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.

Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children.

OBJECTIVE: Dystonias are relatively rare disorders characterized by sustained or intermittent muscle contractions causing abnormal movements or postures. Generalized dystonia is a therapeutic challenge because medications are unable to control dystonia adequately in most patients. These patients may be candidates for surgical therapy. The commonly used surgical procedures in these patients are pallidotomy and deep brain stimulation. Limited studies are available on the role of pallidotomy in children with acquired/heredodegenerative generalized dystonia. The objective of this study was to describe the authors' experience with bilateral pallidotomy in this group of patients. METHODS: The authors retrospectively reviewed all pediatric patients (less than 18 years of age) with acquired/heredodegenerative generalized dystonia who underwent bilateral simultaneous pallidotomy at their center between January 2014 and January 2021. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores before and after surgery were recorded. Complications arising after the procedure were recorded as well. RESULTS: Ten patients (8 male and 2 female) with a mean (range) age of 11.1 (5-17) years were included in this study. The mean duration between disease onset and surgical intervention was 3.9 years. Two patients presented in status dystonicus. The mean ± SD (range) preoperative BFMDRS score of the patients without status dystonicus (n = 8) was 80 ± 18.9 (59.5-108). The mean ± SD BFMDRS score at the time of discharge from the hospital after surgery was 58.8 ± 37.9. Three patients had more than 20% change in BFMDRS score at the time of discharge from the hospital. The mean improvement was 25.5% at the end of 1 year. Of 5 surviving patients in the non-status dystonicus group, 3 patients had more than 40% change in BFMDRS score while the other 2 patients developed recurrence at the last follow-up (4.5 years). Status dystonicus abated after bilateral pallidotomy in both patients. Permanent bulbar complications were seen in 2 patients. CONCLUSIONS: Bilateral pallidotomy may result in clinically significant improvement in children with acquired/heredodegenerative generalized dystonia, although the benefits should be closely weighed against the risk of irreversible bulbar dysfunction. It is a viable option for children in resource-limited settings.

Safety and efficacy of unilateral and bilateral pallidotomy for primary dystonia.

OBJECTIVE: Ablation of the globus pallidus internus (pallidotomy) is an effective surgical intervention for dystonia. However, the current literature on the efficacy and safety of pallidotomy for dystonia is derived only from single-case reports and small cohort studies. METHODS: We retrospectively analyzed patients with primary dystonia who underwent pallidotomy at our institution between 2014 and 2019. Neurological conditions were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS, range: 0-120). We evaluated the total BFMDRS score and each subitem score (nine body regions) in the patients who underwent unilateral and bilateral pallidotomy before surgery and at last available follow-up. Moreover, postoperative complications were analyzed. RESULTS: We found that 69 and 20 patients underwent unilateral and bilateral pallidotomy respectively. The mean age at dystonia onset was 40.4 ± 15.2 years. The mean clinical follow-up period was 17.2 ± 11.6 months. Unilateral pallidotomy significantly improved the total BFMDRS score from 11.2 ± 14.7 preoperatively to 5.4 ± 7.6 at last available follow-up (51.8% improvement, p < 0.001). Furthermore, there was a significant and independent improvement in all midline BFMDRS subitems, including eyes, mouth, speech/swallow, and neck, after unilateral pallidotomy. Bilateral pallidotomy significantly improved the total BFMDRS score from 14.6 ± 10.2 preoperatively to 3.8 ± 8.2 at last available follow-up (74.0% improvement, p < 0.001). However, bilateral pallidotomy induced medically refractory parkinsonism (postural instability and gait disturbance) in five patients, dysarthria in three patients, and dysphagia in one patient. INTERPRETATION: Unilateral radiofrequency pallidotomy remains a viable treatment option for patients with some forms of dystonia. Bilateral pallidotomy cannot be recommended due to unacceptably high complication rates.

Pallidal Deep Brain Stimulation in Patients with Prior Bilateral Pallidotomy and Selective Peripheral Denervation for Treatment of Dystonia.

INTRODUCTION: Deep brain stimulation (DBS) of the globus pallidus internus has become an accepted treatment for severe isolated idiopathic and inherited dystonia. Patients who had other forms of surgery earlier, such as radiofrequency lesioning or selective peripheral denervation, however, usually are not considered candidates for DBS. OBJECTIVE: The aim of this study was to evaluate the long-term outcome of pallidal DBS in a rare subgroup of patients who had undergone both pallidotomy and selective peripheral denervation previously with a waning effect over the years. METHODS: Pallidal DBS was performed according to a prospective study protocol in 2 patients with isolated idiopathic dystonia, and patients were followed for a period of at least 6 years. RESULTS: Both patients benefitted from long-lasting amelioration of dystonia after pallidal DBS, which was comparable to that of patients who did not have previous surgeries. In a 62-year-old female with cervical dystonia both the Burke-Fahn-Marsden (BFM) and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) motor scores were improved at follow-up 8 years after surgery (50 and 39%). In a 32-year-old male with generalized dystonia, the BFM motor and disability scores showed marked improvement at 6.5 years of follow-up (82 and 66%). CONCLUSIONS: Pallidal DBS can yield marked and long-lasting improvement in patients who underwent both pallidotomy and selective peripheral denervation earlier. Therefore, such patients, in general, should not be excluded from DBS.

Successful Relief of Abdominal Dystonia After Sequential GPi Pallidotomy with 2-Year Follow-Up.

BACKGROUND: Abdominal dystonia is very rare. To our knowledge, no clinical study has reported its specific treatment. Stereotactic therapy has been used to treat several movement disorders, including focal and general dystonia. We investigated the use of internal globus pallidum (GPi) pallidotomy for abdominal dystonia after failed oral medication. CASE DESCRIPTION: A 48-year-old man presented with abdominal dystonia and complaints of involuntary undulating and contraction movements of his left abdominal wall for 5 years. Treatment with oral medication for 4 years was ineffective. Lesioning of the right GPi successfully relieved his symptoms. The symptoms recurred at 3 months and right GPi pallidotomy was repeated with complete resolution of symptoms after the second procedure. There was no recurrence or focal deficit at the 2-year follow-up. CONCLUSIONS: GPi pallidotomy is feasible and effective for the treatment of abdominal dystonia that is resistant to standard medical therapy.

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.

Predicting final lesion characteristics during MR-guided focused ultrasound pallidotomy for treatment of Parkinson's disease.

OBJECTIVE: Magnetic resonance-guided focused ultrasound (MRgFUS) ablation of the globus pallidus interna (GPi) is being investigated for the treatment of advanced Parkinson's disease symptoms. However, GPi lesioning presents unique challenges due to the off-midline location of the target. Furthermore, it remains uncertain whether intraprocedural MR thermometry data can predict final lesion characteristics. METHODS: The authors first performed temperature simulations of GPi pallidotomy and compared the results with those of actual cases and the results of ventral intermediate nucleus (VIM) thalamotomy performed for essential tremor treatment. Next, thermometry data from 13 MRgFUS pallidotomy procedures performed at their institution were analyzed using 46°C, 48°C, 50°C, and 52°C temperature thresholds. The resulting thermal models were compared with resulting GPi lesions noted on postprocedure days 1 and 30. Finally, the treatment efficiency (energy per temperature rise) of pallidotomy was evaluated. RESULTS: The authors' modeled acoustic intensity maps correctly demonstrate the elongated, ellipsoid lesions noted during GPi pallidotomy. In treated patients, the 48°C temperature threshold maps most accurately predicted postprocedure day 1 lesion size, while no correlation was found for day 30 lesions. The average energy/temperature rise of pallidotomy was higher (612 J/°C) than what had been noted for VIM thalamotomy and varied with the patients' skull density ratios (SDRs). CONCLUSIONS: The authors' acoustic simulations accurately depicted the characteristics of thermal lesions encountered following MRgFUS pallidotomy. MR thermometry data can predict postprocedure day 1 GPi lesion characteristics using a 48°C threshold model. Finally, the lower treatment efficiency of pallidotomy may make GPi lesioning challenging in patients with a low SDR.

Gamma Knife Radiosurgical Pallidotomy for Dystonia: Not a Fallen Angel.

The authors report a case of successful management of right side hemidystonia with gamma knife radiosurgery. A 24-year-old male with a history of birth asphyxia subsequently developed worsening right-sided torsional hemidystonia which failed to respond to the medical management. MRI of the brain was unremarkable. Stereotactic gamma knife radiosurgery (GKRS) was performed to create a lesion in the left posteroventral globus pallidum. The patient gradually improved over a course of 18 months without any complication. He obtained 61% improvement in dystonia rating scale. Radiosurgical pallidotomy is often viewed with suspicion and functional neurosurgeons show reluctance in preferring it to stereotactic radio frequency lesioning or stimulation surgery. The authors would like to highlight the chances of not only control, but also cure of the disease with this cost-effective treatment modality.

Pallidothalamic Tractotomy (Forel's Field H1-tomy) for Dystonia: Preliminary Results.

OBJECTIVES: The pallidothalamic tract connects the globus pallidus internus with the ventroanterior and ventrolateral parts of the thalamus. Lesioning or stimulation of the pallidothalamic tract has ameliorating effects on dyskinesia and dystonia in patients with Parkinson disease. However, the effect of the procedure on dystonia due to other etiologies has not been reported. METHODS: We retrospectively analyzed patients with dystonia who underwent unilateral pallidothalamic tractotomy between July 2017 and October 2018 at Tokyo Women's Medical University Hospital. The Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale was used to evaluate the severity of dystonia at three time points (before surgery, 3 months postoperatively, and the last available follow-up). Adverse events were also evaluated. RESULTS: Eleven patients underwent unilateral pallidothalamic tractotomy, including 5 with generalized dystonia, 4 with segmental dystonia, and 2 with focal (cervical) dystonia. All patients had undergone unilateral pallidotomy before contralateral pallidothalamic tractotomy. The mean interval between the previous surgery (pallidotomy) and pallidothalamic tractotomy was 9.5 ± 3.1 months. The mean follow-up period was 11.5 ± 4.2 months. The Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale scores at 3 months after pallidothalamic tractotomy (5.8 ± 8.4) and at the last available follow-up (5.6 ± 8.3, P < 0.001) were significantly improved compared with that before pallidothalamic tractotomy (21.8 ± 16.3). The most common adverse event was reduced voice volume (6 patients), which was mild and did not interfere with the patient's daily activities. CONCLUSIONS: This study suggests that pallidothalamic tractotomy can be an alternative treatment target for dystonia. A larger and longer prospective study is needed to elucidate the safety and efficacy of pallidothalamic tractotomy for dystonia.

Staged pallidotomy: MRI and clinical follow-up in status dystonicus.

PURPOSE: We report on a patient affected by Status Distonicus who was treated with Deep Brain Stimulation electrodes implanted in the Globus Pallidus internus (Gpi) and used for serial radiofrequency lesions. MATERIALS AND METHODS: The evolution of radiofrequency lesions was monitored by post-operative and late Magnetic Resonance Imaging (MRI). After the first lesion the patient did improve, though not in a significant fashion. Therefore, three further radiofrequency lesions were delivered 2, 4 and 6 days respectively after surgery with subsequent improvement of dystonic movements. RESULTS: MRI scans performed at 8 days, 3 months, and 6 months after surgery showed a diffuse T2-hyperintense and T1-hypointense GPi signal alteration which progressively decreased over time. CONCLUSION: We confirm that the possibility to stage pallidotomies over time using a couple of new contacts is a safe and efficacious procedure in treating SD patients where the lesions themselves are limited by the appearance of side effects, or in patients showing a poor response to a single lesion. As far as we know, this is the first description of MRI evolution and monitoring of a staged pallidotomy.

Combined Unilateral Posteroventral Pallidotomy and Ventral Intermediate Nucleus Thalamotomy in Tremor-Dominant Parkinson's Disease versus Posteroventral Pallidotomy Alone: A Prospective Comparative Study.

BACKGROUND: The optimum target in surgery for Parkinson's disease (PD) is still controversial, especially in patients with tremor-dominant PD. We aim to compare results in tremor-dominant patients undergoing pallidotomy vs. those undergoing simultaneous posteroventral pallidotomy (PVP) and ventral intermediate nucleus (VIM) thalamotomy. METHODS: Twenty-four patients with tremor-dominant PD were included in this study. Twelve patients received unilateral PVP contralateral to the most affected side. The other 12 patients received simultaneous unilateral PVP and VIM thalamotomy contralateral to the most affected side. Assessment of results in both groups was achieved using both UPDRS "off" motor scores and UPDRS rest tremor subscores. RESULTS: The mean UPDRS off motor score improved in the pallidotomy group from 61.3 preoperatively to 36.8 at 12 months. In the combined group, it improved from 59.6 to 35.2 at 12 months, with no statistically significant difference between both groups. On the other hand, while the mean tremor subscore in the pallidotomy group improved from a mean of 2.3-0.8, the tremors were abolished in all of the patients in the combined group except for 1 patient who showed slight infrequent tremors at 12 months. CONCLUSION: Patients with tremor-dominant PD achieve more improvement in tremor control after combined PVP and VIM thalamotomy.

An unusual surgical indication for cerebral tuberculosis: status dystonicus. Case report.

Actual indications for surgery in tuberculosis are limited to obtaining a diagnosis, acquiring tissue for culture studies, treating hydrocephalus, aspiring a brain abscess, and reducing intracranial pressure in patients with multiple tuberculomas. Tuberculosis-related movement disorders are usually treated pharmacologically. We report on a child affected by post-tubercular generalized dystonia, who progressed to status dystonicus (SD) and underwent stereotactic bilateral pallidotomy. After surgery, SD resolved, and drugs were rapidly tapered. The successful reversal of SD and the motor improvement observed in our patient demonstrate the safety, feasibility, and clinical efficacy of pallidotomy in post-tuberculous-meningoencephalitis dystonia and SD.

Surgical treatment of dystonia.

INTRODUCTION: Treatment of dystonia should be individualized and tailored to the specific needs of patients. Surgical treatment is an important option in medically refractory cases. Several issues regarding type of the surgical intervention, targets, and predict factors of benefit are still under debate. Areas covered: To date, several clinical trials have proven the benefit and safety of deep brain stimulation (DBS) for inherited and idiopathic isolated dystonia, whereas there is still insufficient evidence in combined and acquired dystonia. The globus pallidus internus (GPi) is the target with the best evidence, but data on the subthalamic nucleus seems also to be promising. Evidence suggests that younger patients with shorter disease duration experience greater benefit following DBS. Pallidotomy and thalamotomy are currently used in subset of carefully selected patients. The development of MRI-guided focused ultrasound might bring new options to ablation approach in dystonia. Expert commentary: GPi-DBS is effective and safe in isolated dystonia and should not be delayed when symptoms compromise quality of life and functionality. Identifying the best candidates to surgery on acquired and combined dystonias is still necessary. New insights about pathophysiology of dystonia and new technological advances will undoubtedly help to tailor surgery and optimize clinical effects.

Radiosurgical Pallidotomy for Parkinson's Disease.

Deep brain stimulation (DBS) has been widely accepted as a tool for treating many symptoms of Parkinson's disease (PD); pallidotomy has been nearly abandoned. Concerns about both the safety and efficacy of pallidotomy are based on small series, isolated case reports, and techniques that would now be considered obsolete. The senior author recently reviewed long-term follow-up of a series of patients who had gamma knife pallidotomy (GKP) for advanced PD. GKP leads to durable, clinically significant benefit. Bilateral GKP adds incremental improvement. The complication rate was 4% when calculated on a per lesion basis. GKP is not quite as effective as DBS for tremor and bradykinesia; the results of GKP and DBS are equivalent for dyskinesia. GKP should be considered in patients who are not candidates for DBS. GKP is not as invasive as radiofrequency pallidotomy and avoids the problems and expenses associated with DBS. Patients on anticoagulants, with cognitive deficits or with other contraindications to DBS can be offered GKP to alleviate many of the motor symptoms of PD.

The changing landscape of surgery for Parkinson's Disease.

Neurosurgical interventions have been used to treat PD for over a century. We examined the changing landscape of surgery for PD to appraise the value of various procedures in the context of advances in our understanding and technology. We assessed the number of articles published on neurosurgical procedures for PD over time as an albeit imprecise surrogate for their usage level. We identified over 8,000 publications associated with PD surgery. Over half the publications were on DBS. The field of DBS for PD showed a rapid rise in articles, but is now in a steady state. Thalamotomy and, to a lesser extent, pallidotomy follow a biphasic publication distribution with peaks approximately 30 years apart. Articles on gene therapy and transplantation experienced initial rapid rises and significant recent declines. Procedures using novel technologies, including gamma knife and focused ultrasound, are emerging, but are yet to have significant impact as measured by publication numbers. Pallidotomy and thalamotomy are prominent examples of procedures that were popular, declined, and re-emerged and redeclined. Transplantation and gene therapy have never broken into clinical practice. DBS overtook all procedures as the dominant surgical intervention and drove widespread use of surgery for PD. Notwithstanding, the number of DBS articles appears to have plateaued. As advances continue, emerging treatments may compete with DBS in the future. © 2017 International Parkinson and Movement Disorder Society.

The Pioneering and Unknown Stereotactic Approach of Roeder and Orthner from Göttingen. Part II: Long-Term Outcome and Postmortem Analysis of Bilateral Pallidotomy in the Pre-Levodopa Era.

Before the advent of levodopa, pallidotomy was initially the most effective treatment for Parkinson disease, but it was soon superseded by thalamotomy. It is widely unknown that, similar to Leksell, 2 neurologists from Göttingen, Orthner and Roeder, perpetuated pallidotomy against the mainstream of their time. Postmortem studies demonstrated that true posterior and ventral pallidoansotomy sparing the overwhelming mass of the pallidum was accomplished. This was due to a unique and individually tailored stereotactic technique even allowing bilateral staged pallidotomies. In 1962, the long-term effects (3-year follow-up on average) of the first 18 out of 36 patients with staged bilateral pallidotomies were reported in great detail. Meticulous descriptions of each case indicate long-term improvements in parkinsonian rigidity and associated pain, as well as posture, gait, and akinesia (e.g., improved repetitive movements and arm swinging). Alleviation of tremor was found to require larger lesions than needed for suppression of rigidity. No improvement in speech, drooling, or seborrhea was observed. By 1962, the team had operated 13 patients with postencephalitic oculogyric crises with remarkable results (mean follow-up: 5 years). They also described alleviation of nonparkinsonian hyperkinetic disorders (e.g., hemiballism and chorea) with pallidotomy. The reported rates for surgical mortality and other complications had been remarkably low, even if compared to those reported after the revival of pallidotomy by Laitinen in the post-levodopa era. This applies also to bilateral pallidotomy performed with a positive risk-benefit ratio that has remained unparalleled to date. The intricate history of pallidotomy for movement disorders is incomplete without an appreciation of the achievements of the Göttingen group.

The advances of Deep Brain Stimulation (DBS) for treatment of Parkinson's disease (PD)

Advanced Parkinson's disease (PD) is characterized by the presence of motor fluctuations, various degrees of dyskinesia, and disability with functional impact on daily living and independence. Therapeutic management aims to extend levodopa (L-DOPA) benefit while minimizing motor complications and includes, in selected cases, the implementation of drug infusion and surgical techniques. The concept of deep brain stimulation (DBS) for PD was introduced over 20 years ago, but our understanding of the nuances of this procedure continues to improve. This review aims to demonstrate the advances of DBS in the treatment of PD patients. (AU)

[Unilateral posteroventral pallidotomy in the treatment of drug-induced dyskinesia in Parkinson's disease]. / Odnostoronniaia posteroventral'naia pallidotomiia v lechenii lekarstvennykh diskinezii pri bolezni Parkinsona.

OBJECTIVE: to determine the efficacy of unilateral posteroventral pallidotomy (PVP) in the treatment of drug-induced dyskinesia (DID) in Parkinson's disease (PD). MATERIAL AND METHODS: We analyzed surgical treatment of 14 patients with PD complicated by DID who underwent unilateral PVP at the Research Center of Neurology in the period between 2012 and 2015. The clinical type of DID was mainly represented by peak-dose choreoathetoid dyskinesia, more pronounced in the distal limbs, and predominantly unilateral. The severity of drug-induced dyskinesia was assessed on the UPDRS scale (part IV-A) before surgery and at 1 week and 6 months after surgery. RESULTS: One week after pallidotomy, all of the 14 patients had a regression of contralateral dyskinesia by 68.3±9.7%; 50% of patients had a regression of ipsilateral dyskinesias by 43%, on average. In 50% of cases, the dose of levodopa was reduced by 15%, on average. On examination at 6 months after surgery, regression of contralateral dyskinesia was 55.7±8.8%, and the severity of ipsilateral DID returned to the preoperative level. The use of pallidotomy significantly improved the indicators of daily activity and quality of life of patients. There were no significant postoperative complications. Three patients had mild speech disorders in the form of dysarthria, which regressed 2-3 weeks after surgery.

Understanding and Treating Pain Syndromes in Parkinson's Disease.

Pain affects many people with Parkinson's disease (PD) and diminishes their quality of life. Different types of pain have been described, but their related pathophysiological mechanisms remain unclear. The aim of this chapter is to provide movement disorders specialists an update about the pathophysiology of pain and a practical guide for the management of pain syndromes in clinical practice. This chapter reviews current knowledge on the pathophysiological mechanisms of sensory changes and pain in PD, as well as assessment and treatment procedures to manage these symptoms. In summary, changes in peripheral and central pain processing have been demonstrated in PD patients. A decrease in pain threshold and tolerance to several stimuli, a reduced nociceptive withdrawal reflex, a reduced pain threshold, and abnormal pain-induced activation in cortical pain-related areas have been reported. There is no direct association between improvement of motor symptoms and sensory/pain changes, suggesting that motor and nonmotor symptoms do not inevitably share the same mechanisms. Special care in pain assessment in PD is warranted by the specific pathophysiological aspects and the complexity of motor and nonmotor symptoms associated with pain symptoms. Rehabilitation may represent a valid option to manage pain syndromes in PD. However, further research in this field is needed. An integrated approach to pain involving a multidisciplinary team of medical specialists and rehabilitation experts should allow a comprehensive approach to pain in PD.