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.

Triple bypass for multisystem smooth muscle dysfunction syndrome due to Arg179His ACTA2 mutation.

Missense mutations in the smooth muscle-specific isoform of the alpha-actin (ACTA2) gene, which encodes smooth muscle actin, congenitally cause systemic smooth muscle dysfunction, leading to multiple systemic smooth muscle dysfunction syndrome. This disease is often diagnosed through the development of congenital mydriasis, patent ductus arteriosus, or thoracic aortic aneurysm at a young age. Some patients develop cerebrovascular lesions, also known as ACTA2 cerebral arteriopathy, which cause ischemic stroke and require surgical revascularization. However, an effective and safe treatment has not yet been established owing to the rarity of the disease. Furthermore, most reports of this disease involve children, with only a few reports on adults and few detailed reports on treatment outcomes published to date. We report a 46-year-old woman with ACTA2 cerebral arteriopathy caused by Arg179His, the most common mutation in this disease; she is the oldest patient reported with this disease to the best of our knowledge. The patient was diagnosed with multiple systemic smooth muscle dysfunction syndrome and ACTA2 cerebral arteriopathy after experiencing a stroke in the right cingulate gyrus. She underwent direct triple bypass with three anastomoses of the right superficial temporal artery to the middle and anterior cerebral arteries. She developed an ischemic stroke as a postoperative complication.The efficacy and safety of this procedure have not been clearly confirmed owing to the frailty of the donor superficial temporal artery and the poor development of collateral circulation; however, direct bypass should be considered a treatment option for patients experiencing progressive multiple strokes.

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.

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.

Subclavian Artery-Carotid Artery Bypass for Subclavian Artery or Common Carotid Artery Severe Stenosis or Occlusion.

Various surgical treatments are available for occlusive subclavian and common carotid artery diseases. Nevertheless, to date, when cerebral endovascular treatment is utilized, revascularization via direct surgery may be required. This study reported five symptomatic cases of revascularization for CCA and SCA occlusive and stenotic lesions that were expected to be challenging to treat with endovascular treatment. We performed subclavian artery-common carotid artery or internal carotid artery bypass using artificial blood vessels or saphenous vein grafts in five patients with subclavian steal syndrome, symptomatic common carotid artery occlusion, and severe proximal common carotid artery stenosis. In this study, good bypass patency was achieved in all five cases. Although there were no intraoperative complications, one patient had a postoperative lymphatic leak. Moreover, there was no recurrence of stroke during postoperative follow-up for an average of 2 years. Conclusively, subclavian artery-common carotid artery bypass can be an effective surgical treatment for common carotid artery occlusion, proximal common carotid artery stenosis, and subclavian artery occlusion.

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.

Radiofrequency Ablation for Movement Disorders: Risk Factors for Intracerebral Hemorrhage, a Retrospective Analysis.

BACKGROUND: One of the greatest concerns associated with radiofrequency ablation is intracerebral hemorrhage (ICH). However, the majority of previous studies have mainly evaluated Parkinson disease patients with ablation of the globus pallidus internus (GPi). OBJECTIVE: To investigate the hemorrhagic risk associated with radiofrequency ablation using ventro-oral (Vo) nucleus, ventral intermediate (Vim) nucleus, GPi, and pallidothalamic tract. METHODS: Radiofrequency ablations for movement disorders from 2012 to 2019 at our institution were retrospectively analyzed. Multivariate analyses were performed to evaluate associations between potential risk factors and ICH. RESULTS: A total of 558 patients underwent 721 stereotactic radiofrequency ablations for movement disorders. Among 558 patients, 356 had dystonia, 111 had essential tremor, and 51 had Parkinson disease. Among 721 procedures, the stereotactic targets used in this study were as follows: Vo: 230; Vim: 199; GPi: 172; pallidothalamic tract: 102; Vim/Vo: 18. ICH occurred in 37 patients (5.1%, 33 with dystonia and 4 with essential tremor). Symptomatic ICH developed in 3 Vo nuclei (1.3%), 3 Vim nuclei (1.5%), and 2 GPi (1.2%). Hypertension (odds ratio = 2.69, P = .0013), higher number of lesions (odds ratio = 1.23, P = .0221), and younger age (odds ratio = 1.04, P = .0055) were significant risk factors for ICH associated with radiofrequency ablation. CONCLUSION: The present study revealed that younger age, higher number of lesions, and history of hypertension were independent risk factors for ICH associated with stereotactic radiofrequency ablation.

Case Report: Long-Term Suppression of Paroxysmal Kinesigenic Dyskinesia After Bilateral Thalamotomy.

Background: Paroxysmal kinesigenic dyskinesia (PKD) is a movement disorder characterized by transient dyskinetic movements, including dystonia, chorea, or both, triggered by sudden voluntary movements. Carbamazepine and other antiepileptic drugs (AEDs) are widely used in the treatment of PKD, and they provide complete remission in 80-90% of medically treated patients. However, the adverse effects of AEDs include drowsiness and dizziness, which interfere with patients' daily lives. For those with poor compatibility with AEDs, other treatment approaches are warranted. Case Report: A 19-year-old man presented to our institute with right hand and foot dyskinesia. He had a significant family history of PKD; his uncle, grandfather, and grandfather's brother had PKD. The patient first experienced paroxysmal involuntary left hand and toe flexion with left forearm pronation triggered by sudden voluntary movements at the age of 14. Carbamazepine (100 mg/day) was prescribed, which led to a significant reduction in the frequency of attacks. However, carbamazepine induced drowsiness, which significantly interfered with his daily life, especially school life. He underwent right-sided ventro-oral (Vo) thalamotomy at the age of 15, which resulted in complete resolution of PKD attacks immediately after the surgery. Four months after the thalamotomy, he developed right elbow, hand, and toe flexion. He underwent left-sided Vo thalamotomy at the age of 19. Immediately after the surgery, the PKD attacks resolved completely. However, mild dysarthria developed, which spontaneously resolved within three months. Left-sided PKD attacks never developed six years after the right Vo thalamotomy, and right-sided PKD attacks never developed two years after the left Vo thalamotomy without medication. Conclusion: The present case showed long-term suppression of bilateral PKDs after bilateral thalamotomy, which led to drug-free conditions.

Case Report: Deep Cerebellar Stimulation for Tremor and Dystonia.

Background: The cerebellum plays an important role in the pathogenesis and pathophysiology of movement disorders, including tremor and dystonia. To date, there have been few reports on deep cerebellar stimulation. Case Report: The patient was a 35-year-old previously healthy man with no history of movement disorders. He developed a tremor and stiffness in his left hand at the age of 27 years, which was diagnosed as a dystonic tremor. We performed right thalamotomy, which resulted in a complete resolution of the tremor; however, the dystonia persisted. Subsequently, the patient developed left foot dystonia with inversion and a newly developed tremor in the right hand and foot. The patient underwent left ventralis intermedius (VIM) deep brain stimulation (VIM-DBS) and left pallidothalamic tract DBS (PTT-DBS). Left VIM-DBS completely resolved the right hand and foot tremor, and PTT-DBS significantly improved the left hand and foot dystonia. Three months postoperatively, the patient developed an infection and wound disruption at the surgical site. We performed palliative surgery for deep cerebellar stimulation via the posterior cranial region, which was not infected. The surgery was performed under general anesthesia with the patient lying in the prone position. Eight contact DBS electrodes were used. The placement of electrodes extended from the superior cerebellar peduncle to the dentate nucleus. Both the right hand and foot tremor improved with right cerebellar stimulation. Further, both the left hand and foot dystonia improved with left cerebellar stimulation. Right and left cerebellar stimulation led to no improvement in the left hand and foot dystonia and right hand and foot tremor, respectively. Stimulation-induced complications observed in the patient included dizziness, dysphagia, and dysarthria. After the surgery, the patient developed hypersalivation and hyperhidrosis in the left side of the body, both of which did not improve with adjustments of stimulation parameters. At the 6-month follow-up, the tremor and dystonia had almost completely resolved. Conclusion: Deep cerebellar stimulation deserves consideration as a potential treatment for tremor and dystonia.

Successful Treatment of Auricular Dystonia by Unilateral Pallidothalamic Tractotomy.

Background: Ear movement disorders are rarely reported. Although some patients may respond to botulinum toxin injections, reports on surgical treatment options remain limited. Case Report: A 57-year-old woman was diagnosed with auricular dystonia, which was refractory to botulinum toxin injections. Since involuntary movement and pain were predominantly present on the right side and the patient rejected the implantation of a mechanical device, we decided to perform left pallidothalamic tractotomy. Immediately following lesioning, bilateral ear movements and right auricular pain ceased with no complications. Discussion: Stereotactic neurosurgical treatment can be an alternative for auricular dystonia. Highlights: Ear movement disorders, such as auricular myoclonus or dystonia, are rarely reported.The present case was refractory to repetitive botulinum toxin injections and oral medications.To the best of our knowledge, this is the first case of auricular dystonia that successfully improved with stereotactic neurosurgical treatment (pallidothalamic tractotomy).

Radiofrequency ablation for DYT-28 dystonia: short term follow-up of three adult cases.

Mutations in the lysine methyltransferase 2B (KMT2B) gene have recently been reported to be associated with childhood-onset generalized dystonia. There have been no studies investigating ablative treatments for the management of this disorder. Three patients underwent either a staged unilateral pallidotomy and contralateral pallidothalamic tractotomy (19-year-old man, 2-year follow-up), a unilateral pallidothalamic tractotomy (34-year-old man, 6-month follow-up) or a simultaneous unilateral pallidothalamic tractotomy and ventro-oral thalamotomy (29-year-old man, 6-month follow-up). The average total patient score on the Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale improved from 39.5 to 13.2 (66.6%) after the procedures. No significant complications were identified. Ablative treatments appear to be a promising alternative surgical option for generalized dystonia with KMT2B mutation.

Mechanical Thrombectomy for Middle Cerebral Artery Occlusion Suspected of Ovarian Hyperstimulation Syndrome: Case Report and Review of the Literature.

BACKGROUND: Ovarian hyperstimulation syndrome (OHSS) is an iatrogenic disorder associated with infertility treatment. The main pathology of OHSS is intravascular dehydration and hyperestrogenemia. In mild cases, abdominal symptoms are the main symptoms, but in severe cases, thrombosis such as cerebral infarction may occur. CASE DESCRIPTION: A 36-year-old woman was undergoing infertility treatment in obstetrics and gynecology for infertility. She received HMG-HCG therapy and artificial insemination 1 week before onset, and on the day of onset she had mild abdominal distension and was suspected of having OHSS. She was prescribed aspirin for prevention of thrombosis. She presented with right upper hemiparesis and aphasia. Magnetic resonance imaging showed left middle cerebral artery occlusion. We performed mechanical thrombectomy and finally achieved Thrombolysis in Cerebral Infarction 3 recanalization. We suspected embolism as the etiology of cerebral infarction and started anticoagulation therapy. Various examinations were conducted to investigate the embolism source, but no anatomic abnormality or thrombophilia factors were observed. Because the patient had OHSS since admission, we concluded that OHSS was suspected as the cause of the stroke. CONCLUSIONS: We experienced the first case of mechanical thrombectomy for middle cerebral artery occlusion suspected to be caused by OHSS. It is necessary to suspect OHSS involvement if young women, especially those on infertility treatment, show neurologic deficits.

Intra-arterial Injection of Fasudil Hydrochloride for Cerebral Vasospasm Secondary to Bacterial Meningitis.

The occurrence of cerebral vasospasm secondary to bacterial meningitis is relatively rare. Furthermore, there is no specific treatment cerebral vasospasm. Endovascular treatment may be essential for cases with the advanced clinical course. Balloon angioplasty or intra-arterial injection of verapamil, nicardipine, or nitroglycerin has been previously reported. We experienced successful treatment using intra-arterial infusion of fasudil hydrochloride. To our knowledge, this is the first case to report the intra-arterial injection of fasudil hydrochloride for treating cerebral vasospasm secondary to bacterial meningitis. A 37-year-old female who presented with dizziness had a right cerebellar tumor that was excised and diagnosed as glioblastoma. On postoperative day 10, Streptococcus oralis meningitis was detected. On postoperative day 20, the patient developed right hemiparesis with a severe vasospasm of the bilateral middle cerebral artery and anterior cerebral artery. Intra-arterial fasudil hydrochloride injection was performed for 3 days, following which the patient's symptoms improved. Symptomatic cerebral vasospasm secondary to bacterial meningitis is relatively rare and difficult to treat; in selected cases, intra-arterial fasudil hydrochloride injection was an effective treatment for cerebral vasospasm secondary to bacterial meningitis.