Long-term follow-up study of chronic globus pallidus internus stimulation for posttraumatic hemidystonia

2000 ◽  
Vol 92 (3) ◽  
pp. 457-460 ◽  
Author(s):  
Thomas J. Loher ◽  
Mustafa G. Hasdemir ◽  
Jean-Marc Burgunder ◽  
Joachim K. Krauss

✓ The authors report the first case of chronic globus pallidus internus (GPi) stimulation for treatment of medically intractable hemidystonia for which long-term follow-up data are available. The patient had developed left-sided low-frequency tremor and hemidystonia after a severe head trauma sustained at 15 years of age. He experienced relief of the tremor but not of the hemidystonia after a thalamotomy was performed in the right hemisphere 3 years postinjury. When the patient was 24 years old, the authors performed a magnetic resonance-guided stereotactic implantation of a monopolar electrode in the right-sided posteroventral GPi. Chronic deep brain stimulation resulted in remarkable improvement of dystonia-associated pain, phasic dystonic movements, and dystonic posture, which was accompanied by functional gain. Postoperative improvement was sustained after 4 years of follow up. Chronic GPi stimulation appears to be a valuable treatment option for posttraumatic dystonia.

2004 ◽  
Vol 101 (2) ◽  
pp. 189-194 ◽  
Author(s):  
Philippe Coubes ◽  
Laura Cif ◽  
Hassan El Fertit ◽  
Simone Hemm ◽  
Nathalie Vayssiere ◽  
...  

Object. Primary generalized dystonia (PGD) is a medically refractory disease of the brain causing twisting or spasmodic movements and abnormal postures. In more than 30% of cases it is associated with the autosomal DYT1 mutation. Continuous electrical stimulation of the globus pallidus internus (GPi) has been used successfully in the treatment of PGD. The aim of this study was to examine the long-term efficacy and safety of deep brain stimulation (DBS) in the treatment of PGD in children and adults with and without the DYT1 mutation. Methods. Thirty-one patients with PGD were selected for surgery. Electrodes were bilaterally implanted under stereotactic guidance and connected to neurostimulators that were inserted subcutaneously. Efficacy was evaluated by comparing scores on the clinical and functional Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) before and after implantation. The efficacy of stimulation improved with time. After 2 years, compared with preoperative values, the mean (± standard deviation) clinical and functional BFMDRS scores had improved by 79 ± 19% and 65 ± 33%, respectively. At the 2-year follow-up examination the improvement was comparable in patients with and without the DYT1 mutation in both the functional (p = 0.12) and clinical (p = 0.33) scores. Children displayed greater improvements in the clinical score than adult patients (p = 0.04) at 2 years of follow up. In contrast, there was no significant difference in functional scores between children and adults (p = 0.95). Conclusions. Electrical stimulation of the GPi is an effective, reversible, and adaptable treatment for PGD and should be considered for conditions refractory to pharmaceutical therapies.


2017 ◽  
Vol 14 (5) ◽  
pp. E53-E57 ◽  
Author(s):  
Derrick A Dupre ◽  
Raj Nangunoori ◽  
Sravanthi Koduri ◽  
Cindy Angle ◽  
Diane Cantella ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Primary generalized dystonia (PGD) is a genetic form of dystonia that frequently displays pharmacological resistance and progresses quickly after onset. Deep brain stimulation (DBS) has been used successfully to treat refractory dystonia, specifically globus pallidus interna (GPi) DBS for DYT1-positive PGD patients. Long-term follow-up of the safety and efficacy falls short of the longevity seen in other diseases treated with DBS. CASE PRESENTATION A male patient presented for neurosurgical evaluation with scapular winging, hand contractures, and violent truncal spasms, which forced him to be bedridden. After failing conservative therapy, the 18-yr-old patient was implanted with bilateral GPi-DBS. DBS parameter adjustments were made primarily within the first 3 yr after implantation, with nominal changes thereafter. Initial settings were contact of 3 + 0−, amplitude of 4.9 V, frequency of 185 Hz, and pulse width of 270 μsec on the left and 3 + 0−, 2.8 V, 185 Hz, and 120 μsec on the right. Current settings are 3 + 2 + 1−, 5.2 V, 130 Hz, 330 μsec on the left and 3 + 0−, 3.5 V, 185 Hz, and 180 μsec on the right and have been relatively unchanged in the past 4 yr. Unified dystonia rating scale scores reveal a significant decrease in dystonic symptoms. CONCLUSION While prior reports have shown that GPi-DBS is effective for dystonia, this is the first with 15 yr of long-term follow-up showing disease stabilization, suggesting that stimulation is efficacious and can potentially prevent disease progression. This report reaffirms previous reports that recommend early surgical intervention before the onset of permanent musculoskeletal deficits.


1994 ◽  
Vol 81 (1) ◽  
pp. 133-136 ◽  
Author(s):  
Gregg B. Wells ◽  
Todd M. Lasner ◽  
David M. Yousem ◽  
Eric L. Zager

✓ Recent reports of seven cases of Lhermitte-Duclos disease occurring in adult patients with Cowden's syndrome (multiple hamartoma syndrome) strongly suggest that Lhermitte-Duclos disease is one of the types of neoplasia that characterize this syndrome. A case of Lhermitte-Duclos disease is reported in a 16-year-old girl with craniomegaly, choroidal hamartoma, and conjunctival papilloma of the right eye, and a history of bilateral multinodular adenomatous goiter and cystic hygroma. These findings strongly suggest a diagnosis of Cowden's syndrome. Although the syndrome traditionally has been defined by mucocutaneous criteria, it typically also involves hamartomas and neoplasia of internal organs, most commonly in the thyroid, breast, and female genitourinary tract. Because the mucocutaneous features may develop several decades after birth, the present case both supports the previously reported association between Lhermitte-Duclos disease and Cowden's syndrome and highlights the need for long-term follow-up monitoring of a pediatric patient with Lhermitte-Duclos disease because of the risk of malignancies associated with Cowden's syndrome.


2021 ◽  
Vol 49 (3) ◽  
pp. 030006052110005
Author(s):  
Qiaochu Sun ◽  
Yoo-Duk Choi ◽  
Young Kim

Solitary fibrous tumors (SFTs) are rare benign mesenchymal tumors that occur mainly in the pleura. We herein report the first case of a cellular SFT located in the mental region of the head and neck in a 46-year-old woman. Facial computed tomography revealed a mass measuring 0.8 cm with clear boundaries in the right mental region. After excision of the mass, expert pathologists diagnosed a cellular SFT. To our knowledge, this is the first case of a cellular SFT identified in the subcutaneous tissue of the mental region of the head and neck. Because the postsurgical prognosis of SFTs is unpredictable, long-term follow-up and further studies are necessary to determine the characteristics of cellular SFTs in the head and neck region.


2021 ◽  
pp. 263246362097804
Author(s):  
Vanita Arora ◽  
Pawan Suri

Anatomy and physiology are the basis of human body functioning and as we have progressed in management of various diseases, we have understood that physiological intervention is always better than an anatomical one. For more than 50 years, a standard approach to permanent cardiac pacing has been an anatomical placement of transvenous pacing lead at the right ventricular apex with a proven benefit of restoring the rhythm. However, the resultant ventricular dyssynchrony on the long-term follow-up in patients requiring more than 40% ventricular pacing led to untoward side effects in the form of heart failure and arrhythmias. To counter such adverse side effects, a need for physiological cardiac pacing wherein the electrical impulse be transmitted directly through the normal conduction system was sought. His bundle pacing (HBP) with an intriguing alternative of left bundle branch pacing (LBBP) is aimed at restoring such physiological activation of ventricles. HBP is safe, efficacious, and feasible; however, localization and placement of a pacing lead at the His bundle is challenging with existing transvenous systems due to its small anatomic size, surrounding fibrous tissue, long-learning curve, and the concern remains about lead dislodgement and progressive electrical block distal to the HBP lead. In this article, we aim to take the reader through the challenging journey of HBP with focus upon the hardware and technique, selective versus nonselective HBP, indications and potential disadvantages, and finally the future prospects.


2002 ◽  
Vol 12 (3) ◽  
pp. 224-228 ◽  
Author(s):  
Haifa Abdul Latiff ◽  
Mazeni Alwi ◽  
Hasri Samion ◽  
Geetha Kandhavel

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.


2017 ◽  
Vol 22 (02) ◽  
pp. 232-235
Author(s):  
Seigo Suganuma ◽  
Kaoru Tada ◽  
Daiki Yamamoto ◽  
Hiroyuki Tsuchiya

The Galeazzi-equivalent fracture is a rare injury that occurs in children. The most important issue is the distal ulnar epiphyseal injury. Although there have been some case reports, most of them performed only short term follow-up. This article describes two cases of this fracture with long term follow-up until epiphyseal closure. First case is a 12-year-old girl who sustained a Galeazziequivalent fracture of her right forearm and underwent emergency surgery. At follow-up of 5 years and 10 months postsurgery, radiographs show ulnar growth arrest of one mm and she has mild pain. Second case is a 15-year-old boy who sustained an open Galeazzi-equivalent fracture of his left forearm and underwent emergency surgery. At follow-up of 3 years and 3 months postsurgery, radiographs show no growth arrest of the distal ulna. He has no residual complaint. Long term follow-up is absolutely necessary to monitor ulnar growth.


2020 ◽  
Vol 30 (3) ◽  
pp. 409-412
Author(s):  
Murat Surucu ◽  
İlkay Erdoğan ◽  
Birgül Varan ◽  
Murat Özkan ◽  
N. Kürşad Tokel ◽  
...  

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.


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