Bilateral Pallidal Stimulation with Directional Leads for Primary Focal Lingual Dystonia

Author(s):  
Takashi Asahi ◽  
Kiyonobu Ikeda ◽  
Jiro Yamamoto ◽  
Hiroyuki Tsubono ◽  
Yuko Muro ◽  
...  

There have been limited studies regarding stereotactic and functional neurosurgery for lingual dystonia. Here, we report a patient with primary lingual dystonia who showed significant improvement after bilateral deep brain stimulation (DBS). A 42-year-old woman presented with a 5- to 6-year history of tongue protrusion; however, she lacked a significant medical or medication history before onset. She presented with gradually worsening symptoms and was diagnosed with idiopathic lingual dystonia. Her tongue was injected with botulinum toxin on 6 occasions; however, it had a limited effect. Oral medications were ineffective. She underwent DBS since her involuntary tongue movements were causing nocturnal breathing problems. Directional leads were bilaterally inserted into the internal segment of the globus pallidus (GPi). The directional part of each lead was inserted at the GPi bottom on both sides. The posteromedial contacts were used to deliver stimulation. After 1.5 years, the patient’s Burke-Fahn-Marsden dystonia rating scale score improved from 9 to 1.5 and 2 to 1 for movement and disability, respectively. This case demonstrated the effectiveness of bilateral GPi-DBS. Placing the directional part of the lead in the GPi bottom could improve the stimulation effects.

2017 ◽  
Vol 43 (1) ◽  
pp. E2 ◽  
Author(s):  
Victor Goulenko ◽  
Paulo Luiz da Costa Cruz ◽  
Paulo Niemeyer Filho

Pallidal stimulation has been the usual surgical treatment for dystonia in the last decades. The continuous investigation of the physiopathology and the motor pathways involved leads to the search for complementary targets to improve results. The authors present the case of a 37-year-old woman who had suffered from idiopathic hemidystonia with hyperkinetic and hypokinetic movements for 11 years, and who was treated with deep brain stimulation. A brief literature review is also provided. The globus pallidus internus and the ventral intermediate/ventral oral posterior complex of the thalamus were stimulated separately and simultaneously for 3 months and compared using the Burke-Fahn-Marsden Dystonia Rating Scale and the Global Dystonia Severity Rating Scale, with a 3.5-year follow-up. The synergism of multiple-target stimulation resulted in a complete improvement of the mixed dystonic symptoms.


2021 ◽  
Vol 15 ◽  
Author(s):  
Shiro Horisawa ◽  
Kotaro Kohara ◽  
Masato Murakami ◽  
Atsushi Fukui ◽  
Takakazu Kawamata ◽  
...  

The field of Forel (FF) is a subthalamic area through which the pallidothalamic tracts originating from the globus pallidus internus (GPi) traverse. The FF was used as a stereotactic surgical target (ablation and stimulation) to treat cervical dystonia in the 1960s and 1970s. Although recent studies have reappraised the ablation and stimulation of the pallidothalamic tract at FF for Parkinson’s disease, the efficacy of deep brain stimulation of FF (FF-DBS) for dystonia has not been well investigated. To confirm the efficacy and stimulation-induced adverse effects of FF-DBS, three consecutive patients with medically refractory dystonia who underwent FF-DBS were analyzed (tongue protrusion dystonia, cranio-cervico-axial dystonia, and hemidystonia). Compared to the Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale scores before surgery (23.3 ± 12.7), improvements were observed at 1 week (8.3 ± 5.9), 3 months (5.3 ± 5.9), and 6 months (4.7 ± 4.7, p = 0.0282) after surgery. Two patients had stimulation-induced complications, including bradykinesia and postural instability, all well controlled by stimulation adjustments.


Author(s):  
Marta San Luciano ◽  
Amy Robichaux-Viehoever ◽  
Kristen A. Dodenhoff ◽  
Melissa L. Gittings ◽  
Aaron C. Viser ◽  
...  

OBJECTIVEThe aim of this study was to evaluate the feasibility and preliminary efficacy and safety of combined bilateral ventralis oralis posterior/ventralis intermedius (Vop/Vim) deep brain stimulation (DBS) for the treatment of acquired dystonia in children and young adults. Pallidal DBS is efficacious for severe, medication-refractory isolated dystonia, providing 50%–60% long-term improvement. Unfortunately, pallidal stimulation response rates in acquired dystonia are modest and unpredictable, with frequent nonresponders. Acquired dystonia, most commonly caused by cerebral palsy, is more common than isolated dystonia in pediatric populations and is more recalcitrant to standard treatments. Given the limitations of pallidal DBS in acquired dystonia, there is a need to explore alternative brain targets. Preliminary evidence has suggested that thalamic stimulation may be efficacious for acquired dystonia.METHODSFour participants, 3 with perinatal brain injuries and 1 with postencephalitic symptomatic dystonia, underwent bilateral Vop/Vim DBS and bimonthly evaluations for 12 months. The primary efficacy outcome was the change in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Barry-Albright Dystonia Scale (BADS) scores between the baseline and 12-month assessments. Video documentation was used for blinded ratings. Secondary outcomes included evaluation of spasticity (Modified Ashworth Scale score), quality of life (Pediatric Quality of Life Inventory [PedsQL] and modified Unified Parkinson’s Disease Rating Scale Part II [UPDRS-II] scores), and neuropsychological assessments. Adverse events were monitored for safety.RESULTSAll participants tolerated the procedure well, and there were no safety concerns or serious adverse events. There was an average improvement of 21.5% in the BFMDRS motor subscale score, but the improvement was only 1.6% according to the BADS score. Following blinded video review, dystonia severity ratings were even more modest. Secondary outcomes, however, were more encouraging, with the BFMDRS disability subscale score improving by 15.7%, the PedsQL total score by 27%, and the modified UPDRS-II score by 19.3%. Neuropsychological assessment findings were unchanged 1 year after surgery.CONCLUSIONSBilateral thalamic neuromodulation by DBS for severe, medication-refractory acquired dystonia was well tolerated. Primary and secondary outcomes showed highly variable treatment effect sizes comparable to those of pallidal stimulation in this population. As previously described, improvements in quality of life and disability were not reflected in dystonia severity scales, suggesting a need for the development of scales specifically for acquired dystonia.Clinical trial registration no.: NCT03078816 (clinicaltrials.gov)


Toxins ◽  
2019 ◽  
Vol 11 (1) ◽  
pp. 51 ◽  
Author(s):  
Kazuya Yoshida

Lingual dystonia is a debilitating type of oromandibular dystonia characterized by involuntary, often task-specific, contractions of the tongue muscle activated by speaking or eating. Botulinum neurotoxin (BoNT) has been used to treat lingual dystonia; however, it is known to cause serious complications, such as dysphasia and aspiration. The purpose of this study was to evaluate the efficacy and adverse effects of individualized BoNT therapy for lingual dystonia. One-hundred-and-seventy-two patients (102 females and 70 males, mean age: 46.2 years) with lingual dystonia were classified into four subtypes based on symptoms of involuntary tongue movements: protrusion (68.6%), retraction (16.9%), curling (7.6%), and laterotrusion (7.0%). Patients were treated with BoNT injection into the genioglossus and/or intrinsic muscles via individualized submandibular and/or intraoral routes. Results were compared before and after BoNT therapy. Botulinum neurotoxin was injected in 136 patients (mean: 4.8 injections). Clinical sub-scores (mastication, speech, pain, and discomfort) in a disease-specific rating scale were reduced significantly (p < 0.001) after administration. Comprehensive improvement after BoNT injection, assessed using the rating scale, was 77.6%. The curling type (81.9%) showed the greatest improvement, while the retraction type showed the least improvement (67.9%). Mild and transient dysphasia occurred in 12.5% of patients (3.7% of total injections) but disappeared spontaneously within several days to two weeks. No serious side effects were observed. With careful diagnosis of subtypes and a detailed understanding of lingual muscle anatomy, individualized BoNT injection into dystonic lingual muscles can be effective and safe.


Author(s):  
H.A. Eltahawy ◽  
J. Saint-Cyr ◽  
Y.Y. Poon ◽  
E. Moro ◽  
A.E. Lang ◽  
...  

Objective:Report on the clinical results following bilateral globus pallidus interna deep brain stimulation in four patients (one female and three males) with severe cervical dystonia, mean age 48 years (range 37-67).Methods:All four patients had failed extensive medical and botulinum toxin treatment. The mean duration of the disease was nine years (range 4-15 years). Patients were assessed pre and postoperatively using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Preoperatively, the mean TWSTRS total score was 43.2 (range 28-60.5). Posteroventral pallidal deep brain stimulators were inserted using MRI and microelectrode recording guidance. Last follow-up was 15 months for the four patients.Results:Mean reduction in the TWSTRS total scores at last follow- up was 73% (range 61- 85%). Improvement in pain occurred soon after deep brain stimulation surgery. Motor improvement was delayed and prolonged over several months. Frequent adjustment in the stimulation parameters was necessary in the first three months.Conclusion:Bilateral pallidal stimulation is effective in management of selected cases of intractable cervical dystonia.


2004 ◽  
Vol 100 (4) ◽  
pp. 706-709 ◽  
Author(s):  
Atsushi Umemura ◽  
Jurg L. Jaggi ◽  
Carol A. Dolinskas ◽  
Matthew B. Stern ◽  
Gordon H. Baltuch

✓ Generalized dystonia is one of the most disabling movement disorders. Ablative stereotactic surgery such as pallidotomy has been performed for medically refractory dystonia. Recently, deep brain stimulation (DBS) has appeared as an alternative to ablative procedures. Nevertheless, there have been few published reports detailing improvement in dystonia with DBS. This 36-year-old man with Hallervorden—Spatz syndrome suffered from intractable primary generalized dystonia for 28 years. He was completely dependent for activities of daily living and wheelchair bound because of continuous severe dystonic movements in the face, tongue, neck, trunk, and upper and lower extremities while at rest. The Burke-Fahn-Marsden (BFM) Dystonia Rating Scale score was 112 (maximum 120 points). Bilateral DBS of the globus pallidus internus was performed and resulted in marked improvement in motor functioning and dystonic symptoms with a significant reduction in disability. The BFM score improved to 22.5 points (80% improvement) at 3 months postsurgery and the patient's dystonia was still well suppressed 1 year after surgery. Bilateral pallidal stimulation is an effective and safe treatment for intractable generalized dystonia in Hallervorden—Spatz syndrome, even if the disability is severe and longstanding.


Neurosurgery ◽  
2011 ◽  
Vol 69 (2) ◽  
pp. 357-361 ◽  
Author(s):  
Michael S. Okun ◽  
Samuel S. Wu ◽  
Kelly D. Foote ◽  
Dawn Bowers ◽  
Shilpa Gogna ◽  
...  

Abstract BACKGROUND: Deep brain stimulation (DBS) has been associated with mood sequelae in a subset of patients operated on in either the subthalamic nucleus or the globus pallidus internus for the treatment of Parkinson disease. OBJECTIVE: To compare mood and motor outcomes in those with and without a presurgical history of depression. METHODS: Unilateral subthalamic nucleus or unilateral globus pallidus internus DBS patients followed up for a minimum of 6 months were included. All patients underwent a comprehensive outpatient psychiatric evaluation by a board-certified psychiatrist. Psychiatric diagnoses were based on Diagnostic and Statistical Manual, fourth edition, text revision, nomenclature (American Psychiatric Association, 2000). Motor and mood outcomes were compared. RESULTS: A total of 110 patients were included. There were no significant differences in baseline variables between the 2 groups. Those with a preoperative history of depression had significantly higher Beck Depression Inventory scores than the nondepression group after DBS (8.97 ± 7.55 vs 5.92 ± 5.71; P = .04). Patients with a depression history had less improvement (11.6%) in pre/post-DBS change when Unified Parkinson Disease Rating Scale motor scores were compared (P = .03) after adjustment for stimulation site and baseline demographic and clinical variables. Patients with a higher levodopa equivalent dose had a worse clinical motor outcome. CONCLUSION: Patients with a preoperative depression history had higher Beck Depression Inventory scores after DBS and significantly less (albeit small) improvement in pre/post-DBS change in Unified Parkinson Disease Rating Scale motor scores than patients without a history of depression.


Neurosurgery ◽  
2006 ◽  
Vol 59 (3) ◽  
pp. E702-E702 ◽  
Author(s):  
Hans-Holger Capelle ◽  
Eva Grips ◽  
Ralf Weigel ◽  
Christian Blahak ◽  
Baezner Hansjörg ◽  
...  

Abstract OBJECTIVE: We report on the effect of multifocal deep brain stimulation for the treatment of posttraumatic peripherally-induced dystonia. CLINICAL PRESENTATION: A 34-year-old woman presented with an 8-year history of painful tonic dystonia starting in her left leg after injury of the third metatarsal bone. She did not benefit from right-sided pallidal stimulation by an electrode misplaced in the globus pallidus externus in another hospital. INTERVENTION: Quadripolar deep brain stimulation electrodes were placed in the globus pallidus internus and the ventrolateral thalamus by computed tomographic-guided stereotactic surgery and microelectrode recording contralateral to the side of dystonia. The Burke-Fahn-Marsden motor score of 34 did not improve with chronic pallidal or thalamic stimulation. CONCLUSION: Although deep brain stimulation is received with great enthusiasm, it is important to identify its limitations in certain subtypes of dystonia.


1981 ◽  
Vol 139 (6) ◽  
pp. 526-528 ◽  
Author(s):  
Edmond H. Pi ◽  
George M. Simpson

SummaryForty-two subjects, 20 with and 22 without clinically diagnosed tardive dyskinesia (TD), were rated for TD on the scale of Simpson et al. They also performed a tongue protrusion test and had the presence of tongue movements within the buccal cavity observed. Fifty per cent of the severe or definite TD subjects as classified by rating scale were unable to maintain tongue protrusion for a minimum of 30 seconds, and 85 per cent of them had abnormal tongue movements. In 45 per cent of the possible TDs and in 9 per cent of the doubtful subjects, the movement test was also positive.


2014 ◽  
Vol 14 (6) ◽  
pp. 585-593 ◽  
Author(s):  
Joseph R. Keen ◽  
Allison Przekop ◽  
Joffre E. Olaya ◽  
Alexander Zouros ◽  
Frank P. K. Hsu

Object Deep brain stimulation (DBS) for dystonic cerebral palsy (CP) has rarely been reported, and its efficacy, though modest when compared with that for primary dystonia, remains unclear, especially in the pediatric population. The authors present a small series of children with dystonic CP who underwent bilateral pallidal DBS, to evaluate the treatment's efficacy and safety in the pediatric dystonic CP population. Methods The authors conducted a retrospective review of patients (under the age of 18 years) with dystonic CP who had undergone DBS of the bilateral globus pallidus internus between 2010 and 2012. Two of the authors independently assessed outcomes using the Barry-Albright Dystonia Scale (BADS) and the Burke-Fahn-Marsden Dystonia Rating Scale–movement (BFMDRS-M). Results Five children were diagnosed with dystonic CP due to insults occurring before the age of 1 year. Mean age at surgery was 11 years (range 8–17 years), and the mean follow-up was 26.6 months (range 2–42 months). The mean target position was 20.6 mm lateral to the midcommissural point. The mean preoperative and postoperative BADS scores were 23.8 ± 4.9 (range 18.5–29.0) and 20.0 ± 5.5 (range 14.5–28.0), respectively, with a mean overall percent improvement of 16.0% (p = 0.14). The mean preoperative and postoperative BFMDRS-M scores were 73.3 ± 26.6 (range 38.5–102.0) and 52.4 ± 21.5 (range 34.0–80.0), respectively, with a mean overall percent improvement of 28.5% (p = 0.10). Those stimulated at least 23 months (4 patients) improved 18.3% (p = 0.14) on the BADS and 30.5% (p = 0.07) on the BFMDRS-M. The percentage improvement per body region yielded conflicting results between rating scales; however, BFMDRS-M scores for speech showed some of the greatest improvements. Two patients required hardware removal (1 complete system, 1 unilateral electrode) within 4 months after implantation because of infections that resolved with antibiotics. Conclusions All postoperative dystonia rating scale scores improved with pallidal stimulation, and the greatest improvements occurred in those stimulated the longest. The results were modest but comparable to findings in other similar series. Deep brain stimulation remains a viable treatment option for childhood dystonic CP, although young children may have an increased risk of infection. Of particular note, improvements in the BFMDRS-M subscores for speech were comparable to those for other muscle groups, a finding not previously reported.


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