Benefit of bilateral pallidotomy in the treatment of generalized dystonia

1999 ◽  
Vol 90 (5) ◽  
pp. 974-976 ◽  
Author(s):  
Juei-Jueng Lin ◽  
Ging-Yau Lin ◽  
Chunhsi Shih ◽  
Shinn-Zong Lin ◽  
Dar-Cheng Chang ◽  
...  

✓ This 29-year-old man with cerebral palsy complicated by generalized dystonia was treated by simultaneous bilateral posteroventral pallidotomy. Postoperatively, there was slow, but steady, improvement in the patient's dystonia and disability. However, the improvement in abnormal movements was only prominent for cervical dystonia and oromandibular dyskinesia. The patient's Burke-Fahn-Marsden dystonia scores were 51 preoperatively and 37, 33.5 and 33.5, at 3, 6, and 12 months postoperatively, respectively, demonstrating a maximum improvement of 34%. These results suggest that pallidotomy can be an alternative therapy for those patients suffering from intractable generalized dystonia.

1998 ◽  
Vol 88 (1) ◽  
pp. 73-76 ◽  
Author(s):  
A. Leland Albright ◽  
Margaret J. Barry ◽  
Michael J. Painter ◽  
Barbara Shultz

Generalized dystonia occurs in 15 to 25% of persons with cerebral palsy (CP) and responds poorly to medical and surgical treatments. Object. After the authors observed a woman whose dystonic CP was dramatically improved by continuous infusion of intrathecal baclofen, they designed this pilot study to evaluate the effect of this treatment on a group of patients with dystonic CP. Methods. The authors assessed the short-term response to intrathecal baclofen infusion in 12 patients with dystonic CP. An intrathecal catheter was inserted percutaneously and connected to an external microinfusion pump. The infusion began at a rate of 100 µg/day and was increased by 50 µg every 12 hours until the dystonia abated, adverse effects occurred, or the dose reached 900 mg/day with no improvement. Two observers, one blinded and one not blinded to the patient's treatment status, viewed videotapes made before and after the infusions and graded the dystonia in eight body regions, using a 5-point scale. Overall and regional scores were compared by using Wilcoxon signed-rank tests. Conclusions. Dystonia diminished in 10 of 12 patients whose average daily dose of intrathecal baclofen was 575 µg. Overall dystonia scores and scores for the extremities, trunk, and cervical regions were significantly better after infusion (p = 0.003). The two observers' scores were not significantly different. Programmable infusion pumps were subsequently implanted in eight patients for long-term therapy and improvement was sustained in six (p < 0.05). Intrathecal baclofen infusion is a promising treatment option for generalized dystonia associated with CP. The effects of intrathecal baclofen infusion on dystonia can be evaluated by using short-term continuous infusions.


2000 ◽  
Vol 92 (5) ◽  
pp. 771-778 ◽  
Author(s):  
Peter A. Rasmussen ◽  
John Perl ◽  
John D. Barr ◽  
Georges Z. Markarian ◽  
Irene Katzan ◽  
...  

Object. Patients with intracranial vertebrobasilar artery (VBA) atherosclerotic occlusive disease have few therapeutic options. Unfortunately, VBA transient ischemic attacks (TIAs) herald a lethal or devastating event within 5 years in 25 to 30% of patients. The authors report their initial experience with eight patients in whom medically refractory TIAs secondary to intracranial posterior circulation atherosclerotic occlusive lesions were treated with stent-assisted angioplasty.Methods. Eight patients (six men), ranging in age from 43 to 77 years, experienced signs and symptoms of VBA insufficiency despite combination therapy with warfarin and antiplatelet agents. Angiographic studies revealed severe distal vertebral (four patients), proximal basilar (one patient), or proximal and midbasilar stenoses (three patients). Aspirin and clopidogrel were administered for 3 days before primary angioplasty and stent placement, and this regimen was maintained by the patients on discharge. Patients underwent heparinization during the procedure and were given a bolus and 12-hour infusion of abciximab. A neurologist specializing in stroke evaluated all patients before and after the procedure.The VBAs in all patients were successfully revascularized with 7 to 28% residual stenosis. Six patients experienced no neurological complications. One patient died the evening of the procedure due to a massive subarachnoid hemorrhage. Two patients had groin hematomas, one developed congestive heart failure, and one had transient encephalopathy. All surviving patients are asymptomatic up to 8 months postoperatively.Conclusions. Although primary intracranial VBA angioplasty with stent insertion is technically feasible, complications associated with the procedure can be life threatening. As experience is gained with this procedure, it may be offered routinely as an alternative therapy to patients with medically refractory posterior circulation occlusive disease that may develop into catastrophic VBA insufficiency.


1977 ◽  
Vol 46 (4) ◽  
pp. 506-511 ◽  
Author(s):  
Richard D. Penn ◽  
Mary Liz Etzel

✓ The changes in motor function in cerebral palsy patients produced by chronic anterior lobe cerebellar stimulation were documented with the Milani-Comparetti developmental scale, which allows comparison between functional gains and reflex patterns. Two patients with marked motor retardation and primitive reflexes were followed serially over several months of stimulation. Using these measures, the efficiency of chronic stimulation can be assessed, although many years will be needed to evaluate its role in treating motor disabilities.


1999 ◽  
Vol 91 (5) ◽  
pp. 727-732 ◽  
Author(s):  
Jack R. Engsberg ◽  
Sandy A. Ross ◽  
Tae Sung Park

Object. In this investigation the authors quantified changes in ankle plantarflexor spasticity and strength following selective dorsal rhizotomy (SDR) and intensive physical therapy in patients with cerebral palsy (CP).Methods. Twenty-five patients with cerebral palsy (CP group) and 12 able-bodied volunteers (AB controls) were tested with a dynamometer. For the spasticity measure, the dynamometer was used to measure the resistive torque of the plantarflexors during passive ankle dorsiflexion at five different speeds. Data were processed to yield a single value that simultaneously encompassed the three key elements associated with spasticity: velocity, resistance, and stretch. For the strength test, the dynamometer rotated the ankle from full dorsiflexion to full plantarflexion while a maximum concentric contraction of the plantarflexors was performed. Torque angle data were processed to include the work done by the patient or volunteer on the machine. Plantarflexor spasticity values for the CP group were significantly greater than similar values for the AB control group prior to surgery but not significantly different after surgery. Plantarflexor strength values of the CP group were significantly less than those of the AB control group pre- and postsurgery. Postsurgery strength values did not change relative to presurgery values.Conclusions. The spasticity results of the present investigation agreed with those of previous studies indicating a reduction in spasticity for the CP group. The strength results did not agree with the findings of most previous related literature, which indicated that a decrease in strength should have occurred. The strength results agreed with a previous investigation in which knee flexor strength was objectively examined, indicating that strength did not decrease as a consequence of an SDR. The methods of this investigation could be used to improve SDR patient selection.


2003 ◽  
Vol 99 (1) ◽  
pp. 78-88 ◽  
Author(s):  
Jason A. R. Carr ◽  
Christopher R. Honey ◽  
Marci Sinden ◽  
Anthony G. Phillips ◽  
Jeffrey S. Martzke

Object. The aim of this study was to examine neuropsychological outcome from unilateral posteroventral pallidotomy (PVP) in Parkinson disease while controlling for confounding factors such as test practice and disease progression. Methods. Participants underwent baseline and 2-month follow-up assessments of cognition, quality of life, mood, and motor functioning. The surgery group (22 patients) underwent PVP (15 left, seven right) after baseline assessment. The waitlist group (14 patients) underwent PVP after follow up. At follow up, the left PVP group exhibited a decline on verbal measures of learning, fluency, working memory, and speeded color naming. The incidence of significant decline on these measures after left PVP ranged from 50 to 86%. The right PVP group did not exhibit a significant cognitive decline, but fluency did decline in 71% of patients who underwent right PVP. Participants who underwent PVP reported better bodily pain and social functioning at follow up than participants in the waitlist group. Improved bodily pain was evident for 62% of the surgery group, and social functioning improved for 19%. Surgery did not alter reported physical functioning or mood. Dyskinesia improved after surgery, but there were no improvements in “on-state” manual dexterity or handwriting. Conclusions. Most patients who underwent left PVP exhibited declines in learning, fluency, working memory, and speeded color naming. Accounting for retesting effects altered the magnitude of these declines by up to one quarter of a standard deviation, but did not increase the breadth of postsurgical neuropsychological decline beyond that typically reported in the literature. It was found that PVP improved dyskinesia, bodily pain, and social functioning, but did not lead to improvement on other objective and self-reported measures of motor functioning.


1999 ◽  
Vol 90 (6) ◽  
pp. 1005-1010 ◽  
Author(s):  
Tetsuo Yokoyama ◽  
Yoko Imamura ◽  
Kenji Sugiyama ◽  
Shigeru Nishizawa ◽  
Naoki Yokota ◽  
...  

Object. Pre- and postoperative cognitive function was evaluated in patients with Parkinson's disease (PD) who underwent unilateral posteroventral pallidotomy (PVP) to clarify the effects of this operation on cognitive function.Methods. Cognitive function was assessed before surgery and 1 month and 3 months afterward by using a battery of neuropsychological tests consisting of orientation, digit span (forward and backward), digit learning, 5-minute memory of five words, similarities, serial sevens, animal name listing, and the kana pick-out test, as well as the Japanese version of the Mini-Mental State. Preoperative evaluation revealed that patients experienced difficulties in performing the neuropsychological tasks of reasoning and abstraction, working memory of numerals, word fluency, and concept formation compared with 36 healthy volunteers (p < 0.05). A simple regression analysis showed strong relationships in a negative fashion between preoperative (r = 0.81, p = 0.002) and 1-month (r = 0.79, p = 0.0059) and 3-month (r = 0.85, p = 0.0016) postoperative gross-total scores and Hoehn and Yahr staging. Preoperative and postoperative scores at 1 month and 3 months were analyzed by analysis of variance, but only the Fisher's post hoc test revealed the source of difference. All tasks except orientation (p = 0.0292) were unchanged in the 10 patients who underwent surgery on the right side. The scores for this task at 1 month postsurgery were significantly lower (p = 0.0203) but improved to preoperative values by 3 months. In the 15 patients who underwent surgery on the left side significant differences among tasks were revealed for serial sevens (p = 0.0471) and animal naming (p = 0.0425). The scores for these tasks were significantly lower at 1 month postoperatively (p = 0.0431 for serial sevens and p = 0.0408 for animal naming), but improved by 3 months after the operation.Conclusions. The present data revealed that cognitive dysfunction in patients with PD relates to advancement of Hoehn and Yahr stage, but PVP is not associated with significant long-lasting cognitive deficits.


1974 ◽  
Vol 40 (5) ◽  
pp. 577-582 ◽  
Author(s):  
V. Balasubramaniam ◽  
T. S. Kanaka ◽  
P. B. Ramanujam

✓ An analysis of 94 cases of cerebral palsy treated by stereotaxic surgery is reported. The selection of patients and target areas for surgery are discussed. Hypertonic cases are classified into rigid, rigidospastic, and spastic types on the basis of surface electromyographic studies. For rigidity and rigidospasticity, ventrolateral thalamotomy gives relief, while spastic cases do well with dentatectomy. Centromedian thalamotomy relieves sensory-induced involuntary movements. Involuntary movements unaccompanied by changes in tone are abolished by lesions of the nucleus ventralis intermedius.


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.


2001 ◽  
Vol 94 (4) ◽  
pp. 552-558 ◽  
Author(s):  
Marwan I. Hariz ◽  
A. Tommy Bergenheim

Object. The clinical condition of patients with Parkinson disease (PD) who had undergone posteroventral pallidotomy (PVP) between 1985 and 1990 was evaluated at a mean of 10 years postsurgery. These patients were part of a larger series described in the first paper on Leksell's PVP that was published in 1992. Methods. Thirteen consecutive patients who had undergone pallidotomy at the University Hospital of Northern Sweden were tracked. Hospital and clinic records that had been updated regularly by the patients' various neurologists, geriatricians, and other clinicians were reviewed. Emphasis was placed on assessing the evolution of PD symptoms after surgery, and changes in the general health and social condition of the patients. The mean follow-up duration was 10.5 years (range 3–13.5 years). Five patients underwent a total of seven subsequent surgeries for their PD, 4 months to 11 years after the initial pallidotomy. The mean Hoehn and Yahr stage was 3 at the first surgery and 3.7 at the last follow-up review (p < 0.005). Dosages of levodopa and dopamine agonists were increased in all patients, without recurrence or induction of dyskinesias contralateral to the pallidotomy. Contralateral tremor, if it was initially controlled by surgery, remained improved. However, most patients exhibited a gradual recurrence of akinesia and an increase in gait freezing. Cognitive decline and presentation with diseases unrelated to PD were not uncommon. Conclusions. The long-term effect of PVP on dyskinesias was not only curative but also appeared to be prophylactic. Contralateral tremor was improved in the majority of patients, although additional surgeries for PD were needed in some patients. Further progression of axial and akinetic symptoms, and an eventual decline in cognition together with other concomitant illnesses, contributed to increased disability in several patients.


2003 ◽  
Vol 98 (6) ◽  
pp. 1247-1254 ◽  
Author(s):  
Aaron A. Cohen-Gadol ◽  
J. Eric Ahlskog ◽  
Joseph Y. Matsumoto ◽  
Mary A. Swenson ◽  
Robyn L. McClelland ◽  
...  

Object. Selective peripheral denervation is currently the primary surgical treatment for intractable cervical dystonia. The authors assessed preoperative factors to determine which, if any, correlated with outcomes in patients with torticollis who had undergone this procedure. Methods. The records of 168 consecutive patients who had undergone selective peripheral denervation for cervical dystonia between 1988 and 1996 at the Mayo Clinic were reviewed. There were 89 women (53%) and 79 men (47%) with a mean age of 53.4 years. Selection of muscles for denervation was based on the patient's clinical presentation and electromyography mapping results. The most common torticollis vectors were rotational in 141 patients (84%) and laterocollis in 59 (35%). Seventy patients (42%) presented with combined vectors. The technique used to remedy both conditions involved denervation of the ipsilateral posterior cervical paraspinal and splenius capitis muscles. Denervation of the sternocleidomastoid muscle was performed on the contralateral side for rotational torticollis and on the ipsilateral side for laterocollis. A rigorous physical therapy program followed surgery. At the 3-month postoperative evaluation, 125 patients (77%) of the 162 who were available for follow up had moderate to excellent improvement in their head position, and pain was moderately to markedly improved in 131 patients (81%). The long-term follow up lasted a mean of 3.4 years and was undertaken in 130 patients. The original level of moderate to excellent improvement in head position and pain was retained in at least 71 patients (70%). Outcome was not predicted by preoperative head position, severity of abnormal posture of head, symptom duration, presence of tremor or phasic dystonic movements, or failure to respond to botulinum toxin treatment. Five patients recovered from postoperative complications including one myocardial infarction, one pulmonary embolism, and three respiratory failures. Three patients suffered from persistent C-2 distribution dysesthesias and three from slight shoulder weakness; one had a wound infection, and one died of respiratory arrest. Conclusions. Selective peripheral denervation is an effective method of achieving lasting improvement of dystonia in most patients with intractable torticollis.


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