients with cervical dystonia who underwent unilateral pallidothalamic tractotomy at Forel's field H1 were retrospectively analyzed. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores, the neck score of the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), and adverse events were evaluated preoperatively and at the last available follow-up period. RESULTS: The mean clinical follow-up period was 13.9 ± 6.5 months. The mean TWSTRS total scores were 34.3 ± 14.0 preoperatively and 18.4 ± 16.5 at the last available follow-up period (46.4% improvement, p < 0.0001). The BFMDRS neck score also improved significantly from 6.2 ± 2.9 preoperatively to 2.8 ± 2.8 at the last available follow-up period (55.0% improvement on the neck score, p < 0.0001). Reduced hand dexterity in seven patients, hypophonia in five patients, dysarthria in four patients, and executive dysfunction in one patient were confirmed as adverse events at the last available follow-up evaluation. One patient had postoperative hemorrhage. CONCLUSION: The current study confirmed significant improvement in TWSTRS total scores and BFMDRS neck scores at the 13.9-month follow-up after unilateral pallidothalamic tractotomy. The pallidothalamic tract in Forel's field H1 is expected to be an alternative treatment target for cervical dystonia.