Globus Pallidus Internus Deep Brain Research Articles

Deep brain stimulation for Gilles de la Tourette syndrome: A case series targeting subregions of the globus pallidus internus

Deep brain stimulation remains an experimental treatment for patients with Gilles de la Tourette syndrome. Currently, a major controversial issue is the choice of brain target that leads to optimal patient outcomes within a presumed network of basal ganglia and cortical pathways involved in tic pathogenesis. This report describes our experience with patients with severe refractory Gilles de la Tourette syndrome treated with globus pallidus internus deep brain stimulation. Five patients were selected for surgery, 2 targeting the posteroventral globus pallidus internus and 2 targeting the anteromedial region. The remaining patient was first targeted on the posterolateral region, but after 18 months the electrodes were relocated in the anteromedial area. Tics were clinically assessed in all patients pre- and postoperatively using the Modified Rush Video protocol and the Yale Global Tic Severity Scale. Obsessive-compulsive behaviors were quantified with the Yale-Brown Obsessive Compulsive Scale. The Gilles de la Tourette Syndrome-Quality of Life Scale was also completed. All patients experienced improvements in tic severity but to variable extents. More convincing improvements were seen in patients with electrodes sited in the anteromedial region of the globus pallidus internus than in those with posterolateral implants. Mean reduction in the Modified Rush Video Rating scale for each group was 54% and 37%, respectively. Our open-label limited experience supports the use of the anteromedial globus pallidus internus as a promising target for future planned randomized double-blind trials of deep brain stimulation for patients with Gilles de la Tourette syndrome.

The Need for a Multifactorial Approach to Raise the Standard in Deep Brain Stimulation Reporting

Deep brain stimulation (DBS) has been demonstrated to be an effective therapeutic strategy for the treatment of pharmacologically refractory Parkinson disease (PD) ( 1. Kleiner-Fisman G Herzog J Fisman DN et al. Subthalamic nucleus deep brain stimulation: summary and meta-analysis of outcomes. Mov Disord. 2006; 21: S290-S304 Crossref Scopus (761) Google Scholar ). Since its renaissance in 1987 ( 2. Benabid AL Pollak P Louveau A Henry S de Rougemont J Combined (thalamotomy and stimulation) stereotactic surgery of the VIM thalamic nucleus for bilateral Parkinson disease. Appl Neurophysiol. 1987; 50: 344-346 PubMed Google Scholar , 3. Hariz MI Blomstedt P Zrinzo L Deep brain stimulation between 1947 and 1987: the untold story. Neurosurg Focus. 2010; 29: E1 Crossref Scopus (150) Google Scholar ), it has become widely accepted in the movement disorders community with more than 33,000 patients currently implanted. Targets have evolved from the ventral intermediate nucleus of the thalamus ( 2. Benabid AL Pollak P Louveau A Henry S de Rougemont J Combined (thalamotomy and stimulation) stereotactic surgery of the VIM thalamic nucleus for bilateral Parkinson disease. Appl Neurophysiol. 1987; 50: 344-346 PubMed Google Scholar , 4. Benabid AL Pollak P Gervason C et al. Long-term suppression of tremor by chronic stimulation of the ventral intermediate thalamic nucleus. Lancet. 1991; 337: 403-406 Abstract PubMed Scopus (1393) Google Scholar ) to the globus pallidus internus ( 5. Siegfried J Lippitz B Bilateral chronic electrostimulation of ventroposterolateral pallidum: a new therapeutic approach for alleviating all parkinsonian symptoms. Neurosurgery. 1994; 35 (discussion 1129–1130): 1126-1129 Crossref PubMed Scopus (409) Google Scholar ), subsequently to the subthalamic nucleus (STN) ( 6. Pollak P Benabid AL Gross C et al. [Effects of the stimulation of the subthalamic nucleus in Parkinson disease]. Rev Neurol (Paris). 1993; 149: 175-176 PubMed Google Scholar ), and recently to the pedunculopontine nucleus ( 7. Stefani A Lozano AM Peppe A et al. Bilateral deep brain stimulation of the pedunculopontine and subthalamic nuclei in severe Parkinson’s disease. Brain. 2007; 130: 1596-1607 Crossref PubMed Scopus (636) Google Scholar ). While debate exists with respect to the optimal therapeutic target ( 8. Okun MS Foote KD Subthalamic nucleus vs globus pallidus interna deep brain stimulation, the rematch: will pallidal deep brain stimulation make a triumphant return?. Arch Neurol. 2005; 62: 533-536 Crossref PubMed Scopus (64) Google Scholar , 9. Minguez-Castellanos A Escamilla-Sevilla F Pallidal vs subthalamic deep brain stimulation for Parkinson disease: winner and loser or a sharing of honors?. Arch Neurol. 2005; 62 (author reply 1643): 1642-1643 Crossref Google Scholar ), DBS of STN is the most common surgical intervention for PD ( 10. Benabid AL Chabardes S Mitrofanis J Pollak P Deep brain stimulation of the subthalamic nucleus for the treatment of Parkinson’s disease. Lancet Neurol. 2009; 8: 67-81 Abstract Full Text Full Text PDF PubMed Scopus (971) Google Scholar ). DBS of the STN has resulted in substantial motor improvements and often leads to a significant reduction in levodopa administration ( 11. Molinuevo JL Valldeoriola F Tolosa E et al. Levodopa withdrawal after bilateral subthalamic nucleus stimulation in advanced Parkinson disease. Arch Neurol. 2000; 57: 983-988 Crossref PubMed Scopus (191) Google Scholar ). Now that these major advances in motor function have been achieved, the apparent limbic side-effects of DBS have come into greater focus ( 12. Okun MS Green J Saben R Gross R Foote KD Vitek JL Mood changes with deep brain stimulation of STN and GPi: results of a pilot study. J Neurol Neurosurg Psychiatry. 2003; 74: 1584-1586 Crossref PubMed Scopus (93) Google Scholar ).

Micrographia induced by pallidal DBS for segmental dystonia: a subtle sign of hypokinesia?

Recently parkinsonism has been reported as a rare side effect of globus pallidus internus (GPi) deep brain stimulation (DBS) for dystonia. In the present systematic prospective study in 11 patients with segmental dystonia not affecting distal arm function, we could demonstrate significant changes in handwriting characterized by mild micrographia following GPi-DBS. We propose that this finding reflects GPi-DBS-induced disturbances of basal ganglia function in terms of a mild hypokinetic syndrome, as a result of outflow alterations in pallido-thalamo-cortical pathways.

Bilateral globus pallidus internus deep brain stimulation in parkinson’s disease with periodic limb movement disorder

Deep brain stimulation for Parkinson's disease is a well-established therapeutic intervention for refractory disease. The main nuclear targets are the subthalamic nucleus and the globus pallidus internus. Periodic limb movements are often an associated condition in patients with Parkinson's disease. Both conditions can respond to dopaminergic agents. The case of a 57-year-old man with severe Parkinson's disease and periodic limb movement who underwent bilateral globus pallidus deep brain stimulation surgery for his Parkinson's disease is presented and discussed. Intraoperatively the patient's periodic limb movements responded to voltage stimulation higher than those required for improvement of his parkinsonian symptoms. The immediate and reversible improvement in these symptoms contralateral to the stimulation site suggests pallidal stimulation may directly ameliorate periodic limb movement symptoms.

Suicide Without Depression After Withdrawal of a Dopamine Agonist in a Patient With Parkinson's Disease

Back to table of contents Previous article Next article LettersFull AccessSuicide Without Depression After Withdrawal of a Dopamine Agonist in a Patient With Parkinson's DiseaseMélanie Flament, M.D., Gwenolé Loas, M.D., Ph.D., Olivier Godefroy, M.D., Ph.D., and Pierre Krystkowiak, M.D., Ph.D.Mélanie FlamentSearch for more papers by this author, M.D., Gwenolé LoasSearch for more papers by this author, M.D., Ph.D., Olivier GodefroySearch for more papers by this author, M.D., Ph.D., and Pierre KrystkowiakSearch for more papers by this author, M.D., Ph.D.Published Online:1 Oct 2011https://doi.org/10.1176/jnp.23.4.jnpe32AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: A recent multicenter study1 reported that the prevalence of impulse-control disorders (ICD) was 2.72-fold higher in Parkinson's disease (PD) patients treated by dopamine agonists than in PD patients not treated by these medications. Several case reports of attempted suicide or suicide in patients treated by dopamine agonists with pathological gambling have been published.2 A recent cross-sectional study3 on 128 PD patients examined associations between current suicidal and/or death ideation and neurological, neuropsychological, and psychiatric variables. Interestingly, a history of ICD disorders was not a statistically significant factor, but was associated with a trend (p<0.07). On multivariate logistic regression, major depression, psychosis, and elevated score on the Beck Hopelessness Scale were independent predictors of current suicidal and/or death ideation.Case ReportWe report the case of a 44-year-old man treated for juvenile idiopathic Parkinson's disease (PD) with pramipexole (0.7 mg three times daily) and l-dopa-benzeraside (62.5 mg three times daily) for 2 years. The dose of pramipexole was then increased to 0.7-mg tablets 4.5 per day. Several days later, he developed severe ICD with hypersexuality: increased libido, visiting of pornographic websites, and adultery. Six months later, pramipexole was decreased, then stopped, and l-dopa was continued alone. Hypersexuality rapidly resolved, but the patient experienced mixed anxiety/depressive disorder, and he was then admitted to the psychiatric department of a general hospital for 3 weeks and was treated with fluoxetine (20 mg/day). At the last psychiatric visit, 4 weeks after discharge from hospital, the patient's main complaint was related to his sexual activity; he said that he had sexual intercourse much less frequently than while he was taking pramipexole. Despite his marked anxiety, he did not present any symptoms of depression. The following day, he experienced a benign fall while jogging and then committed suicide by hanging from a tree, 2 months after withdrawal of pramipexole.DiscussionThis case report illustrates, for the first time, a potential link between suicide and ICD. Suicide due to depression was excluded by the psychiatric assessment on the day before the patient's death. When pramipexole was stopped, compulsive hypersexuality diminished dramatically and then resolved. The patient was then unable to accept his “normal” sexual activity and experienced a kind of pathological bereavement leading to hopelessness. Another hypothesis could be that the suicide was the result of impulsive behavior secondary to dopamine-agonist treatment, but the fall appeared to be the triggering event in a “hopelessness” patient. This potential cause can be excluded because L-dopa treatment was stable at low dose (187.5 mg/day). However, duration of the illness usually facilitates psychological acceptance and elaboration of the illness by the patient, as proposed by several authors using the term “work of disease” or “disease experience.” The patient's paradoxically increased physical or sexual capacities could considerably interfere with this “work of disease” and increase the risk of suicide. This case report suggests the need to identify patients at risk of suicidal behavior before administering dopamine agonists. The presence of impulse-control disorder and not only depression must therefore be investigated in PD patients who attempt suicide.University Department of NeurologyUniversity Department of PsychiatryUniversité de Picardie Amiens, FranceCorrespondence: Prof. Gwenolé Loas e-mail: Loas.[email protected]fr1. Weintraub D , Koester J , Potenza MN , et al.: Impulse-control disorders in Parkinson disease: a cross-sectional study of 3,090 patients. Arch Neurol 2010; 67:589–595Crossref, Medline, Google Scholar2. Kolla BN , Mansukhani MP , Barraza R , et al.: Impact of dopamine agonists on compulsive behaviors: a case series of pramipexole-induced pathological gambling. Psychosomatics 2010; 51:271–273Crossref, Medline, Google Scholar3. Kostic V , Pekmezovic T , Tomic A , et al.: Suicide and suicidal ideation in Parkinson's disease. J Neurol Sci 2010; 289:40–43Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByDopamine Agonist Withdrawal Syndrome and Suicidality in Parkinson’s Disease8 July 2022 | Canadian Journal of Neurological Sciences / Journal Canadien des Sciences NeurologiquesAnhedonia and Suicide19 April 2022Hospital care for mental health and substance abuse conditions in Parkinson's disease9 December 2016 | Movement Disorders, Vol. 31, No. 12Subthalamic Nucleus Versus Globus Pallidus Internus Deep Brain Stimulation: Translating the Rematch Into Clinical Practice10 April 2014 | Movement Disorders Clinical Practice, Vol. 1, No. 1 Volume 23Issue 4 Fall 2011Pages E32-E32 Metrics PDF download History Published online 1 October 2011 Published in print 1 October 2011

Bilateral Deep Brain Stimulation for Cervical Dystonia: Long-term Outcome in a Series of 10 Patients

Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) was shown to be effective in cervical dystonia refractory to medical treatment in several small short-term and 1 long-term follow-up series. Optimal stimulation parameters and their repercussions on the cost/benefit ratio still need to be established. To report our long-term outcome with bilateral GPi deep brain stimulation in cervical dystonia. The Toronto Western Spasmodic Torticollis Rating Scale was evaluated in 10 consecutive patients preoperatively and at last follow-up. The relationship of improvement in postural severity and pain was analyzed and stimulation parameters noted and compared with those in a similar series in the literature. The mean (standard deviation) follow-up was 37.6 (16.9) months. Improvement in the total Toronto Western Spasmodic Torticollis Rating Scale score as evaluated at latest follow-up was 68.1% (95% confidence interval: 51.5-84.6). In 4 patients, there was dissociation between posture severity and pain improvement. Prevalently bipolar stimulation settings and high pulse widths and amplitudes led to excellent results at the expense of battery life. Improvement in all 3 subscale scores of the Toronto Western Spasmodic Torticollis Rating Scale with bilateral GPi deep brain stimulation seems to be the rule. Refinement of stimulation parameters might have a significant impact on the cost/benefit ratio of the treatment. The dissociation of improvement in posture severity and pain provides tangible evidence of the complex nature of cervical dystonia and offers interesting insight into the complex functional organization of the GPi.

Comparison of Treatment Results between Selective Peripheral Denervation and Deep Brain Stimulation in Patients with Cervical Dystonia

Background/Aims: Cervical dystonia (CD) is the most common form of focal dystonia. The treatment options for CD include medical therapy and surgery. The 3 basic types of CD are tonic, phasic and tremulous. Selective peripheral denervation (SPD) and globus pallidus internus (GPi) deep brain stimulation (DBS) have been developed as therapeutic options for the treatment of CD. Methods: Between 1997 and 2009, 24 patients with CD underwent operations: either SPD (n = 16) or DBS (n = 8). The mean follow-up period was 29.5 months (range = 2–59). The mean age at onset of symptoms was 46.6 years (range = 27–65). The patients were evaluated with the subjective scores and the Toronto Western Spasmodic Torticollis Scale scores. Results: All patients showed gradual improvement after SPD and DBS. No statistically significant differences were seen between the SPD group and the DBS group. However, there was a trend toward greater pain reduction in the DBS group (p = 0.094). Conclusion: Both the SPD group and the DBS group showed successful improvement in their Toronto Western Spasmodic Torticollis Scale scores as well as subjective scores. The GPi DBS group showed a remarkable improvement on the pain scale and there was a trend toward greater pain reduction in the DBS group.

Early Globus Pallidus Internus Stimulation in Pediatric Patients With Generalized Primary Dystonia: Long-Term Efficacy and Safety

Primary generalized dystonia presents mainly at a young age and commonly is severely disabling. The authors report the long-term follow-up (mean, 73 months; range, 50-101 months) of 5 pediatric patients (mean age at surgery 13 years; range, 8-16 years) undergoing globus pallidus internus deep brain stimulation. Mean improvement in the Burke-Fahn-Marsden movement score was 67.4% (range, 47.0%-87.5%), 75.4% (range, 61.5%-91.7%), and 83.5% (range, 72.0%-93.3%) at 3 months, 12 months, and long-term follow-up (>36 months), respectively. Hardware problems (electrode dislocation/breakage of extension cable, and imminent perforation of extension cable) were observed in 2 patients (operative revision without sequelae). Except for mild dysarthria in 2 patients, no other therapy-related morbidity was observed. The authors found globus pallidus internus stimulation to offer a very effective and safe therapy in pediatric patients with primary dystonia. Early neurosurgical intervention seems to be crucial to prevent irreversible impairment of motor function.

Long‐term results of a multicenter study on subthalamic and pallidal stimulation in Parkinson's disease

We report the 5 to 6 year follow-up of a multicenter study of bilateral subthalamic nucleus (STN) and globus pallidus internus (GPi) deep brain stimulation (DBS) in advanced Parkinson's disease (PD) patients. Thirty-five STN patients and 16 GPi patients were assessed at 5 to 6 years after DBS surgery. Primary outcome measure was the stimulation effect on the motor Unified Parkinson's Disease Rating Scale (UPDRS) assessed with a prospective cross-over double-blind assessment without medications (stimulation was randomly switched on or off). Secondary outcomes were motor UPDRS changes with unblinded assessments in off- and on-medication states with and without stimulation, activities of daily living (ADL), anti-PD medications, and dyskinesias. In double-blind assessment, both STN and GPi DBS were significantly effective in improving the motor UPDRS scores (STN, P < 0.0001, 45.4%; GPi, P = 0.008, 20.0%) compared with off-stimulation, regardless of the sequence of stimulation. In open assessment, both STN- and GPi-DBS significantly improved the off-medication motor UPDRS when compared with before surgery (STN, P < 0.001, 50.5%; GPi, P = 0.002, 35.6%). Dyskinesias and ADL were significantly improved in both groups. Anti-PD medications were significantly reduced only in the STN group. Adverse events were more frequent in the STN group. These results confirm the long-term efficacy of STN and GPi DBS in advanced PD. Although the surgical targets were not randomized, there was a trend to a better outcome of motor signs in the STN-DBS patients and fewer adverse events in the GPi-DBS group.

Outcome of Pallidal Deep Brain Stimulation in Meige Syndrome

Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with medically refractory Meige syndrome. Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was 23.1 ± 6.4 months. The mean age at time of surgery was 58.0 ± 7.8 years. The mean duration of symptoms was 8.7 ± 7.6 years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After 2.4 ± 1.3 days of trial tests, the stimulation device was implanted under general anesthesia. Patients were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). BFMDRS total movement scores improved by 59.8%, 63.5%, 74.1%, 74.5%, and 85.5% during the immediate postoperative period of test stimulation, 3, 6, 12, and 24 months (n = 5) after surgery, respectively. The BFMDRS total movement scores were reduced gradually and the results reached statistical significance in the postoperative period (test period, p < 0.001; 3 months, p < 0.001; 6 months, p = 0.003; 12 months, p < 0.001; 24 months, p = 0.042). There was no statistical difference between 12 months and 24 months. BFM subscores improved by 63.3% for the eyes, 80.9% for the mouth, 68.4% for speech/swallowing, and 87.9% for the neck at 12 months after surgery. The adverse effects were insignificant. The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.

P3.192 Effect of bilateral globus pallidus internus deep brain stimulation on periodic limb movements in Parkinson's disease patient and possible mechanism

P3.192 Effect of bilateral globus pallidus internus deep brain stimulation on periodic limb movements in Parkinson's disease patient and possible mechanism

An evaluation of rating scales utilized for deep brain stimulation for dystonia

The objective of this study was to examine globus pallidus internus deep brain stimulation (GPi-DBS) outcomes in primary and secondary dystonia, derived from blinded ratings using two scales and two raters. Twenty-five patients with variable presentations of dystonia were evaluated with videotaped standardized dystonia rating scales at preoperative baseline and at 6 and 12 months following GPi-DBS implantation. These 75 examination videos were retrospectively evaluated, independently and in random order, by two movement disorder neurologists who were blinded to the treatment status. Both neurologists scored each videotaped evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-motor part (BFMDRS-M) and the Unified Dystonia Rating Scale (UDRS). A final score for each video was assigned by averaging the raters' scores. An intra-class correlation coefficient was used to calculate inter-rater reliability. A linear mixed model was fitted to investigate the time effect and its interaction with type of dystonia (primary versus secondary) for each rating scale. Inter-rater reliability was excellent. Intraclass correlation coefficients ranged from 0.994 to 0.997 for both scales at baseline, 6 and 12 months. The average motor improvement scores after GPi DBS for the entire heterogeneous group of dystonia patients after 6 and 12 months of stimulation was 21.32% (p = 0.0010) and 28.95% (p = 0.0017), respectively, when the UDRS score was used. Similar levels of improvement 20.46% (p = 0.0055) at 6 months and 27.39% (p = 0.00197) at 12 months were found using the BFMDRS-M score. Analysis using unblinded scores from our database revealed a 32.99 and 37.27% UDRS improvement at 6 and 12 months, and an improvement in UDRS score of 38.5 and 43.7% when the analysis was limited to only primary dystonia. If the data were further segregated to include only cases of DYT-1 primary generalized dystonia, the UDRS benefit increased to 48.24%. Our primary dystonia group was diluted by the presence of both old- and young-onset patients, as well as focal, segmental and generalized dystonia. In conclusion, (1) evaluating motor outcomes of DBS therapy for dystonia using independent, randomized retrospective rating by blinded raters' results in lower improvement scores than when outcomes are rated by unblinded treating neurologists. Blinded methodology may be superior and might produce a more realistic assessment of motor outcomes after DBS in patients with dystonia; (2) outcomes were similar whether the BFMDRS-M or UDRS was utilized; (3) GPi-DBS was effective in treating sustained involuntary motor co-contractions in medication refractory dystonia patients, more so in primary dystonia.

Cognition and mood in Parkinson's disease in subthalamic nucleus versus globus pallidus interna deep brain stimulation: The COMPARE Trial

Our aim was to compare in a prospective blinded study the cognitive and mood effects of subthalamic nucleus (STN) vs. globus pallidus interna (GPi) deep brain stimulation (DBS) in Parkinson disease. Fifty-two subjects were randomized to unilateral STN or GPi DBS. The co-primary outcome measures were the Visual Analog Mood Scale, and verbal fluency (semantic and letter) at 7 months post-DBS in the optimal setting compared to pre-DBS. At 7 months post-DBS, subjects were tested in four randomized/counterbalanced conditions (optimal, ventral, dorsal, and off DBS). Forty-five subjects (23 GPi, 22 STN) completed the protocol. The study revealed no difference between STN and GPi DBS in the change of co-primary mood and cognitive outcomes pre- to post-DBS in the optimal setting (Hotelling's T(2) test: p = 0.16 and 0.08 respectively). Subjects in both targets were less "happy", less "energetic" and more "confused" when stimulated ventrally. Comparison of the other 3 DBS conditions to pre-DBS showed a larger deterioration of letter verbal fluency in STN, especially when off DBS. There was no difference in UPDRS motor improvement between targets. There were no significant differences in the co-primary outcome measures (mood and cognition) between STN and GPi in the optimal DBS state. Adverse mood effects occurred ventrally in both targets. A worsening of letter verbal fluency was seen in STN. The persistence of deterioration in verbal fluency in the off STN DBS state was suggestive of a surgical rather than a stimulation-induced effect. Similar motor improvement were observed with both STN and GPi DBS.

Factors predicting improvement in primary generalized dystonia treated by pallidal deep brain stimulation

Despite the beneficial effects of Globus Pallidus internus (GPi) deep brain stimulation (DBS) in patients with primary generalized dystonia (PGD), the degree of improvement varies from one patient to another. The objective of this study was to examine the effects of clinical, anatomical (volume of the GPi), and electrical variables on the postoperative Burke-Fahn-Marsden Dystonia rating scale (BFMDRS) motor score to identify which factors may be predictive of the degree of improvement. We reviewed retrospectively the clinical records of 40 steady-state patients with PGD who had been treated by bilateral GPi lead implantation. The follow-up period was 2 to 8 years. The correlation between the electrical parameters (voltage, impedance, and current) and the clinical outcome was studied. An analysis of covariance was performed to identify factors predictive of the magnitude of improvement. The most influential factors according to the model are as follows: the preoperative BFMDRS score (P < 0.0001); age at surgery (P < 0.0001); the right GPi volume (P = 0.002); the left stimulated GPi volume (P = 0.005). No significant correlation was found between the electrical parameters used and the mean motor scores in steady state.

Pallidal Deep Brain Stimulation in Primary Cervical Dystonia with Phasic Type : Clinical Outcome and Postoperative Course

The purpose of this study was to analyze in detail the relationship between outcome and time course of effect in medically refractory primary cervical dystonia (CD) with phasic type that was treated by bilateral globus pallidus internus (Gpi) deep brain stimulation (DBS). Six patients underwent bilateral implantation of DBS into the Gpi under the guide of microelectrode recording and were followed for 18.7 +/- 11.1 months. The mean duration of the CD was 5.8 +/- 3.4 years. The mean age at time of surgery was 54.2 +/- 10.2 years. Patients were evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and relief scale using patient self-reporting. The TWSTRS total scores improved by 64.5%, 65.5%, 75.8%, and 76.0% at 3, 6, 12 months, and at the last available follow-up after surgery, respectively. Statistically significant improvements in the TWSTRS scores were observed 3 months after surgery (p = 0.028) with gradual improvement up to 12 months after surgery, thereafter, the improvement was sustained. However, there was no statistically significant difference between the scores at 3 and 12 months. Subjective improvement reported averaged 81.7 +/- 6.8% at last follow-up. Mild dysarthria, the most frequent adverse event, occurred in 3 patients. Our results show that the bilateral Gpi-DBS can offer a significant therapeutic effect from 3 months postoperatively in patients with primary CD with phasic type, without significant side effects.

Bilateral deep brain stimulation of the globus pallidus internus in tardive dystonia

Tardive dystonia is a disabling movement disorder as a consequence of exposure to neuroleptic drugs. We followed 6 patients with medically refractory tardive dystonia treated by bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) for 21 +/- 18 months. At last follow-up, the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor score improved by 86% +/- 14%, and the BFMDRS disability score improved by 80% +/- 12%. Bilateral GPi-DBS is a beneficial therapeutic option for the long-term relief of tardive dystonia.

Deep brain stimulation for parkinsonian gait disorders

The cardinal motor manifestation of Parkinson's disease (PD) is being treated with greater and greater efficacy with both newer medications as well as both subthalamic nucleus (STN) and globus pallidus internus (GPI) deep brain stimulation (DBS). The burden of disease is shifting towards the non-dopaminergic disease manifestations including gait and posture. Based on evidence in the literature and in animal models, recent trials are underway to examine the effects of pedunculopontine nucleus DBS on the treatment of parkinsonian gait disorder. We review the rationale behind this treatment and the status of the current trials.

Stereotactic MRI in Dyt1 Dystonia: Focal Signal Abnormalities in the Basal Ganglia Do Not Contraindicate Deep Brain Stimulation

Aims: To study stereotactic magnetic resonance imaging (MRI) features of the basal ganglia in DYT1 primary dystonia. Methods: Twenty-five genetically confirmed DYT1 dystonia patients (age range, 8–66 years; mean age, 22 years) underwent brain MRI under general anesthesia at the time of globus pallidus internus (GPi) deep brain stimulation (DBS) surgery. MR images were retrospectively reviewed for signal intensity alterations. Clinical improvement of patients was assessed by comparing pre- and postoperative Burke-Fahn-Marsden Dystonia Rating Scale scores. Results: Seventeen patients out of 25 (68%) exhibited T₁-weighted hypointense/T₂-weighted hyperintense signal abnormalities in the putamen and globus pallidus on MR images. Signals were isointense with cerebrospinal fluid in all sequences. The mean volume of focal signal abnormalities was 15 mm³ (maximum, 154.5 mm³). The total volume of focal signal abnormalities in the basal ganglia was correlated with the duration of the disease (p = 0.01). Although clinical outcome did not differ as a function of the presence of focal signal abnormalities overall, patients with signals within the GPi tended to show lesser improvement (p = 0.04). Conclusions: T₁-hypointense/T₂-hyperintense signal abnormalities are common findings in the putamen and globus pallidus of DYT1 patients but do not contraindicate DBS. However, their presence within the GPi may reduce the efficacy of DBS treatment.

LOCATION OF ACTIVE CONTACTS IN PATIENTS WITH PRIMARY DYSTONIA TREATED WITH GLOBUS PALLIDUS DEEP BRAIN STIMULATION

Deep brain stimulation of the globus pallidus internus has been used for the treatment of various forms of dystonia, but the factors influencing postoperative outcomes remain unknown. We compared the location of the contacts being used for stimulation (active contacts) in patients with cervical dystonia, generalized dystonia, and Parkinson's disease and correlated the results with clinical outcome. Postoperative magnetic resonance scans of 13 patients with cervical dystonia, six patients with generalized dystonia, and five patients with Parkinson's disease who underwent globus pallidus internus deep brain stimulation were analyzed. We assessed the location of the active contacts relative to the midcommisural point and in relation to the anteroposterior and mediolateral boundaries of the pallidum. Postoperative outcome was measured with the Toronto Western Spasmodic Torticollis Rating Scale (for cervical dystonia) and the Burke-Fahn-Marsden Dystonia Rating Scale (for generalized dystonia) during the last follow-up. We found that the location of the active contacts relative to the midcommisural point and the internal boundaries of the pallidum was similar across the groups. In our series, the contacts used for stimulation were clustered in the posterolateral region of the pallidum. Within that region, we found no correlation between the location of the contacts and postoperative outcome. The location of the active contacts used for globus pallidus internus deep brain stimulation was similar in patients with cervical dystonia, generalized dystonia, and Parkinson's disease.

Exacerbation of blepharospasm associated with craniocervical dystonia after placement of bilateral globus pallidus internus deep brain stimulator

To report a case of exacerbation of blepharospasm after bilateral globus pallidus internus (GPi) deep brain stimulator (DBS) placement. A 69-year-old male presented after bilateral GPi DBS placement for blepharospasm and craniocervical dystonia with worsening eyelid spasms and associated apraxia of lid opening (ALO). Numerous attempts to adjust DBS parameters were ineffective. Consequently, bilateral upper eyelid myectomy was performed. Myectomy surgery was free of complications. The patient had significant improvement of blepharospasm and ALO. Although early success has been reported with DBS placement in a small number of patients with focal dystonias, further studies and longer follow-up are needed to demonstrate whether this will prove to be a useful approach in the treatment of blepharospasm. Upper eyelid myectomy can provide an effective means for treating blepharospasm and associated ALO.