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["Asleep-awake-asleep"-anaesthetic technique for awake craniotomy].

Awake craniotomy in tumor and epilepsy surgery or for the implantation of electrodes for deep brain stimulation requires specific anesthesiological strategies. Propofol allows for quick emergence and has little effect on the respiratory function of the usually spontaneously breathing patient. Pain control may be instituted by hemiscalp block for trepanation or local infiltration for deep brain electrode implantation. In addition, low dose remifentanil is recommended for trepanation (i.e. tumor or epilepsy surgery). The airway may be secured by an ordinary Magill tube placed transnasally with its tip underneath the epiglottis. To protect the patient against vomiting an adequate antiemetic prophylaxis is required.

Anesthesia↗

Excellent response to apomorphine in Parkinsonism with optic atrophy unresponsive to oral antiparkinsonian medication.

Two patients presented with the cardinal symptoms of Parkinson's disease (PD), which had developed slowly and progressively. One patient underwent brain imaging of the dopaminergic system, the results of which were compatible with PD. However, both patients showed no response to L-dopa or oral dopaminergic medication. They also had optic nerve atrophy of unknown etiology. The severe motor symptoms of both patients were conspicuously alleviated by apomorphine delivered subcutaneously. One patient underwent bilateral subthalamic nucleus deep brain stimulation, which also proved effective.

Administration, Oral↗

Changes in blink reflex excitability after globus pallidus internus stimulation for dystonia.

A pathophysiological feature of dystonia is reduced inhibition at various levels of the nervous system, which may be detected in clinically unaffected body parts. Chronic deep brain stimulation (DBS) of the globus pallidus internus (GPi) has emerged as an effective treatment for primary torsion dystonia (PTD), although its mechanism of action and impact on inhibitory abnormalities in dystonia are unknown. We sought to understand the effect of GPi DBS on brainstem excitability in patients with PTD. We measured the blink reflex from orbicularis oculi in response to paired electrical stimulation of the supraorbital nerve at interstimulus intervals of 500 and 1,000 milliseconds in 10 patients with PTD before and at intervals of 1, 3, and 6 months after bilateral GPi DBS and in 10 healthy subjects. Patients were clinically evaluated using the Burke-Fahn-Marsden dystonia rating scale. We found R2 inhibition was significantly decreased in PTD patients compared with control subjects and progressively increased after GPi DBS, which correlated with clinical improvement in dystonia. We conclude that GPi DBS for PTD results in functional reorganization of the nervous system, which includes a long-term increase in brainstem inhibition.

Adult↗

Aphasia and thalamotomy: important issues.

Patients may present with classical symptoms suggesting aphasia following thalamotomy (repetition, comprehension, fluency and naming abnormalities). They may also present with 'freezing of speech', and this symptom should not be considered as a speech disorder or a symptom of Parkinson's disease progression, without careful testing to rule out language deficits, particularly dysfluency. There are important issues related to all language complications of thalamotomy, including (1) the time course of problems following surgery, (2) the impact of preexistingspeech problems, (3) the importance of the size and location of lesions, (4) the potential circuits important in the pathogenesis of a thalamic language disturbance and (5) whether laterality makes a difference (left- versus right-sided thalamic lesions). As more centers switch from thalamotomy to deep brain stimulation, the issues regarding aphasia will need to be addressed.

Aphasia↗

Impact of subthalamic nucleus stimulation on the initiation of gait in Parkinson's disease.

The effects of subthalamic nucleus (STN) stimulation on the anticipatory postural actions associated with the initiation of gait were studied in ten patients with idiopathic Parkinson's disease undergoing therapeutic deep brain stimulation. Kinematic, dynamic and electromyographic analysis was performed before and while subjects were starting gait in response to an external cue. Effects of STN stimulation on the standing posture preceding the go signal included significant improvement of the vertical alignment of the trunk and shank, decrease of the hip joint moment, backward shift of the center of pressure (CoP) and reduction of abnormal tonic and/or rhythmic activity in the thigh and leg muscles. Responses to bilateral STN stimulation were more consistent than those evoked by unilateral stimulation. Moreover, comparison between postural changes induced by STN stimulation applied prior to the gait initiation cue and during simple quiet standing revealed more significant responses in the former condition. Effects on the actual gait initiation process included shortening of the imbalance phase, larger backward/lateral displacement of CoP and more physiological expression of the underlying anticipatory muscular synergy. Additional changes were shortening of the unloading phase, shortening of the first-swing phase and increase in the length of the first step. Results demonstrate substantial influence of STN stimulation on functionally basic motor control mechanisms. In particular, the evidence of more significant responses upon attention-demanding conditions and the remarkable effects on postural programmes sub-serving feed-forward regulation of the onset of complex multijoint movements, suggests a consistent action on postural sub-systems relying on cognitive data processing and internal models of body mechanics.

Aged↗

Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington's disease: technical case report.

OBJECTIVE AND IMPORTANCE: Huntington's disease (HD) produces debilitating motor abnormalities that are poorly responsive to medical therapy. Deep brain stimulation (DBS) may offer a treatment option for afflicted patients, but its role in the management of HD remains unclear. In the present case, DBS leads were implanted bilaterally into the posteroventral globus pallidus internus (GPi) to control disabling and medically intractable choreathetosis in a severely affected HD patient. The surgical procedure, intraoperative electrophysiological findings, and 12-month postoperative course, with patient video, are presented. CLINICAL PRESENTATION: This 41-year-old man with genetically confirmed HD developed motor symptoms at age 28. He had completed multiple medical trials without alleviation of his progressive and debilitating choreathetosis. Extensive clinical assessment, including neuropsychological testing, was performed to determine surgical candidacy. INTERVENTION: DBS leads were bilaterally implanted, under stereotactic guidance, into the posteroventral GPi. Disease progression and symptom control were assessed at regular postoperative intervals. Bilateral pallidal stimulation produced a dramatic reduction in choreathetoid movements and improvement in overall motor functioning. The patient also exhibited normalization of body weight, mood, and energy level, as well as improved performance of activities of daily living. These effects were sustained at 1 year after surgery. CONCLUSION: The clinical benefits of DBS observed in this HD patient were comparable to those reported in other hyperkinetic disorders and demonstrate that pallidal stimulation can provide long-term alleviation of HD-associated choreathetosis.

Adult↗

Effects of bilateral subthalamic nucleus stimulation on sleep, daytime sleepiness, and early morning dystonia in patients with Parkinson disease.

OBJECT: The aim of this study was to assess the long-term effects of bilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) for Parkinson disease (PD) on sleep, daytime sleepiness, and early morning dystonia and to evaluate the relationship between total sleep time and motor function. METHODS: Patients who had undergone bilateral STN DBS and a follow-up evaluation of 6 months (89 patients), 12 months (83 patients), and 24 months (43 patients) were included in this study. The patients were preoperatively assessed using the Unified Parkinson's Disease Rating Scale (UPDRS) in the medication-on and -off conditions, and they completed patient diaries. A subset of patients also completed the Epworth Sleepiness Scale. These assessments were repeated postoperatively with stimulation. The UPDRS activities of daily living (ADL) and motor scores as well as total sleep hours were significantly improved at 6, 12, and 24 months poststimulation and with no medication compared with baseline values. Increased sleep time was significantly correlated with improvements in bradykinesia but not with tremor or rigidity. Patient-reported sleep problems and early morning dystonia were reduced after STN DBS. Antiparkinsonian medications were significantly reduced after STN DBS; however, there were no changes in excessive daytime sleepiness 6, 12, or 24 months after surgery. CONCLUSIONS: Bilateral STN DBS increased total sleep time and reduced patient-reported sleep problems and early morning dystonia for up to 24 months posttreatment. These changes in sleep were related to improvements in functioning, specifically those affected by bradykinesia. Despite significant reductions in antiparkinsonian medications, STN DBS did not reduce excessive daytime sleepiness.

Activities of Daily Living↗

Hypothalamic stimulation in chronic cluster headache: a pilot study of efficacy and mode of action.

We enrolled six patients suffering from refractory chronic cluster headache in a pilot trial of neurostimulation of the ipsilateral ventroposterior hypothalamus using the stereotactic coordinates published previously. After the varying durations needed to determine optimal stimulation parameters and a mean follow-up of 14.5 months, the clinical outcome is excellent in three patients (two are pain-free; one has fewer than three attacks per month), but unsatisfactory in one patient, who only has had transient remissions. Mean voltage is 3.28 V, diplopia being the major factor limiting its increase. When the stimulator was switched off in one pain-free patient, attacks resumed after 3 months until it was turned on again. In one patient the implantation procedure had to be interrupted because of a panic attack with autonomic disturbances. Another patient died from an intracerebral haemorrhage that developed along the lead tract several hours after surgery; there were no other vascular changes on post-mortem examination. After 1 month, the hypothalamic stimulation induced resistance against the attack-triggering agent nitroglycerin and tended to increase pain thresholds at extracephalic, but not at cephalic, sites. It had no detectable effect on neurohypophyseal hormones or melatonin excretion. We conclude that hypothalamic stimulation has remarkable efficacy in most, but not all, patients with treatment-resistant chronic cluster headache. Its efficacy is not due to a simple analgesic effect or to hormonal changes. Intracerebral haemorrhage cannot be neglected in the risk evaluation of the procedure. Whether it might be more prevalent than in deep-brain stimulation for movement disorders remains to be determined.

Adult↗

Long-term benefits in quality of life provided by bilateral subthalamic stimulation in patients with Parkinson disease.

OBJECT: The goals of this study were to evaluate long-term benefits in quality of life in patients with Parkinson disease (PD) after bilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) and to evaluate the relationship between improvements in motor function and quality of life. METHODS: Seventy-one patients who received bilateral STN stimulation implants and participated in follow-up review for at least 12 months were included in the study. Fifty-nine patients participated in a 12-month follow-up review and 43 patients in a follow-up review lasting at least 24 months. Patients' symptoms were assessed preoperatively by using the Unified PD Rating Scale (UPDRS) in the "medication-on" and "medication-off' conditions and quality of life was examined using the 39-item PD Questionnaire (PDQ-39). Patient evaluations were repeated postoperatively during periods of stimulation. The UPDRS activities of daily living (ADL) and motor scores as well as the PDQ-39 total, mobility, ADL, emotional well-being, stigma, and bodily discomfort scores were significantly improved at 12 months compared with baseline scores; the UPDRS ADL and motor scores as well as the PDQ-39 total, mobility, ADL, stigma, and bodily discomfort scores were significantly improved at the longest follow-up examination compared with baseline scores. There was a strong correlation between UPDRS motor and ADL scores and the PDQ-39 total, mobility, and ADL scores. Further analyses indicated that improvements in tremor were only correlated with PDQ-39 ADL subscale scores and rigidity was not correlated with any aspect of quality of life. Nevertheless, bradykinesia was strongly correlated with improvements in the PDQ-39 total, mobility, and ADL scores. CONCLUSIONS: Improvements in quality of life following bilateral DBS of the STN are maintained in the long term. These improvements are strongly correlated with improvements in motor function, primarily with regard to bradykinesia.

Activities of Daily Living↗

Protection of nigral cell death by bilateral subthalamic nucleus stimulation.

In Parkinson disease (PD), the subthalamic nucleus (STN) becomes hyperactive (disinhibited), which is reported to cause excitotoxic damage to midbrain dopaminergic neurons. Here, we examined whether silencing of the hyperactive STN by chronic bilateral deep brain stimulation (DBS) increased the survival of midbrain dopaminergic neurons in a rat model of PD. High-precision design-based stereologic examination of the total number of neurons and tyrosine tydroxylase (TH) immunoreactive neurons in the substantia nigra pars compacta revealed that STN DBS resulted in a significant survival of these neurons. These data provide the first evidence in vivo that bilateral STN DBS is useful for protecting midbrain dopaminergic neurons from cell death in PD.

Adrenergic Agents↗

A young onset Parkinson's patient: a case study.

Young onset Parkinson's disease (YOPD) is defined as idiopathic Parkinson's disease (IPPD) occurring in people between 21 and 40 years of age; it strikes approximately 5% of Parkinson's patients. YOPD has earlier onset of motor complications than later onset Parkinson's disease. Motor complications and disease progression are responsible for devastating morbidity. Current medical and surgical treatments can dramatically ameliorate motor complications and help maintain function and employment. Patient education, support, and advocacy provided by nursing staff can influence the treatment options for these patients, having a significant effect on the future course of the disease. This case history documents the course of a YOPD patient with unusually severe motor complications. He is the only patient at Puget Sound Neurology ever to develop rhabdomyolysis due to dyskinesias. Following bilateral subthalamic nucleus deep brain stimulation, his Parkinson's symptoms have improved dramatically, and his motor complications are significantly improved.

Activities of Daily Living↗

Visualization and navigation system development and application for stereotactic deep-brain neurosurgeries.

We present the development of a visualization and navigation system and its application in pre-operative planning and intra-operative guidance of stereotactic deep-brain neurosurgical procedures for the treatment of Parkinson's disease, chronic pain, and essential tremor. This system incorporates a variety of standardized functional and anatomical information, and is capable of non-rigid registration, interactive manipulation, and processing of clinical image data. The integration of a digitized and segmented brain atlas, an electrophysiological database, and collections of final surgical targets from previous patients facilitates the delineation of surgical targets and surrounding structures, as well as functional borders. We conducted studies to compare the surgical target locations identified by an experienced stereotactic neurosurgeon using multiple electrophysiological exploratory trajectories with those located by a non-expert using this system on 70 thalamotomy, pallidotomy, thalamic deep-brain stimulation (DBS), and subthalamic nucleus (STN) DBS procedures. The average displacement between the surgical target locations in both groups was 1.95 +/- 0.86 mm, 1.83 +/- 1.07 mm, 1.88 +/- 0.89 mm and 1.61 +/- 0.67 mm for each category of surgeries, respectively, indicating the potential value of our system in stereotactic deep-brain neurosurgical procedures, and demonstrating its capability for accurate surgical target initiation.

Adult↗

Motor responses of muscles supplied by cranial nerves to subthalamic nucleus deep brain stimuli.

The distribution of human corticobulbar motor excitatory and inhibitory output is not fully understood. In particular, it is unclear whether the pattern of innervation is the same for upper and lower facial muscles, and what is the motor cortical area giving rise to such innervation. We used electrodes implanted in the subthalamic nucleus (STN) in patients with Parkinson's disease to activate motor tracts at a subcortical level. We examined the excitatory and inhibitory effects of unilateral single STN deep brain stimulation (sSTN-DBS) in 14 patients by taking recordings from facial, cervical and upper limb muscles on both sides. We measured the latency and amplitude of the motor-evoked potentials (MEPs), and the latency and duration of the silent periods, and compared ipsilateral with contralateral responses and responses obtained in different muscles. Unilateral sSTN-DBS induced strictly contralateral MEPs in the trapezius, deltoid, biceps and thenar muscles. The same stimulus always induced bilateral MEPs in the orbicularis oculi, orbicularis oris, masseter and sternocleidomastoid at a mean latency in the range 6.0-9.1 ms. MEP latencies in the orbicularis oculi and orbicularis oris were significantly longer than in the masseter and sternocleidomastoid (P < 0.01). A short latency small action potential was recorded in the ipsilateral orbicularis oculi that was likely generated by activation of extraocular muscles. During sustained voluntary muscle contraction, a silent period was recorded at similar onset latency on both sides. This period was significantly shorter in orbicularis oculi than in masseter, and in the ipsilateral side for both muscles (P < 0.01). sSTN-DBS is able to activate the descending projecting fibres in the corticobulbar tract eliciting bilateral MEPs and silent periods in facial and cranial muscles. This suggests that fibres to both ipsi- and contralateral motor nuclei descend together at the level of the STN. These findings are relevant in the discussion of the innervation of upper and lower facial muscles in humans and in the interpretation of previous results obtained with transcranial cortical stimulation.

Aged↗

Extradural motor cortex stimulation (EMCS) for Parkinson's disease. History and first results by the study group of the Italian neurosurgical society.

The preliminary results obtained by the Study Group for Treatment of Involuntary Movements by Extradural Motor Cortex Stimulation (EMCS) of the Italian Neurosurgical Society, are reported. The series includes 16 cases of very advanced Parkinson's Disease (PD), aged 46-81; 15 of them were not eligible for Deep Brain Stimulation. Ten cases have been evaluated at 3-30 months after implantation. Unilateral, sub-threshold extradural motor cortex stimulation (2 8 Volt, 100-400 microsec., 20-120 Hz) by chronically implanted electrodes, relieves, at least partially, but sometime dramatically, the whole spectrum of symptoms of advanced PD. Tremor and rigor bilaterally in all limbs and akinesia are reduced. Standing, gait, motor performance, speech and swallowing are improved. Benefit is marked as far as axial symptoms is concerned. Also the symptoms of Long Term Dopa Syndrome -dyskinesias, motor fluctuations - and other secondary effect of levodopa administration psychiatric symptoms - are improved. Levodopa dosage may be reduced by 50%. The effect seems persistent and does not fade away with time. Improvement ranged, on the basis of the UPDRS scale, from <25% to 75%. There was only one case of complete failure. Quality of life is markedly improved in patients who were absolutely incapable of walking and unable arise out of chair. After stimulation they could walk, even if assistance was necessary. Improvement was observed also in those with disabling motor fluctuation and dyskinesias which could be abolished.

Aged↗

Improvement of upper limb ataxia and intention tremor allowing cessation of thalamic electrostimulation after four years.

A 34-year-old female patient suffering from multiple sclerosis was treated with thalamic electrostimulation for right dominant brachial ataxia and intention tremor. At the end of the fourth year, the energy of the battery of the impulse generator was depleted. However, the patient was able to use her hands without major impairment with the stimulator OFF; due to a stable reduction of the degree of ataxia and intention tremor. The opposite thalamus received an electrode that was never activated because of a permanent thalamotomy effect. Thus, it can be concluded that restoration of function by means of deep brain stimulation might have been beneficial towards achieving a reorganization and stabilization of subcortico-cortical and cerebellar circuitry, supporting the process of self-repair in this patient with a less aggressive course of multiple sclerosis. However, scientific proof has yet to be found. This exceptional observation emphasizes the need for longterm studies.

Adult↗

Neurosurgical treatment of multiple sclerosis.

OBJECTIVES: To describe the role of neurosurgical techniques in treatment of symptoms of multiple sclerosis (MS). METHODS: A review of the existing world literature searching (using PubMed, August 22, 2005) for key words 'multiple sclerosis' and multiple symptoms associated with MS including trigeminal neuralgia, pain (pump and rhizotomy), spasticity, intrathecal pump, hydrocephalus and stimulation (brain and spinal). RESULTS: In total, 1,404 articles met selection criteria. These were narrowed based upon uniqueness of technique described, nature of information provided and avoidance of redundancy. Further, the methods listed were classified into groups of cranial and spinal techniques. Indications, brief description of technique and outcome/side effects were noted for each technique. DISCUSSION: A vast, growing body of literature exists for various neurosurgical applications for MS treatment. Older techniques such as rhizolysis for neuralgia and rhizotomy for spasticity have been modified using stereotactic radiosurgery and deep brain stimulation, along with central nervous system stimulation and intrathecal pumps. Other applications continue to be found with varying degrees of success. CONCLUSIONS: Neurosurgical techniques, based upon proper selection criteria, can significantly improve MS-related symptoms with low procedure-associated risk and therefore can substantially improve patients' quality of life.

Deep Brain Stimulation↗

Development and application of functional databases for planning deep-brain neurosurgical procedures.

This work presents the development and application of a visualization and navigation system for planning deep-brain neurosurgeries. This system, which incorporates a digitized and segmented brain atlas, an electrophysiological database, and collections of final surgical targets of previous patients, provides assistance for non-rigid registration, navigation, and reconstruction of clinical image data. The fusion of standardized anatomical and functional data, once registered to individual patient images, facilitates the delineation of surgical targets. Our preliminary studies compared the target locations identified by a non-expert using this system with those located by an experienced neurosurgeon using regular technique on 8 patients who had undergone subthalamic nucleus (STN) deep-brain stimulations (DBS). The average displacement between the surgical target locations in both groups was 0.58 mm +/- 0.49 mm, 0.70 mm +/- 0.37 mm, and 0.69 mm +/- 0.34 mm in x, y, and z directions respectively, indicating the capability of accurate surgical target initiation of our system, which has also shown promise in planning and guidance for other stereotactic deep-brain neurosurgical procedures.

Artificial Intelligence↗

Frameless localization for functional neurosurgical procedures: a preliminary accuracy study.

BACKGROUND: Targeting for functional stereotactic procedures is traditionally carried out in a noninteractive fashion, without real-time positional feedback. In addition, stereotactic frames are uncomfortable for patients and may impede intraoperative neurological evaluation. As an initial step toward a fully frameless approach to functional surgery, we have investigated the use of an image-guided microdrive coupled to a stereotactic frame system. METHODS: For initial laboratory tests, a stereotactic phantom was imaged using high-resolution CT scanning. Three representative targets were chosen within the phantom. Targeting was carried out in the usual fashion using the StealthStation planning suite, utilizing the Radionics CRW system. An LED-equipped reference arc was attached to the CRW base ring. Registration of the base ring was accomplished using a spherical probe. A custom-built microdrive was fitted with an LED array, mounted on the CRW arc and tracked by the StealthStation. The distance between the Stealth real-time localization and the CRW localization was measured on-screen. To evaluate the accuracy of the system in the operating room, a similar procedure was carried out in 13 functional neurosurgical operations (pallidotomy or deep brain stimulator placement). RESULTS: Errors of localization in the laboratory setting ranged from 0.53 to 0.70 mm. In 11 operative cases, the average difference between the CRW localization and the Stealth localization was 1.77 mm. In the remaining 2 cases, equipment malfunction prevented measurement of localization error. CONCLUSIONS: Frameless image-guided localization compares favorably to targeting performed noninteractively. In addition, real-time positional feedback confers advantages in target region visualization and confidence in placement of lesions and stimulators for functional procedures. With small improvements in accuracy and system reliability, fully frameless functional procedures could be safely carried out.

Deep Brain Stimulation↗