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1.
OBJECTIVE: To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. BACKGROUND: Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus) pallidotomies for Parkinson's disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. METHODS: Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Scale (BFM). Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM) by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. RESULTS: The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58% was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. CONCLUSIONS: Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure.  相似文献   

2.
Internal globus pallidotomy in dystonia secondary to Huntington's disease.   总被引:4,自引:0,他引:4  
INTRODUCTION AND METHOD: The prototypic motor feature of Huntington's disease (HD) is chorea, but parkinsonism and involuntary movements such as dystonia and myoclonus can also be present. Pallidotomy has been shown to be an effective treatment for medically refractory Parkinson's disease (PD). We performed bilateral microelectrode guided-stereotactic pallidotomies targeted at globus pallidum internus (GPi) to treat a 13-year-old patient diagnosed with Westphal variant of HD with intractable generalized dystonia and parkinsonism. RESULTS: Intraoperative microelectrode recordings of GPi cells showed a relatively low firing rate, 29 +/- 14 Hz, with most neurons showing pauses. Acutely, after surgery, limb dystonia mildly improved but trunk dystonia persisted. Postoperative follow up 3 months later showed minimal clinical improvement in dystonic features with marked worsening of spasticity. CONCLUSION: In our case, bilateral pallidotomy produced modest palliative functional improvement in dystonic features. Cellular firing patterns were markedly different than in PD and were similar to those found in dystonia.  相似文献   

3.
Treatment options for patients in dystonic state include sedation, artificial ventilation, intrathecal baclofen infusions and stereotactic procedures. The main aim of this overview is the presentation and assessment of stereotactic procedures applied for treating patients in severe dystonic state. We performed literature overview starting from 1998 to 2012 with case reports regarding all patients treated by stereotactic procedures for dystonic state. We were able to find 15 articles describing 22 patients. Ablative procedures were described in 5 articles (3 thalamotomies, 3 pallidotomies) and were done in 6 patients. In the remaining 10 articles, globus pallidus internus stimulation was utilized in another 16 patients. We can conclude that bilateral pallidal deep brain stimulation seems to be the best stereotactic target for patients in dystonic state.  相似文献   

4.
BACKGROUND AND PURPOSE: The newest studies notify that globus pallidus, besides the motor control, plays an important role in cognitive functioning of: memory, attention, linguistic skills, visuospatial ability, and executive functions. Stereotactic pallidotomy (the lesion of the motor region of the globus pallidus) is one of the surgery options that is used in the treatment of the primary dystonia. Motor region is located in the postero-ventral part of the internal globus pallidus. The goal of the study was to assess of the influence of pallidotomy on cognitive functioning in the group of patients suffering from the primary dystonia. MATERIAL AND METHODS: Eighteen patients diagnosed with primary dystonia were treated by stereotactic pallidotomy and they were investigated neuropsychologically. The study was performed between March 2004 and February 2005. Neuropsychological assessment was conducted 1-2 days prior to operation and 2 days following the surgery. The clinical course of the cognitive functions and emotional status were assessed by the following neuropsychological tests: Mini Mental State Examination (MMSE), Trail Making Test A and B (TMT A and B), Stroop Color-Word Interference Test, N-back Test, Auditory Verbal Learning Test (AVLT), Benton Visual Retention Test (BVRT), Wisconsin Card Sorting Test (WCST), Beck Depression Inventory (BDI), Montgomery-Asberg Depression Rating Scale (MADRS). RESULTS: The statistically significant differences have been obtained between the results before and after the surgery in the depression scales: BDI (p<0.02) and in MADRS (p<0.01). None of the neuropsychological tests revealed significant differences between the results before and after the surgery. The only trend (p=0.06-0.07) was noted between results of AVLT before and after the surgery. The average results were worse after the surgery in the first, fifth and sixth attempt of the reply. CONCLUSIONS: Stereotactic pallidotomy has not significantly changed cognitive functions in the patients suffering from primary dystonia. Whereas, the emotional state has significantly improved in consequence of the applying surgery. Stereotactic pallidotomy in primary dystonia seems to be a treatment option safe for the patients' cognitive functions.  相似文献   

5.
OBJECTIVE: To evaluate the impact of stereotactic surgery on daily activities of Parkinson's disease (PD) patients. METHOD: Thirty patients with idiopathic PD were evaluated before surgery and one, three, six and twelve months after surgery. Patients were evaluated with the UPDRS - part II (Unified Parkinson's Disease Rating Scale) and the Schwab & England scales. Nine of the patients had also been evaluated after twenty four months. RESULTS: We performed nine posteroventral pallidotomies (PVP), two on the left hemisphere and seven on the right; 17 ventrolateral thalamotomies (VLT), 12 on the left and five on the right; two VLT on the left associated with PVP on the right at the same surgical procedure. The mean "off" phase scores of Schwab & England scale were: 60.6 before surgery, 74 after the first, 76.6 after the third, 75.6 after the sixth, 72.3 after the twelfth and 71.1 after the twenty fourth months after surgery. The mean "off" phase scores of UPDRS - part II scale were: 21 before surgery, 12.3 after the first, 14.7 after the third, 15.27 after the sixth, 17.1 after the twelfth and 17.5 after the twenty fourth months after surgery. CONCLUSION: VTL and PVP are useful procedures to improve daily living activities of the PD patients and the best results are seen by six months after surgery.  相似文献   

6.
The aim of this study was to evaluate the efficacy and safety of epidural premotor stimulation in patients with primary focal dystonia. Seven patients were selected: 6 had cervical dystonia and 1 had right upper limb dystonia. In 2 patients, sustained muscle contractions led to a prevalently fixed head posture. Patients with cervical dystonia received a bilateral implant, whereas the patient with hand dystonia received a unilateral implant. Neurological and neuropsychological evaluations were performed before surgery (baseline), and 1, 3, 6, and 12 months afterward. The Burke-Fahn-Marsden scale (BFMS) and the Toronto Western spasmodic torticollis rating scale (TWSTRS) were administered at the same time points. Patients underwent resting (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) scans, before and 12 months after surgery. No adverse events occurred. An overall improvement was observed on the BFMS and TWSTRS after surgery. Patients with prevalently fixed cervical dystonia had a reduced benefit. Presurgical neuroimaging revealed a significant bilateral metabolic increase in the sensorimotor areas, which was reduced after surgery.  相似文献   

7.
Recent randomized trials have shown that cilostazol is superior to aspirin for secondary stroke prevention. We hypothesized that combining cilostazol with aspirin is more effective than aspirin alone in patients with acute ischemic stroke. This randomized study compared the effects of oral aspirin alone to aspirin plus cilostazol in patients with non-cardioembolic ischemic stroke within 48 h of stroke onset. NIH Stroke Scale (NIHSS) and modified Rankin Scale (mRS) scores were checked before and after 14 days and 6 months of drug administration. The primary and secondary endpoints were neurological deterioration or stroke recurrence (NIHSS score ≥ 1) within 14 days and 6 months, respectively. For statistical analysis, on-treatment analysis was conducted. Seventy-six patients were enrolled in the study. The primary endpoint was significantly higher in the aspirin group than in the aspirin plus cilostazol group (28% vs. 6%, relative risk (RR): 0.21, 95% confidence intervals (CI): 0.05-0.87, p=0.013). Among the patients who did not reach these endpoints, the mean improvement in the NIHSS score at day 14 tended to be better (-1.8 ± 1.2 vs. -1.2 ± 1.0, p=0.078) and the frequency of the favorable functional status of mRS 0-1 at month 6 was significantly higher (RR: 1.48, 95% CI: 1.07-2.06, p=0.0048) in the aspirin plus cilostazol group than in the aspirin group. Patients treated with aspirin plus cilostazol during the acute phase of stroke had less neurological deterioration and more favorable functional status than those treated with aspirin alone.  相似文献   

8.
Brain damage after coronary artery bypass grafting   总被引:3,自引:0,他引:3  
BACKGROUND: Coronary artery bypass grafting (CABG) is associated with a risk for focal neurological deficits and neuropsychological impairment postoperatively. OBJECTIVES: To examine the brain damage after CABG using diffusion-weighted magnetic resonance imaging and (1)H-magnetic resonance spectroscopy (MRS) and to correlate the results with neurological and neuropsychological findings. PATIENTS AND METHODS: Thirty-five consecutive patients undergoing elective CABG were included. Patients underwent a neurological and neuropsychological examination before and after CABG. The magnetic resonance protocol was applied before and after (mean, 3 days) surgery and included a diffusion-weighted sequence and single-voxel MRS measurements in the frontal lobes. RESULTS: None of the patients revealed a new focal neurological deficit after surgery. Diffusion-weighted magnetic resonance imaging demonstrated new ischemic lesions in 9 (26%) of the patients. The presence of an ischemic lesion was not related to impaired postoperative test performance (P>.50). The apparent diffusion coefficient values in the cerebellum and the centrum semiovale exhibited an increase after surgery (P<.01), consistent with vasogenic edema. Following surgery, MRS revealed a significant decrease in the metabolite ratio of N-acetylaspartate-creatine (mean +/- SD, 1.69 +/- 0.20 vs 1.52 +/- 0.19; P<.001). The extent of deterioration in neuropsychological test performance after surgery was closely related to the degree of the N-acetylaspartate-creatine ratio decrease (P<.01). A follow-up MRS scan revealed a normalization of the N-acetylaspartate-creatine ratio, which accompanied the recovery in psychological test performance. CONCLUSIONS: Postoperative impairment in neuropsychological test performance is associated with a transient metabolic neuronal disturbance. Focal ischemic lesions after CABG are more frequent than the apparent neurological complication rate; however, they are not related to the diffuse postoperative encephalopathy.  相似文献   

9.
The aim of this study was to evaluate the efficacy and safety of epidural premotor stimulation in patients with primary focal dystonia. Seven patients were selected: 6 had cervical dystonia and 1 had right upper limb dystonia. In 2 patients, sustained muscle contractions led to a prevalently fixed head posture. Patients with cervical dystonia received a bilateral implant, whereas the patient with hand dystonia received a unilateral implant. Neurological and neuropsychological evaluations were performed before surgery (baseline), and 1, 3, 6, and 12 months afterward. The Burke‐Fahn‐Marsden scale (BFMS) and the Toronto Western spasmodic torticollis rating scale (TWSTRS) were administered at the same time points. Patients underwent resting 18F‐fluorodeoxyglucose (FDG) positron emission tomography (PET) scans, before and 12 months after surgery. No adverse events occurred. An overall improvement was observed on the BFMS and TWSTRS after surgery. Patients with prevalently fixed cervical dystonia had a reduced benefit. Presurgical neuroimaging revealed a significant bilateral metabolic increase in the sensorimotor areas, which was reduced after surgery. © 2012 Movement Disorder Society  相似文献   

10.
脑干病变的立体定向治疗   总被引:3,自引:0,他引:3  
目的:评价计算机体层摄影(CT)引导下的立体定向法在治疗脑干病变中的意义。方法:29例病人(脑干肿瘤24例,脑干出血5例),男14例,女15例,平均年龄38岁。采用CT引导下的立体定向法(为Leksell立体定向仪)。治疗种类包括脑干病变的活检、间质内放疗、后装置管放疗及血肿排空术。结果:29例病人均一次穿刺成功,术中无一例死亡。6个月后统计的治疗有效率:好转89%,恶化5.5%,死亡5.5%,主要并发症有意识障碍、恶心呕吐、四肢抽搐、外展神经麻痹等。结论:CT引导下的立体定向法在治疗脑干病变中具有损伤小(不开颅),适应范围广,疗效好,致残、致死率比开颅术低,并发症、后遗症少,以及简便安全等微侵袭的特点。同时具有诊断和治疗的双重作用,是一种有实用价值的治疗方法。  相似文献   

11.
BACKGROUND: Chronic bilateral subthalamic deep brain stimulation (STN-DBS) is known to improve motor function in patients with Parkinson disease (PD). However, the possible effects of STN-DBS on neuropsychological functions have been studied less. OBJECTIVE: To investigate the effects of STN-DBS on neuropsychological functions in PD. DESIGN: Before-after trial. PATIENTS AND METHODS: Fifteen consecutive patients were assessed before and 3 months after implantation of stimulators for STN-DBS (postsurgical assessment with the stimulators switched on). Both assessments were performed with patients in a drug-free condition. The neuropsychological battery consisted of tests measuring memory and visuospatial and frontal functions. RESULTS: The comparison between presurgical and postsurgical performance showed a moderate deterioration in verbal memory and prefrontal and visuospatial functions, and a moderate improvement in a prefrontal task and obsessive-compulsive traits. The motor state improved in all patients. CONCLUSION: Therapy with STN-DBS improves motor symptoms in PD without any clinically relevant neuropsychological deterioration.  相似文献   

12.
The results of deep brain stimulation (DBS) of the globus pallidus internus (Gpi) in six patients with generalized, focal, and segmental dystonia are presented. Pre- and postoperative assessments are given for one patient with generalized inherited dystonia and for five patients with idiopathic segmental or cervical dystonia. Clinical symptoms were evaluated before and 3-12 months after surgery using the Burke-Fahn-Marsden (BFM) dystonia rating scale for primary torsion dystonia and the Tsui scale for cervical dystonia. The Short-Form Health Survey (SF-36) was completed by each patient to document preoperative and postoperative health status. Also, neurological status was documented by video before and during chronic stimulation. Magnetic resonance imaging studies were performed to show the anatomical localization of the electrode leads. Five patients showed a progressive improvement within 7 days. One patient with cervical dystonia and Meige's syndrome showed no improvement for 3 months, but beneficial effects were observed after 12 months. On average, the BFM movement scale scores decreased by 72.5% and Tsui scale scores by 63%. SF-36 showed an improvement in health status by an average of 36% according to eight different health categories. We conclude that chronic high-frequency Gpi stimulation in different types of dystonia is a very effective and safe treatment.  相似文献   

13.
Tardive dystonia usually occurs with a delay after neuroleptic exposure in patients with major psychosis. A subgroup of patients, however, is given such medication for “mild depression” or “neurasthenia.” Tardive dystonia, in general, may respond favorably to pallidal deep brain stimulation (DBS). Nevertheless, it remains unclear thus far whether or not similar beneficial outcome is achieved with pallidal DBS in different subgroups of patients with tardive dystonia. Four women (mean age 59 years at surgery) underwent stereotactic pallidal DBS in the frame of an observational study. Tardive dystonia occurred secondary to medication with fluspirilene and haloperidol, and injection of long‐acting depot neuroleptics prescribed for mild depression or “nervousness.” Assessment included the Burke‐Fahn‐Marsden (BFM) scale preoperatively and at 12 months follow‐up. Extended follow‐up was available at a mean of 27.3 months postoperatively (range 16–36 months). There were no surgically related complications. All 4 patients experienced sustained statistically significant benefit from pallidal DBS. Mean improvement at 12 months was 77% for the BFM motor score (range, 45–91%; P = 0.043), and 84% at the last available follow‐up (range, 70–91%; P = 0.03). This was paralleled by improvement of the BFM disability score. Chronic pallidal DBS in patients with tardive dystonia without a history of major psychosis provides sustained improvement which is similar to that in other subgroups of patients with tardive dystonia. This effect is stable on extended follow‐up for up to 3 years. © 2010 Movement Disorder Society  相似文献   

14.
BACKGROUND AND PURPOSE: The authors report the results of stereotactic surgery for severe posttraumatic tremor in 7 patients. MATERIAL AND METHODS: All patients were severely incapacitated in their daily living activities by mainly kinetic and postural components of Holmes tremor. The 7 patients underwent 8 stereotactic operations. The stereotactic target in all patients was the ventrolateral thalamus. Patients were evaluated using the Clinical Rating Scale For Tremor (CRST) before and up to 24 months after stereotactic thalamotomy. RESULTS: In the postoperative period there was marked amelioration in kinetic and postural components of Holmes tremor in contralateral upper and lower extremity. The mean presurgery value for contralateral upper extremity kinetic tremor (scores 5-6 CRST) dropped from 3.25 to mean postsurgery value of 0.5. There was also a marked functional improvement. It was reduced from a mean value of 71% of maximum disability according to CRST (scores 15-21, part C CRST, 0% independent, 100% total dependent) to 28.5% over 2-year follow-up. CONCLUSIONS: Ventrolateral thalamotomy alleviates effectively Holmes tremor and improves greatly performance of activities of daily living.  相似文献   

15.
继发性全身性肌张力障碍的手术治疗分析   总被引:4,自引:3,他引:1  
目的探讨采用立体定向核团毁损和核团毁损加选择性脊神经后根切断术治疗兼有锥体系统损害的继发性全身性肌张力障碍的手术疗效。方法18例病人随机分成两组:a组12例,单纯采用立体定向核团毁损手术治疗;b组6例,采用核团毁损加选择性脊神经后根切断术。随访3个月,观察并对比术后运动功能改善情况。结果单纯立体定向核团毁损手术治疗效果不明显,核团毁损手术加选择性脊神经后根切断术能使病人运动功能得到明显提高。结论联合采用核团毁损手术与选择性脊神经后根切断术,对兼有锥体系统损害的继发性全身性肌张力障碍病人近期疗效较好。  相似文献   

16.
Deep brain stimulation (DBS) of globus pallidus internus (GPi) has emerged as an effective treatment for primary generalized dystonia. However, the physiological mechanisms of improvement are not fully understood. Cortical activity in response to pallidal stimulation was recorded in 6 patients with primary generalized dystonia >6 months after bilateral GPi DBS. Scalp electroencephalogram was recorded using 60 surface electrodes during 10 Hz bipolar pallidal DBS at each electrode contact pair. Anatomical position of the electrode contacts in relation to the GPi, medial medullary lamina and globus pallidus externus (GPe) was determined from the postoperative stereotactic MRI. In all six patients an evoked potential (EP) was observed with average onset latency of 10.9 ms ± 0.77, peak latency 26.6 ms ± 1.6, distributed mainly over the ipsilateral hemisphere, maximal centrally. The mean amplitude of this potential was larger with stimulation in posteroventral GPi than in GPe (3.36 μV vs. 0.50 μV, P < 0.0001). The EP was absent in one patient‐side, ipsilateral to a previous thalamotomy. Low frequency GPi stimulation produces an EP distributed centrally over the ipsilateral hemisphere. The latency and distribution of the EP are consistent with stimulation of pallidothalamic neurons projecting to the sensorimotor cortex. Because the EP is larger and more consistently present with stimulation of posteroventral GPi than GPe, it may provide a physiological tool to identify contacts within the optimal surgical target. © 2007 Movement Disorder Society  相似文献   

17.
Disorganization of sensory cortical somatotopy has been described in adult onset primary torsion dystonia (AOPTD). Although botulinum toxin type A (BTX-A) acts peripherally, some studies have suggested a central effect. Our primary hypothesis was that sensory cortical reorganization occurs after BTX-A treatment of AOPTD. Twenty patients with cervical dystonia and 18 healthy age-matched control patients had spatial discrimination thresholds (SDTs) measured at baseline and monthly for 3 months. Mean baseline SDT (+/-SD) was 1.75 +/-0.76 mm in the dystonia group, greater than the control group mean of 1.323 +/- 0.45 mm (P = 0.05). Mean control group SDT did not vary significantly over time. A transient improvement of 23% from baseline (P = 0.005) occurred in the dystonia group 1 month after injection, which did not positively correlate with changes in physician and patient ratings of torticollis severity. The presumed mechanism of SDT improvement is a modulation of afferent cortical inputs from muscle spindles. Society.  相似文献   

18.
Background: The D216H single‐nucleotide polymorphism (SNP) (rs1801968) in DYT1 exon 4 has been suggested to be a genetic modifier in primary dystonia. Methods: To further explore this question, we assessed rs1801968 variations in a cohort of 210 Chinese patients with primary dystonia devoid of DYT1 mutations. Results: We found that focal dystonia, specifically cervical dystonia, was the most common form of dystonia, with 8.1% of all the patients having a positive family history of dystonia. No association of the D216H SNP with primary dystonia was identified. In a subsequent subgroup analysis, the 216H allele was found to occur more frequently in patients with writer’s cramp, but no correlation was found between the allele and other forms of dystonia or age of onset. Conclusions: Our findings do not confirm that the allele contributes to the risk of D216H SNP primary dystonia.  相似文献   

19.
OBJECTIVE: To evaluate whether subthalamic nucleotomy produces adverse cognitive effects in patients with Parkinson's disease. METHOD: Twelve patients with Parkinson's disease underwent stereotactic surgery to the subthalamic nucleus. Presurgical and postsurgical neuropsychological assessment of attention, memory, executive function, language, and verbal intellect were undertaken with a battery of tests designed to minimise potential contamination of cognitive effects by motor symptoms. RESULTS: There was no statistically significant difference in the cognitive tests results after operation for the group as a whole. Reliable change indexes were generated for the cognitive tests. Reliable change postoperatively was found on specific tests of verbal memory, attention, and planning. Left sided operations were associated with greater incidence of deterioration postsurgery. CONCLUSIONS: Preliminary data on the first reported cognitive changes after subthalamic nucleotomy suggested few adverse cognitive effects of the surgery although discrete neuropsychological changes were seen in some patients. These effects were consistent with current theories on the cognitive functions of the basal ganglia.  相似文献   

20.
PURPOSE: To assess the add-on efficacy of levetiracetam on the EEG, behavior, and cognition of children with continuous spikes and waves during slow sleep (CSWS). METHODS: Charts of children with behavioral and/or cognitive deterioration associated with CSWS who received levetiracetam at 50 mg/kg/day as add-on treatment were retrospectively reviewed. Awake and sleep EEG recordings and detailed neuropsychological and behavioral assessments were available at baseline and 2 months after levetiracetam initiation. In children showing clinical and/or electrophysiological improvement after 2 months, levetiracetam was continued with a new evaluation at 1 year. RESULTS: Twelve patients were included (9 cryptogenic and 3 symptomatic cases). Seven patients (58.3%) showed improvement of EEG record. Among these seven patients, neuropsychological evaluation was improved in three, and in the other four patients, not testable because of severe cognitive impairment, behavior was improved. Two patients improved in neuropsychological evaluation despite the lack of EEG improvement. Eight patients (66.6%) continued levetiracetam treatment after 2 months. After 1 year, four patients were still on levetiracetam, two because sustained effect on EEG and behavior and the two others because improvement in neuropsychological testing despite unchanged EEG. Levetiracetam was discontinued in the other four patients because of neuropsychological or behavioral deterioration associated with CSWS pattern, between 9 and 11 months after treatment initiation. CONCLUSIONS: This retrospective study suggests that levetiracetam has a positive effect on the EEG, the behavior, and the cognition of patients with epilepsy and CSWS. Additional studies are warranted in order to assess the place of this drug in these epileptic conditions.  相似文献   

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