OBJECTIVES: Pallidal stimulation and pallidotomy are known to improve the symptoms of Parkinsons disease (PD). However, it is not known which modality produces greater benefit in patients who have already undergone unilateral pallidotomy. It is also suggested that the original pallidal surgery provides a greater benefit than subsequent pallidal surgery. The aim of this study was to analyze which modality produced greater PD symptom improvement in patients with a prior pallidotomy and whether the chronological order of the pallidal surgery influenced the size of the improvement. METHODS: Five patients who had undergone a prior unilateral pallidotomy for PD were studied. Because of ongoing Parkinsonian symptoms, all patients subsequently underwent contralateral pallidal surgery, either a further pallidotomy or pallidal stimulation. All surgeries were performed by a single functional neurosurgeon and the patients prospectively assessed and scored at routine follow-ups. Paired-sample t-tests were used to
OBJECT: The object of this study was to identify a preoperative physiological index by using surface electromyography (EMG) signals that would correlate with clinical outcome in dystonic patients following bilateral pallidal stimulation. METHODS: In 14 patients with spasmodic torticollis, generalized dystonia, and myoclonic dystonia, surface EMG signals were recorded from the most affected muscle groups. Although the dystonia affected different body segments, the EMG signals in all patients could be decomposed into bursting and sustained components. Subsequently, a ratio of the EMG amplitude was calculated between the two components and then correlated with clinical outcome. Patients who experienced rapid improvement following bilateral pallidal stimulation had a significantly higher EMG ratio compared with those who did not. Furthermore, a significant correlation was found between the EMG ratio and clinical improvement during the 12-month period following pallidal stimulation. CONCLUSIONS: The authors
The medial globus pallidus (or internal globus pallidus, GPi) is one of the output nuclei of the basal ganglia (the other being the substantia nigra pars reticulata). The GABA-containing neurons send their axons to specific nuclei of the dorsal thalamus (VA and VL), to the centromedian complex and to the pedunculopontine complex.[1][2]. The efferent bundle is constituted first of the ansa and fasciculus lenticularis, then crosses the internal capsule as the Edingers comb system then arrives at the laterosuperior corner of the subthalamic nucleus and constitutes the field H2 of Forel, then H, and suddenly changes its direction to form field H1 that goes to the inferior part of the thalamus. The distribution of axonal islands is widespread in the lateral region of the thalamus. The innervation of the central region is done by collaterals.[3]. The medial globus pallidus contains GABAergic neurons, which allow for its inhibitory function. As the medial globus pallidus, along with the substantia ...
Objective: To explore the role of neuronal activity in the globus pallidus internus (GPi) in the generation of tic movements.. Methods: We studied eight Tourette syndrome patients with medically intractable tics who underwent a unilateral pallidotomy for severe tics. They ranged in age from 17-24 years; disease duration was 7-19 years. Microelectrode recording was performed in the GPi. The electromyogram (EMG) was simultaneously recorded in muscle groups appropriate for the patients tics. The relationship between neuronal firing pattern and the EMG was studied.. Results: Two hundred and thirty-two neurons were recorded during tics from 8 trajectories. Of these neurons, in addition to decreased neuronal firing rate and irregular firing pattern, 105 (45%) were tic-related showing either a burst of activity or a pause in ongoing tonic activity. They could be synchronous (n=75), earlier than EMG onset (n=27), or following EMG onset (n=3). The GPi neuronal bursts preceded EMG onset with decreased ...
In the current era of functional surgery for movement disorders, deep brain stimulation (DBS) of the globus pallidus internus (GPi) is emerging as the favoured intervention for patients with dystonia. Here we report our results in 20 patients with medically intractable dystonia treated with GPi stimulation. The series comprised 14 patients with generalized dystonia and six with spasmodic torticollis. Although comparisons were limited by differences in their respective neurological rating scales, chronic DBS clearly benefited both patient groups. Data conveying the rate of change in neurological function following intervention are also presented, demonstrating the gradual but progressive and sustained nature of improvement following stimulation of the GPi in dystonic patients.
Abstract: Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep brain stimulation in patients with neurodegeneration with brain iron accumulation. A total of 16 centres contributed 23 patients with confirmed neurodegeneration with brain iron accumulation and ...
We report on the clinical efficacy of bilateral globus pallidus internus deep brain stimulation in two patients with myoclonus dystonia/essential myoclonus who lack mutations in the epsilon sarcoglycan gene. The primary outcome measures were the Burke-Fahn-Marsden Dystonia Scale motor severity and the Unified Myoclonus Rating Scale scores, and the secondary outcome measure was the 36-item Short Form Health Survey score at the last postoperative follow up. Neuronal firing rates were also calculated from microelectrode recordings.
The striatum is divided into the caudate nucleus and the putamen by the internal capsule, a major collection of fibers that run between the neocortex and thalamus in both directions [16]. The striatum is the main receiver of inputs to the basal ganglia from the cerebral cortex, thalamus, and brain stem. The striatums neurons project out into the globus pallidus and substantia nigra. The globus pallidus and substantia nigra give rise to the major output projections from the basal ganglia [21]. Anatomically, the globus pallidus is situated medially to the putamen and is positioned slightly laterally to the internal capsule. It is divided into an external and internal segment. The internal pallidal segment relates functionally to the pars reticulata of the substantia nigra, which lies in the midbrain on the medial side of the internal capsule [10]. The cells of the internal pallidal segment and pars reticulata use amino butyric acid GABA as a neurotransmitter, which is responsible for the ...
Distinct populations of D1- and D2-dopamine receptor expressing medium spiny neurons (D1-/D2-MSNs) comprise the nucleus accumbens, and activity in D1-MSNs promotes, while activity in D2-MSNs inhibits motivated behaviors. We used chemogenetics to extend D1-/D2-MSN cell specific regulation to cue-reinstated cocaine seeking in a mouse model of self-administration and relapse, and found that either increasing activity in D1-MSNs or decreasing activity in D2-MSNs augmented cue-induced reinstatement. Both D1- and D2-MSNs provide substantial GABAergic innervation to the ventral pallidum, and chemogenetic inhibition of ventral pallidal neurons blocked the augmented reinstatement elicited by chemogenetic regulation of either D1- or D2-MSNs. Since D1- and D2-MSNs innervate overlapping populations of ventral pallidal neurons, we next used optogenetics to examine whether changes in synaptic plasticity in D1- versus D2-MSN GABAergic synapses in the ventral pallidum could explain the differential regulation ...
OBJECTIVE: To study the functional accuracy of stereotactic targeting for the posteroventral pallidotomy (PVP), comparing targets chosen on magnetic resonance images (MRI), and fused MRI to computed tomographic (CT) images, with electrophysiologicall
As part of the basal ganglia circuitry, these anatomic locations play a critical role in the control of movement. The effects of stimulating these brain regions are indicated in the Table. Thalamic (Vim) stimulation is only effective for tremor, not for the other symptoms of Parkinsons disease. Stimulation of the globus pallidus or subthalamic nucleus, in contrast, may benefit not only tremor but also other parkinsonian symptoms such as rigidity, bradykinesia, dyskinesias, and gait problems. For most patients with Parkinsons disease, DBS of the globus pallidus or subthalamic nucleus are more appropriate choices than thalamic DBS because stimulation at these targets affects a broader range of symptoms. A recent studycompared the outcomes of 300 patients who were randomized to deep brain stimulation of either the globus pallidus interna or subthalamic nucleus (Follett et al 2010). Two years after surgery, there was no clear advantage to either location. Patients undergoing subthalamic ...
The objective of this study was to explore the functional anatomy of the globus pallidus internus (GPi) by studying the effects of unilateral pallidotomy on parkinsonian off signs and levodopa-induced dyskinesias (LID), We found significant positive! correlations between the preoperative levodopa responsiveness of motor signs and the levodopa responsiveness of scores in timed tests (Core Assessment Program for Intracerebral Transplantations) in the contralateral limbs and the improvement in these scores after surgery, whereas there was no correlation with the improvement in LID, We also found a highly significant correlation (P , 0,0001, p = 0.8) between the volume of the ventral lesion in the GPi and the improvement in LID in the contralateral limbs, whereas there was no correlation between the ventral volume and the improvement in parkinsonian off signs. The volumes of the total Lesion cylinder and the dorsal lesion did not correlate with the outcome of either dyskinesias or parkinsonian ...
Olga Waln and Joseph Jankovic Bilateral globus pallidus internus deep brain stimulation after bilateral pallidotomy in a patient with generalized early-onset primary dystonia Movement Disorders 28. Version of Record online: 11 APR 2013 , DOI: 10.1002/mds.25456. Complete the form below and we will send an e-mail message containing a link to the selected article on your behalf. Required = Required Field. ...
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The globus pallidus (Latin for "pale globe") also known as paleostriatum, is a sub-cortical structure of the brain. It is part of the telencephalon, but retains close functional ties with the subthalamus - both of which are part of the extrapyramidal motor system. The globus pallidus is a major component of the basal ganglia core along with the striatum and its direct target, the substantia nigra. The latter are made up of similar neuronal elements, have similar afferents from the dorsal striatum, and have a similar synaptology; neither receives cortical afferents.. Function. The globus pallidus is a structure in the brain involved in the regulation of voluntary movement. It is part of the basal ganglia, which, among many other things, regulate movements that occur on the subconscious level. If the globus pallidus is damaged, it can cause movement disorders, as its regulatory function will be impaired. There may be cases in which damage is deliberately induced, as in a procedure known as a ...
Two major neurological disorders - Parkinsons disease and dystonia - are believed to involve pathology in the activity of the basal ganglia, a subcortical brain structure, whose output nuclei (internal Globus Pallidus, GPi) projects to thalamus and modulates thalamocortical relay. While these disorders may ultimately involve different network and cellular pathologies, some pathological physiology may be shared between them because surgical treatment of both conditions includes surgical lesion or electrical stimulation to GPi (pallidotomy and GPi DBS). This work compares the thalamocortical relay responses to inhibitory inputs from internal segment of GPi in Parkinsons disease and in dystonia. ...
This work examined the electrophysiological correlates of chorea induced by bicuculline microinjection into the GPe. The behavioral effects in the present study are in line with those described earlier (Grabli et al. 2004), showing a direct relationship between the location of injections in the GPe and the induced abnormal hyperbehavioral symptoms. Bicuculline caused a marked increase in the firing rate of GPe neurons, which was associated with drastic changes in firing pattern. In some bicuculline-affected GPe neurons, pauses became more pronounced (BHF), whereas in others they were almost completely abolished (CHF). These abnormal activity patterns were associated with a reduction in the information capacity of neurons. Bicuculline also induced regularity in both the spiking and bursting activity in the GPe. However, there was very little correlated activity in the GPe either in the temporal or in the spectral domains. There were no gross changes in the activity of GPi neurons after GPe ...
Pathophysiology of Parkinsons disease (PD) is characterized by increased firing rates of cells in the basal ganglia, a tendency toward bursting and abnormal synchronization in cells of subthalamic nucleus (STN) and globus pallidus pars externa (GPe) [1]. In advanced PD, deep brain stimulation (DBS) can be used to disrupt this pathological activity. The standard protocol for DBS is continuous high frequency stimulation of target cells, such as STN. It has been proposed that short-duration stimulation protocols may also disrupt the pathological activity [2]. The mechanism underlying this protocol is supposedly synaptic plasticity. The goal of this study is to investigate, with a biophysically plausible model, the role of synaptic plasticity in stabilizing firing patterns in the basal ganglia. ...
In this study, we found that differential axonal projections and apical tuft structures segregate corticostriatal cells into two types, with CCS cells further differentiated according to their depth-dependent differences in dendritic morphology. This suggests that corticostriatal neurons are heterogeneous according to their extracortical target and the laminar location. Similarly, in the striatum, projection cells are heterogeneous from two independent points of view: the extrastriatal target and intrastriatal location.. In terms of extrastriatal targets, they are divided mainly into two groups (Gerfen and Young, 1988; Kawaguchi et al., 1990; Parent et al., 1995). These two types are considered to affect basal ganglia outputs in opposite ways (Albin et al., 1989; Alexander and Crutcher, 1990). One group exclusively projects to the external pallidal segment (GPe-exclusive cells; indirect pathway), whereas another group, while sending axon collaterals to the external pallidal segment, directly ...
Udall Center of Excellence in Parkinsons Research (Project 2). Sponsor: NIH. Project Description: Over the past few years there has been a resurgence of interest in using the internal segment of the globus pallidus (GPi) as the target for deep brain stimulation (DBS). Yet, despite increased consideration of the pallidum as a target, surprisingly little is known about the neurophysiological characteristics of resting and movement-related activity across the pallidum (from the ventral GPi to the external segment of the pallidum, GPe) or the optimal site and frequency of stimulation required to produce the therapeutics effects. The goal of this project is to gain a greater understanding of the mechanisms, locations and pathways mediating the effects (both adverse and beneficial) of pallidal DBS on clinical and quantitative measures of motor function, including speech.. The experiments will be conducted in individuals with chronically implanted stimulators in the pallidum. A subset of subjects will ...
Deep brain stimulation (DBS) is a type of brain surgery used to treat dystonia. During surgery, two small holes will be drilled into your skull.. The surgeon will pass electrodes through each hole and position them in a part of the basal ganglia called the globus pallidus. The basal ganglia is part of the brain that affects muscle movement.. The electrodes will be connected to a small pulse generator thats similar to a pacemaker. It will be implanted under your skin, usually on your chest or lower abdomen.. The pulse generator sends signals to the globus pallidus. This alters nerve impulses produced by the basal ganglia and improves the symptoms of dystonia.. The most common complication of DBS is that either the pulse generator stops working or the electrodes become displaced, which may require further surgery to correct.. DBS is a relatively new technique, so theres little information regarding its long-term safety or effectiveness. Therefore, before deciding to have DBS, you should discuss ...
Deep brain stimulation (DBS) is a type of brain surgery used to treat dystonia. During surgery, two small holes will be drilled into your skull.. The surgeon will pass electrodes through each hole and position them in a part of the basal ganglia called the globus pallidus. The basal ganglia is part of the brain that affects muscle movement.. The electrodes will be connected to a small pulse generator thats similar to a pacemaker. It will be implanted under your skin, usually on your chest or lower abdomen.. The pulse generator sends signals to the globus pallidus. This alters nerve impulses produced by the basal ganglia and improves the symptoms of dystonia.. The most common complication of DBS is that either the pulse generator stops working or the electrodes become displaced, which may require further surgery to correct.. DBS is a relatively new technique, so theres little information regarding its long-term safety or effectiveness. Therefore, before deciding to have DBS, you should discuss ...
Bowes E; Levy F; Lawson J; Mandalis A; Mohan A; Weickert CS, 2015, Anti-N-methyl-D-Aspartate encephalitis - a case study of symptomatic progression, Australasian Psychiatry, vol. 23, pp. 422 - 425, http://dx.doi.org/10.1177/1039856215588229. Sachdev PS; Mohan A; Taylor L; Jeste DV, 2015, DSM-5 and mental disorders in older individuals: An overview, Harvard Review of Psychiatry, vol. 23, pp. 320 - 328, http://dx.doi.org/10.1097/HRP.0000000000000090. Sachdev PS; Mohan A; Cannon E; Crawford JD; Silberstein P; Cook R; Coyne T; Silburn PA, 2014, Deep brain stimulation of the antero-medial globus pallidus interna for Tourette syndrome, PLoS ONE, vol. 9, http://dx.doi.org/10.1371/journal.pone.0104926. Mohan A; Lee T; Sachdev P, 2014, Surviving acute cyanide poisoning: a longitudinal neuropsychological investigation with interval MRI, BMJ case reports, vol. 2014, http://dx.doi.org/10.1136/bcr-2013-203025. Shiner E; Taylor L; Mohan A; Watson S; Sachdev PSI, 2014, Severe depression masquerading ...
In order to continuously support the European computing infrastructures and to exploit possible synergies, the Initiative for Globus in Europe (IGE) coordinates European Globus activities, drive forward Globus developments according to the requirements of European users, and strengthen the influence of European developers in the Globus Alliance.
These results, Dr. Joseph Mercola commented, "revealed areas of high intensity, or hyperintensity, in two brain regions (the dentate nucleus (DN) and globus pallidus (GP)), which correlated with the number of gadolinium-based enhanced MRIs. Its unknown at this time what the hyperintensity may mean, however hyperintensity in the DN is associated with multiple sclerosis. Its now being suggested that this hyperintensity may be the result of the large number of enhanced MRI scans often received by multiple sclerosis patients. Hyperintensity of the GP, meanwhile, is linked with liver dysfunction. The studys lead author noted, Hyperintensity in the dentate nucleus and globus pallidus on unenhanced MRI may be a consequence of the number of previous Gd-CM administrations… Because gadolinium has a high signal intensity in the body, our data may suggest that the toxic gadolinium component remains in the body even in patients with normal renal function. " (Dr. Mercola, 2014.) ...
Iron levels in the basal ganglia steadily increase throughout development and, in two sub-regions, continue to increase into adulthood. Decreased levels of iron in the putamen was correlated with impaired cognitive performance involving reasoning and spatial processing. Findings suggest the brain requires iron for healthy cognitive development.... Read More... ...
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For air departure dates within 331 days, youll be offered a choice of carriers and flight schedules as well as price points to choose from when selecting your flights. Your schedule will be provided to you at the time of booking (schedules are subject to change by carriers). Globus offers 2 air options: Flex Air and Instant Purchase Air. Flex Air allows flexibility if you want to make changes to your vacation schedule. Instant Purchase Air may offer additional airline options but has more restrictions. Some vacations may only offer one Air type option. Guaranteed air-inclusive prices: air is only available to passengers traveling from the United States and only available when booked in conjunction with a land vacation. An additional $300 per person non-refundable air deposit is required for Flex Air booked in conjunction with any land vacation (i.e. air-inclusive vacation). Air can only be booked by Globus in conjunction with a vacation package. Instant Purchase Air requires air payment in full ...
Striatala medium spiny neuroner (MSNs) spelar en stor roll för olika motoriska och kognitiva funktioner. Beroende på huruvida dessa neuroner uttrycker dopaminreceptorer av D1- eller D2-typ, klassificeras de som tillhörande den direkta (dMSN) respektive den indirekta (iMSN) vägen genom basala ganglierna. I denna avhandling undersöker jag hur inputet från kortex till de två typerna av MSNs processas och jag karakteriserar aktiviteten från dMSNs, samt undersöker även vilken effekt avvikande iMSN aktivitet ger upphov till i det basala ganglienätverk som består av den subthalamiska kärnan (STN) och globus pallidus externa (GPe).För att verifiera resultaten från en tidigare modelleringsstudie, som predicerat att dMSNs erhåller fler eller får starkare inputs från kortex jämfört med iMSNs, analyserade jag in vivo data från MSN wholecell registreringar gjorda i nedsövda möss som antingen tillhört en kontroll-grupp (friska möss) eller en grupp där ...
HIPPI in neurons and neuropil throughout the brain: cortex , striatum, globus pallidus, hypothalamus and cerebellum. 55kDa by WB. Tissues (except skin) expressing HIPPI also express Hip- ...
When I just tried this, I felt anxious during the first breath and the second breath felt soothing. Thats what happens for most people. Our breath tends to be short and shallow when we feel worried, so taking long, slow breaths can help us to feel calm.. There are several more suggested breathing practices in a post at MindfulTeachers.org on Breath-Based Practices for Mindfulness or Stress Reduction. 2: Knowledge. Many youth find it helpful to understand whats going on in their brains when they have strong feelings. Theres no need to overwhelm them with a detailed description of every single part of the brain, from the medulla oblongata to the globus pallidus. But its important not to be overly simplistic, either.. Some kids are being taught that the thinking part of the brain is "good" and should be used to suppress the "bad" emotional parts, like the amygdalae. (See The Magnificent, Mysterious, Wild, Connected, and Interconnected Brain) Its more accurate to say that different parts of ...
The idea behind GRIP was to combine the advantages of the two Grid developments: the seamless access to resources of UNICORE and the wide support that already exists for Globus," says Erwin. "In the first year of the project, we successfully completed our objective of making Globus resources accessible from the UNICORE interface. This was done by extending some of the components within UNICORE and without having to modify Globus in any way. We demonstrated various applications in the meteorological field and molecular science applications. Our GRIP partners in Warsaw use GRIP for bio-molecular calculations and on a regular basis. Especially for CPMD (Car-Parrinello molecular dynamics), a graphical interface for performing certain molecular computations has been developed in UNICORE that even a novice can use. The point is that users are no longer constrained to using only locally available applications. So long as UNICORE or Globus have been installed, there is no difference in the user interface ...
Globus Medical, Inc. (NYSE:GMED) Director David D. Davidar sold 55,383 shares of the businesss stock in a transaction dated Monday, December 4th. The stock was sold at an average price of $40.66, for a total transaction of $2,251,872.78. Following the sale, the director now owns 752,236 shares in the company, valued at $30,585,915.76. The sale […]
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Eschmeyer, W.N. (ed.), 2003. Catalog of fishes. Updated database version of March 2003. Catalog databases as made available to FishBase in March 2003. (Ref. 46206 ...
Published In: Bulletin de lAcademie Imperiale des Sciences de St-Petersbourg, sér. 3 15(1): 90. 1871. (Bull. Acad. Imp. Sci. Saint-Petersbourg, sér. 3) ...
흰배박쥐(Antrozous pallidus)는 애기박쥐과 흰배박쥐아과에 속하는 박쥐의 일종이다. 캐나다 서부부터 멕시코 중부 지역까지 분포한다. 흰배박쥐속(Antrozous)의 유일종이다. 흰배박쥐속으로 분류되기도 하는 반겔더박쥐(Bauerus dubiaquercus)와 근연종 관계를 갖는다.[3] 흰배박쥐과(Antrozoidae)로 분류하기도 했으나 현재는 애기박쥐과에 속하는 흰배박쥐아과 또는 애기박쥐아과로 분류한다.[4] ...
Primary cervical dystonia (CD) affects about 20-40/100.000 population. The disease is chronic and life-long. The therapy of choice are local intramuscular Botulinum Toxin injections given every three months. Oral medication such as anticholinergics or dopamine depleting drugs are usually of limited efficacy or their use is limited by intolerable side-effects. About 5-10% of CD patients develop neutralizing antibodies against Botulinum Toxin. Two previous controlled multicenter trials have shown the efficacy and safety of bilateral pallidal stimulation in patients with primary segmental and generalized dystonia (one study was performed by our group).. Following surgery, patients will be randomized 1:1 to verum or placebo stimulation for a period of three months. Primary outcome measure is the TWSTRS (Toronto Western Spasmodic Torticollis Rating Scale) - a validated and widely accepted physician-based outcome measure for cervical dystonia. The independent TWSTRS raters are movement disorders ...
Deep brain stimulation of the globus pallidus internus alleviates involuntary movements in patients with dystonia. However, the mechanism is still not entirely understood. One hypothesis is that deep brain stimulation suppresses abnormally enhanced synchronized oscillatory activity within the motor cortico-basal ganglia network. Here, we explore deep brain stimulation-induced modulation of pathological low frequency (4-12 Hz) pallidal activity that has been described in local field potential recordings in patients with dystonia. Therefore, local field potentials were recorded from 16 hemispheres in 12 patients undergoing deep brain stimulation for severe dystonia using a specially designed amplifier allowing simultaneous high frequency stimulation at therapeutic parameter settings and local field potential recordings. For coherence analysis electroencephalographic activity (EEG) over motor areas and electromyographic activity (EMG) from affected neck muscles were recorded before and immediately after
Recent landmark studies have demonstrated the effectiveness of STN and GPi DBS for appropriate Parkinson disease patients.{ref82} In a randomized, controlled trial of 255 patients enrolled in the Vete... more
Deep brain stimulation (DBS) is the technique of neurostimulation of deep brain structures for the treatment of conditions such as essential tremor, dystonia, Parkinsons disease and chronic pain syndromes. The procedure uses implanted deep brain stimulation electrodes connected to extension leads and an implantable pulse generator (IPG). Hardware failure related to the DBS procedure is not infrequent, and includes electrode migration and disconnection. We describe a patient who received bilateral globus pallidus internus DBS for dystonia with initially good clinical response, but the device eventually failed. Radiographs showed multiple twisting of the extension leads with disconnection from the brain electrodes and a diagnosis of Twiddlers syndrome was made. Twiddlers syndrome was first described in patients with cardiac pacemakers. Patients with mental disability, elderly and obese patients are at increased risk. Twiddlers syndrome should be suspected whenever there is a failure of the DBS device
Dystonia is a disabling condition characterised by involuntary muscle spasms and abnormal postures. Its pathophysiology is incompletely understood but most lines of evidence point to an underlying defect of basal ganglia function leading to abnormal corticomotor output. Various abnormalities have been shown, including abnormal neuronal activity in basal ganglia output nuclei, defective neural inhibition at the spinal, brainstem, cortical level and sensorimotor misprocessing. More recently, increased neural plasticity has been found in dystonia patients in response to transcranial magnetic stimulation (TMS) protocols which induce motor cortex plasticity. Excessive plasticity might contribute to dystonia by promoting or reinforcing abnormal patterns of connectivity. The most significant advance in the treatment of generalised dystonia has been the development of globus pallidus internus (GPi) deep brain stimulation (DBS). Interestingly its beneficial effects are progressive over weeks to months ...
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Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep brain stimulation in patients with neurodegeneration with brain iron accumulation. A total of 16 centres contributed 23 patients with confirmed neurodegeneration with brain iron accumulation and bilateral ...
We investigated the effect of pallidal deep brain stimulation (GPi-DBS) in dystonia on cognition, mood, and quality of life and also assessed if DYT1 gene status influenced cognitive outcome following GPi-DBS. Fourteen patients with primary generalized dystonia (PGD) were assessed, measuring their estimated premorbid and current IQ, memory for words and faces, and working memory, language, executive function, and sustained attention, one month before and one year or more after surgery. Changes in mood and behaviour and quality of life were also assessed.
The anterograde transport of Phaseolus vulgaris-leucoagglutinin (PHA-L) was combined with postembedding immunocytochemistry for gamma-aminobutyric acid (GABA) to study the topography, the synaptic organization and the neurotransmitter content of the pallidosubthalamic projection in the rat. After injections of PHA-L in different parts of the globus pallidus a rich plexus of anterogradely labelled fibres and terminals was found in the ipsilateral subthalamic nucleus. The immunoreactive elements were distributed according to a mediolateral and rostrocaudal topography. Injections of PHA-L restricted to the lateral two-thirds of the globus pallidus gave rise to a massive anterograde labelling confined to the lateral half of the subthalamic nucleus. On the other hand, injections of PHA-L strictly confined to the medial part of the globus pallidus resulted in anterograde labelling that occupied the ventromedial pole of the subthalamic nucleus. In some cases a few retrogradely labelled cells were found ...
Abbreviations AADC: aromatic L-amino acid decarboxylase. Aβ: amyloid β. ACh: acetylcholine. AChE: acetylcholinesterase. AD: Alzheimer disease. ALDH: aldehyde dehydrogenase. ALS: amyotrophic lateral sclerosis. apoE: apolipoprotein E. APP: amyloid precursor protein. BuChE: butyrylcholinesterase. CNS: central nervous system. COMT: catechol-O-methyltransferase. DA: dopamine. DAT: DA transporter. DβH: dopamine-β-hydroxylase. DOPAC: 3,4-dihydroxyphenylacetic acid. GABA: γ-aminobutyric acid. Glu: glutamatergic. GPe: globus pallidus extern. GPi: globus pallidus interna. HD: Huntington disease. 5HT: serotonin. HVA: homovanillic acid. MAO: monamine oxidase. MCI: mild cognitive impairment. MPTP: N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. 3MT: 3-methoxyltyramine. NE: norepinephrine. NET: NE transporter. NMDA: N-methyl-D-aspartate. 3-OMD: 3-O-methyl dopa. PD: Parkinson disease. PDD: Parkinson disease dementia. PET: positron emission tomography. PH: phenylalanine hydroxylase. REM: rapid eye ...
Patients mean age was 41 years, ranging from 28 to 49 years with a mean duration of antipsychotic exposure of 3.7 years. Patients 2 and 3 received first-generation antipsychotics and patient 1 was administered second-generation medication. Preoperatively, all patients required either assisted living or was homebound. The craniospinal regions were the most seriously affected regions and all demonstrated opisthotonus and retrocollis. Chronic rhythmic neck hyperextension movements resulted in premature cervical spondylosis, and patients 1 and 3 required nasogastric tube feeding. At the time of surgery, all were unable to walk independently. The mean preoperative BFMDRS score was 61, ranging from 44 to 80, and mean GDS score was 47, ranging from 40 to 52. There were no treatment-related complications and the procedure was well tolerated. As expected, there was no minimal response before stimulation but after pulse generator activation, marked amelioration of dystonic symptoms was observed. The ...
Recent publications have demonstrated that deep brain stimulation for Parkinsons disease still exerts beneficial effects on tremor, rigidity, and bradykinesia for up to 10 years after implantation of the stimulator. However with the progression of Parkinsons disease, features such as cognitive decline or freezing become prominent, and the presence of an implanted and functioning deep brain stimulator can impose a profound burden of care on the clinical team and family. The authors describe their experience in treating 4 patients who underwent removal of the implanted device due to either progressive dementia requiring full-time nursing or due to infection, and who subsequently underwent a unilateral pallidotomy.