Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term results

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.

2003 ◽  
Vol 98 (4) ◽  
pp. 785-792 ◽  
Author(s):  
Joachim K. Krauss ◽  
Thomas J. Loher ◽  
Ralf Weigel ◽  
H. Holger Capelle ◽  
Sabine Weber ◽  
...  

Object. The authors studied the long-term efficacy of deep brain stimulation (DBS) of the posteroventral lateral globus pallidus internus up to 2 years postoperatively in patients with primary non-DYT1 generalized dystonia or choreoathetosis. The results are briefly compared with those reported for DBS in DYT1 dystonia (Oppenheim dystonia), which is caused by the DYT1 gene. Methods. Enrollment in this prospective expanded pilot study was limited to adult patients with severely disabling, medically refractory non-DYT1 generalized dystonia or choreoathetosis. Six consecutive patients underwent follow-up examinations at defined intervals of 3 months, 1 year, and 2 years postsurgery. There were five women and one man, and their mean age at surgery was 45.5 years. Formal assessments included both the Burke-Fahn-Marsden dystonia scale and the recently developed Unified Dystonia Rating Scale. Two patients had primary generalized non-DYT1 dystonia, and four suffered from choreoathetosis secondary to infantile cerebral palsy. Bilateral quadripolar DBS electrodes were implanted in all instances, except in one patient with markedly asymmetrical symptoms. There were no adverse events related to surgery. The Burke-Fahn-Marsden scores in the two patients with generalized dystonia improved by 78 and 71% at 3 months, by 82 and 69% at 1 year, and by 78 and 70% at 2 years postoperatively. This was paralleled by marked amelioration of disability scores. The mean improvement in Burke-Fahn-Marsden scores in patients with choreoathetosis was 12% at 3 months, 29% at 1 year, and 23% at 2 years postoperatively, which was not significant. Two of these patients thought that they had achieved marked improvement at 2 years postoperatively, although results of objective evaluations were less impressive. In these two patients there was a minor but stable improvement in disability scores. All patients had an improvement in pain scores at the 2-year follow-up review. Medication was tapered off in both patients with generalized dystonia and reduced in two of the patients with choreoathetosis. All stimulation-induced side effects were reversible on adjustment of the DBS settings. Energy consumption of the batteries was considerably higher than in patients with Parkinson disease. Conclusions. Chronic pallidal DBS is a safe and effective procedure in generalized non-DYT1 dystonia, and it may become the procedure of choice in patients with medically refractory dystonia. Postoperative improvement of choreoathetosis is more modest and varied, and subjective ratings of outcome may exceed objective evaluations.


2004 ◽  
Vol 100 (6) ◽  
pp. 997-1001 ◽  
Author(s):  
Mitsuhiro Ogura ◽  
Naoyuki Nakao ◽  
Ekini Nakai ◽  
Yuji Uematsu ◽  
Toru Itakura

Object. Although chronic electrical stimulation of the globus pallidus (GP) has been shown to ameliorate motor disabilities in Parkinson disease (PD), the underlying mechanism remains to be clarified. In this study the authors explored the mechanism for the effects of deep brain stimulation of the GP by investigating the changes in neurotransmitter levels in the cerebrospinal fluid (CSF) during the stimulation. Methods. Thirty patients received chronic electrical stimulation of the GP internus (GPi). Clinical effects were assessed using the Unified PD Rating Scale (UPDRS) and the Hoehn and Yahr Staging Scale at 1 week before surgery and at 6 and 12 months after surgery. One day after surgery, CSF samples were collected through a ventricular tube before and 1 hour after GPi stimulation. The concentration of neurotransmitters such as γ-aminobutyric acid (GABA), noradrenaline, dopamine, and homovanillic acid (HVA) in the CSF was measured using high-performance liquid chromatography. The treatment was effective for tremors, rigidity, and drug-induced dyskinesia. The concentration of GABA in the CSF increased significantly during stimulation, although there were no significant changes in the level of noradrenaline, dopamine, and HVA. A comparison between an increased rate of GABA concentration and a lower UPDRS score 6 months postimplantation revealed that the increase in the GABA level correlated with the stimulation-induced clinical effects. Conclusions. Stimulation of the GPi substantially benefits patients with PD. The underlying mechanism of the treatment may involve activation of GABAergic afferents in the GP.


2001 ◽  
Vol 94 (5) ◽  
pp. 757-764 ◽  
Author(s):  
José Guimarães-Ferreira ◽  
Fredrik Gewalli ◽  
Pelle Sahlin ◽  
Hans Friede ◽  
Py Owman-Moll ◽  
...  

Object. Brachycephaly is a characteristic feature of Apert syndrome. Traditional techniques of cranioplasty often fail to produce an acceptable morphological outcome in patients with this condition. In 1996 a new surgical procedure called “dynamic cranioplasty for brachycephaly” (DCB) was reported. The purpose of the present study was to analyze perioperative data and morphological long-term results in patients with the cranial vault deformity of Apert syndrome who were treated with DCB. Methods. Twelve patients have undergone surgery performed using this technique since its introduction in 1991 (mean duration of follow-up review 60.2 months). Eleven patients had bicoronal synostosis and one had a combined bicoronal—bilambdoid synostosis. Perioperative data and long-term evolution of skull shape visualized on serial cephalometric radiographs were analyzed and compared with normative data. Changes in mean skull proportions were evaluated using a two-tailed paired-samples t-test, with differences being considered significant for probability values less than 0.01. The mean operative blood transfusion was 136% of estimated red cell mass (ERCM) and the mean postoperative transfusion was 48% of ERCM. The mean operative time was 218 minutes. The duration of stay in the intensive care unit averaged 1.7 days and the mean hospital stay was 11.8 days. There were no incidences of mortality and few complications. An improvement in skull shape was achieved in all cases, with a change in the mean cephalic index from a preoperative value of 90 to a postoperative value of 78 (p = 0.000254). Conclusions. Dynamic cranioplasty for brachycephaly is a safe procedure, yielding high-quality morphological results in the treatment of brachycephaly in patients with Apert syndrome.


1979 ◽  
Vol 50 (2) ◽  
pp. 179-186 ◽  
Author(s):  
Thomas R. Keucher ◽  
John Mealey

✓ This series of 228 patients with infantile non-neoplastic hydrocephalus who received either a ventriculoatrial (VA) or a ventriculoperitoneal (VP) shunt before 2 years of age was followed for an average of 7 years, and the results with the two types of shunt were compared. Mortality and infection rates were similar for both groups of patients, but children with VP shunts required significantly fewer revisions and had a much greater likelihood of not having any revisions during the follow-up period. Late complications occurred more frequently with VA shunts and were more serious. It is concluded that VP shunts offer significant advantages over VA systems in this population.


1994 ◽  
Vol 80 (5) ◽  
pp. 810-819 ◽  
Author(s):  
Joachim K. Krauss ◽  
Mohsen Mohadjer ◽  
Fritz Nobbe ◽  
Fritz Mundinger

✓ The authors report the long-term results of stereotactic surgery for severe posttraumatic appendicular tremor in 35 patients. The tremors developed after severe head trauma in 33 patients (94%) and after mild to moderate head trauma in two (6%). In all but one, the tremor was most evident during activity. The amplitude of the kinetic tremor was greater than 5 cm in 33 patients (94%) and greater than 12 cm in 19 patients (54%). All were severely incapacitated in their daily living activities due to the tremors. The 35 patients underwent 42 stereotactic operations; five patients were reoperated on the same side and two were treated with a bilateral staged procedure. The contralateral zona incerta was the stereotactic target in 12 patients and was targeted in combination with the base of the ventrolateral (oroventral) thalamus in 23 patients. Long-term postoperative follow-up review was obtained in 32 patients (mean follow-up period 10.5 years). Persistent improvement of tremor was noted in 88%. The tremor was absent or markedly reduced in 65%. Functional disability was assessed and quantified with a modified form of an established rating scale for patients with tremor; it was reduced from a mean value of 57% of maximum disability to 37% over the long term (p < 0.001). Follow-up lesion assessment was obtained in 18 patients by multiplanar magnetic resonance imaging and at autopsy in one patient whose death was unrelated to surgery. As in previous studies, the frequency of persistent side effects was relatively high (38%). These consisted mainly of aggravation of preoperative symptoms. The results are compared to those of a total of 55 patients reported from 1960 to 1992. The occurrence of dystonia and dystonic postures is discussed. Stereotactic surgery is a powerful tool to alleviate posttraumatic tremor and to improve functional disability. However, as there is considerable risk of persistent morbidity in patients after severe head trauma, the operation should be restricted to selected cases with disabling tremor.


2002 ◽  
Vol 96 (3) ◽  
pp. 527-531 ◽  
Author(s):  
Elizabeth C. Tyler-Kabara ◽  
Amin B. Kassam ◽  
Michael H. Horowitz ◽  
Louise Urgo ◽  
Constantinos Hadjipanayis ◽  
...  

Object. Microvascular decompression (MVD) has become one of the primary treatments for typical trigeminal neuralgia (TN). Not all patients with facial pain, however, suffer from the typical form of this disease; many patients who present for surgical intervention actually have atypical TN. The authors compare the results of MVD performed for typical and atypical TN at their institution. Methods. The results of 2675 MVDs in 2264 patients were reviewed using information obtained from the department database. The authors examined immediate postoperative relief in 2003 patients with typical and 672 with atypical TN, and long-term follow-up results in patients for whom more than 5 years of follow-up data were available (969 with typical and 219 with atypical TN). Outcomes were divided into three categories: excellent, pain relief without medication; good, mild or intermittent pain controlled with low-dose medication; and poor, no or poor pain relief with large amounts of medication. The results for typical and atypical TN were compared and patient history and pain characteristics were evaluated for possible predictive factors. Conclusions. In this study, MVD for typical TN resulted in complete postoperative pain relief in 80% of patients, compared with 47% with complete relief in those with atypical TN. Significant pain relief was achieved after 97% of MVDs in patients with typical TN and after 87% of these procedures for atypical TN. When patients were followed for more than 5 years, the long-term pain relief after MVD for those with typical TN was excellent in 73% and good in an additional 7%, for an overall significant pain relief in 80% of patients. In contrast, following MVD for atypical TN, the long-term results were excellent in only 35% of cases and good in an additional 16%, for overall significant pain relief in only 51%. Memorable onset and trigger points were predictive of better postoperative pain relief in both atypical and typical TN. Preoperative sensory loss was a negative predictor for good long-term results following MVD for atypical TN.


2019 ◽  
Vol 64 ◽  
pp. 118-123 ◽  
Author(s):  
M.L. Lachenmayer ◽  
C. Bettschen ◽  
C. Bernasconi ◽  
K. Petermann ◽  
I. Debove ◽  
...  

1996 ◽  
Vol 85 (2) ◽  
pp. 231-238 ◽  
Author(s):  
Manucher J. Javid

✓ This long-term prospective study evaluates the clinical results of subsequent laminectomy in 103 consecutive patients who initially underwent chemonucleolysis (CNL) or laminectomy for lumbar disc herniation. Between 1981 and 1994, 53 patients who had received CNL initially and then underwent laminectomy and 50 patients treated initially with laminectomy underwent a repeat laminectomy. Clinical assessment at 6 weeks showed a success rate of 80.8% for post-CNL laminectomy and 78% for repeat laminectomy. At 6 months, the success rate for patients treated with CNL was 86% versus 78.7% for laminectomy. At 12 months, the overall success rate for the CNL group was 80.4% versus 83.3% for the laminectomy group, but in patients who had not obtained relief from the first procedure the success rate for the second procedure was higher for the post-CNL patients. A questionnaire was sent to all patients for 1- to 13-year follow-up review. The average follow-up period was 6.6 years for post-CNL laminectomy and 5.2 years for repeat laminectomy. The long-term success rate (81.8%) was higher in the post-CNL group compared to 64.4% in the repeat laminectomy group. Seven patients in the post-CNL group and nine in the repeat laminectomy group had undergone a third operation. When these originally successfully treated patients were reassigned after unsuccessful outcomes, the success rate for the CNL groups was 72.7%, versus 51.1% in the laminectomy group (p = 0.049). Employment rates were 80% for patients with CNL (21.8% changed jobs) and 76.3% for patients undergoing laminectomy (48.3% changed jobs) (p = 0.036). In conclusion, patients who underwent laminectomies after receiving CNL had significantly better long-term results than those who had repeat laminectomies.


2001 ◽  
Vol 94 (1) ◽  
pp. 121-125 ◽  
Author(s):  
Al O. Taşçioǧlu ◽  
Ayhan Attar ◽  
Beliz Taşçioǧlu

✓ The authors present the long-term follow-up results in three cases in which pure lateral disc herniations were surgically treated via an anterior foraminotomy (uncinatectomy) that avoided entering the disc space. The pertinent anatomical details essential for a safe approach are discussed in light of nine cadaveric neck dissections of every segment between C-1 and C-7. The surgical technique was initially studied in the cadaveric specimens and then applied in patients. The long-term results (> 2 years) in the first three patients are as good as our short-term results in another eight cases.


1985 ◽  
Vol 62 (3) ◽  
pp. 389-396 ◽  
Author(s):  
Ronald F. Young ◽  
Richard Kroening ◽  
Wayne Fulton ◽  
Robert A. Feldman ◽  
Israel Chambi

✓ Forty-eight patients underwent electrical stimulation of the brain for treatment of chronic pain between 1978 and 1983. Average pain duration prior to treatment was 4.5 years. Before selection for this procedure patients underwent pain treatment in a multidisciplinary pain center, intensive psychological and psychiatric evaluation, and assessment of pain responsiveness to intravenous administration of placebo, morphine, and naloxone. A total of 71 electrodes were placed in the 48 patients at a variety of stimulating targets, including the periaqueductal gray matter, periventricular gray matter, thalamus, and internal capsule. Seventy-two percent of patients experienced complete or partial pain relief. In addition, 59% of patients were able to discontinue narcotic usage. Twenty-five percent of patients returned to normal physical activities and another 33% showed marked improvement in functional capacity. Follow-up periods ranged from 2 to 60 months; with a mean follow-up period of 20 months. A variety of relatively minor complications occurred, but no mortality or permanent sequelae were experienced. No patient's pain was made worse as a result of electrical stimulation. Electrical stimulation of the brain offers a safe and relatively effective method for the treatment of chronic pain in appropriately selected patients, who are unresponsive to other forms of therapy.


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