Deep Brain Stimulation in Adult Huntington Disease for Treatment of Axial Dystonia

2020 ◽  
pp. 211-216
Author(s):  
Jessica A. Karl ◽  
Kathleen Shannon ◽  
Konstantin Slavin ◽  
Leo Verhagen Metman
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Huntington Disease ◽  
Neurodegenerative Disorder ◽  
Case Reports ◽  
Common Symptom ◽  
Psychiatric Disturbances ◽  
Optimal Medical Management ◽  
Recent Case Study ◽  
Deep Brain

Huntington disease (HD) is a progressive neurodegenerative disorder characterized by chorea and cognitive and psychiatric disturbances. Dystonia is also a common symptom, and a recent case study points toward 50% of HD patients having truncal dystonia. Although tetrabenazine is approved for treatment of Huntington chorea, no medications have proved efficacious for the dystonia and bradykinesia that become more prominent as the disease progresses. Although several reports have outlined the effect of globus pallidus interna (GPi) deep brain stimulation (DBS) in HD patients with a choreic phenotype, less is known about the effect of DBS in HD patients with a predominately dystonic phenotype. When specific symptoms known to respond well to DBS, such as dystonia, interfere significantly with a patient’s quality of life, and optimal medical management has failed, DBS could be considered a viable option. This case reports demonstrates the utility of bilateral GPi DBS for dystonia in a patient with HD, so long as there is no neuropsychiatric impairment that would interfere with the patient’s comprehension of the potential risks of surgery. The patient and caregivers should have realistic expectations of outcomes and be willing to follow through with regular programming.

Visual Effects of Deep Brain Stimulation in a Patient with Parkinson’s Disease

2019 ◽  
pp. 158-173
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Deep Brain Stimulation ◽  
Visual System ◽  
Brain Stimulation ◽  
Neurodegenerative Disorder ◽  
Initial Examination ◽  
Before And After ◽  
Deep Brain ◽  
Cover Test

Background: Parkinson’s disease (PD) is a progressive neurodegenerative disorder caused by a dopamine deficiency that presents with motor symptoms. Visual disorders can occur concomitantly but are frequently overlooked. Deep brain stimulation (DBS) has been an effective treatment to improve tremors, stiffness and overall mobility, but little is known about its effects on the visual system. Case Report: A 75-year-old Caucasian male with PD presented with longstanding binocular diplopia. On baseline examination, the best-corrected visual acuity was 20/25 in each eye. On observation, he had noticeable tremors with an unsteady gait. Distance alternating cover test showed exophoria with a right hyperphoria. Near alternating cover test revealed a significantly larger exophoria accompanied by a reduced near point of convergence. Additional testing with a 24-2 Humphrey visual field and optical coherence tomography (OCT) of the nerve and macula were unremarkable. The patient underwent DBS implantation five weeks after initial examination, and the device was activated four weeks thereafter. At follow up, the patient still complained of intermittent diplopia. There was no significant change in the manifest refraction or prism correction. On observation, the patient had remarkably improved tremors with a steady gait. All parameters measured were unchanged. The patient was evaluated again seven months after device activation. Although vergence ranges at all distances were improved, the patient was still symptomatic for intermittent diplopia. OCT scans of the optic nerve showed borderline but symmetric thinning in each eye. All other parameters measured were unchanged. Conclusion: The case found no significant changes on ophthalmic examination after DBS implantation and activation in a patient with PD. To the best of the authors’ knowledge, there are no other cases in the literature that investigated the effects of DBS on the visual system pathway in a patient with PD before and after DBS implantation and activation.

Database of MNI stereotactic coordinates for deep brain stimulation targets in neuropsychiatric disorders

2011 ◽  
Vol 26 (S2) ◽  
pp. 1149-1149
Author(s):  
U. Moser ◽  
M. Savli ◽  
R. Lanzenberger ◽  
S. Kasper
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Functional Neuroimaging ◽  
Case Reports ◽  
Three Dimensional ◽  
Compulsive Disorder ◽  
Neuroimaging Data ◽  
Specific Disorders ◽  
Image Position ◽  
Deep Brain

IntroductionDeep brain stimulation (DBS) is a promising therapy option for otherwise treatment-resistant neuropsychiatrie disorders, especially in obsessive-compulsive disorder (OCD), major depression (TRD) and Tourette's Syndrome (TS).ObjectiveThe brain coordinates of the DBS targets are mainly reported using measurements in original, unnormalized brains. In the neuroimaging community stereotactic data are mainly indicated in the standardized Montreal Neurological Institute (MNI) space, i.e. a three-dimensional proportional grid system.AimsImproved comparability between targets in DBS studies and molecular and functional neuroimaging data from PET, SPECT, MRI, fMRI, mostly published with stereotactic data.MethodsA comprehensive and systematic literature search for published DBS case reports or studies in TRD, OCD and TS was performed. We extracted the tip positions of electrode leads as provided in the publications or by the authors, and transferred individual coordinates to the standard brain in the MNI space.Results46 publications fulfilled the inclusion criteria. The main targets for the specific disorders and one or two examples of their calculated MNI coordinates are indicated in the table:[MNI coordinates of the main DBS targets]ConclusionsWe provide DBS data of neuropsychiatrie disorders in the MNI space, improving the comparability to molecular, functional and structural neuroimaging data.

Long-term follow-up of Huntington disease treated by bilateral deep brain stimulation of the internal globus pallidus

2008 ◽  
Vol 109 (1) ◽  
pp. 130-132 ◽  
Author(s):  
Brigitte Biolsi ◽  
Laura Cif ◽  
Hassan El Fertit ◽  
Santiago Gil Robles ◽  
Philippe Coubes
Keyword(s):  
Deep Brain Stimulation ◽  
Globus Pallidus ◽  
Brain Stimulation ◽  
Movement Disorders ◽  
Huntington Disease ◽  
Internal Globus Pallidus ◽  
Efficient Treatment ◽  
Deep Brain ◽  
Stimulation Of

Deep brain stimulation is now accepted as a safe and efficient treatment for movement disorders including selected types of dystonia and dyskinesia. Very little, however, is known about its effect on other movement disorders, particularly for “choreic” movements. Huntington disease is a fatal autosomal-dominant neurodegenerative disorder characterized by movement disorders, progressive cognitive impairment, and psychiatric symptoms. Bilateral chronic stimulation of the internal globus pallidus was performed to control choreic movements in a 60-year-old man with a 10-year history of Huntington disease. Chronic deep brain stimulation resulted in remarkable improvement of choreic movements. Postoperative improvement was sustained after 4 years of follow-up with a marked improvement in daily quality of life.

scholarly journals A Case of Previously Unsuspected Huntington Disease Diagnosed at Autopsy

2017 ◽  
Vol 7 (1) ◽  
pp. 136-144
Author(s):  
Catherine R. Miller ◽  
Nobby C. Mambo ◽  
Jianli Dong ◽  
Gerald A. Campbell
Keyword(s):  
Genetic Testing ◽  
Huntington Disease ◽  
Clinical Symptoms ◽  
Neurodegenerative Disorder ◽  
Case Reports ◽  
Neuronal Loss ◽  
Cag Repeat ◽  
Cag Repeats ◽  
Psychiatric Disturbances ◽  
Personality Changes

Huntington disease (HD) is a neurodegenerative disorder with a worldwide prevalence of four to ten per 100 000. It is characterized by choreiform movements, behavioral/psychiatric disturbances, and eventual cognitive decline. Symptoms usually present between 30 and 50 years of age and the diagnosis is based on the combination of clinical symptoms, family history, and genetic testing. A variation of HD, juvenile Huntington disease (JHD), presents earlier, with more severe symptoms and with a worse prognosis. Symptoms are different in JHD, with personality changes and learning difficulties being the predominant presenting features. Seizures are common in JHD, and chorea is uncommon; movement disorders at presentation of JHD are predominantly nonchoreiform. The inheritance pattern for both HD and JHD is autosomal dominant and the disease is caused by an elongation of the CAG repeat in the huntingtin gene. There are many published case reports of Huntington disease that were confirmed at autopsy, but to our knowledge, there are no reports in the literature where the diagnosis of Huntington disease was first made at autopsy. We present a case of a 28-year-old African-American male who was in a state of neglect due to a lifetime of abuse, cognitive difficulties, and seizures, whose cause of death was pneumonia. The gross autopsy findings included bilateral caudate nucleus atrophy and lateral ventricular dilation. Microscopically, severe bilateral neuronal loss and gliosis of the caudate and putamen nuclei were seen. Genetic testing for the number of CAG repeats confirmed the diagnosis and was consistent with JHD.

scholarly journals Impulse Control Disorders Following Deep Brain Stimulation of the Subthalamic Nucleus in Parkinson's Disease: Clinical Aspects

2011 ◽  
Vol 2011 ◽  
pp. 1-9 ◽  
Author(s):  
Polyvios Demetriades ◽  
Hugh Rickards ◽  
Andrea Eugenio Cavanna
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Impulse Control ◽  
Case Reports ◽  
Impulse Control Disorders ◽  
Clinical Aspects ◽  
Dopaminergic Medication ◽  
Deep Brain

Parkinson's disease (PD) has been associated with the development of impulse control disorders (ICDs), possibly due to overstimulation of the mesolimbic system by dopaminergic medication. Preliminary reports have suggested that deep brain stimulation (DBS), a neurosurgical procedure offered to patients with treatment-resistant PD, affects ICD in a twofold way. Firstly, DBS allows a decrease in dopaminergic medication and hence causes an improvement in ICDs. Secondly, some studies have proposed that specific ICDs may develop after DBS. This paper addresses the effects of DBS on ICDs in patients with PD. A literature search identified four original studies examining a total of 182 patients for ICDs and nine case reports of 39 patients that underwent DBS and developed ICDs at some point. Data analysis from the original studies did not identify a significant difference in ICDs between patients receiving dopaminergic medication and patients on DBS, whilst the case reports showed that 56% of patients undergoing DBS had poor outcome with regards to ICDs. We discuss these ambivalent findings in the light of proposed pathogenetic mechanisms. Longitudinal, prospective studies with larger number of patients are required in order to fully understand the role of DBS on ICDs in patients with PD.

scholarly journals Neuropsychiatric Outcome of an Adolescent Who Received Deep Brain Stimulation for Tourette's Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
S. J. Pullen ◽  
C. A. Wall ◽  
K. H. Lee ◽  
S. M. Stead ◽  
B. T. Klassen ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Adaptive Functioning ◽  
Case Reports ◽  
Tourette's Syndrome ◽  
Adolescent Male ◽  
Tourette’S Syndrome ◽  
Patient Reported ◽  
Deep Brain ◽  
Neuropsychiatric Outcome

This case study followed one adolescent patient who underwent bilateral deep brain stimulation of the centromedian parafascicular complex (CM-Pf) for debilitating, treatment refractory Tourette's syndrome for a period of 1.5 years. Neurocognitive testing showed no significant changes between baseline and follow-up assessments. Psychiatric assessment revealed positive outcomes in overall adaptive functioning and reduction in psychotropic medication load in this patient. Furthermore, despite significant baseline psychiatric comorbidity, this patient reported no suicidal ideation following electrode implantation. Deep brain stimulation is increasingly being used in children and adolescents. This case reports on the positive neurologic and neuropsychiatric outcome of an adolescent male with bilateral CM-Pf stimulation.

scholarly journals A Systematic Review of the Biological Processes Involved in Deep-Brain Stimulation for Parkinson's disease: A Focus on the Potential Disease-Modifying Effects

2021 ◽  
Vol 05 (02) ◽  
pp. 1-1
Author(s):  
Francesco Sciancalepore ◽  
◽  
Giulia Remoli ◽  
Leonardo Tariciotti ◽  
Giulia Sarti ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Early Stage ◽  
Case Reports ◽  
Biological Processes ◽  
Inclusion Criteria ◽  
Disease Modifying ◽  
Deep Brain

Deep-Brain Stimulation (DBS) is an important treatment option for the management of Parkinson’s disease (PD) and is a common symptomatic treatment. However, an increasing number of studies have examined the biological processes to assess if DBS can also modify the natural history of PD by acting on its pathophysiological mechanisms. Relevant literature published up to November 2020 was systematically searched on databases such as PubMed, ISI Web of Knowledge, Academic Search Index, and Science Citation Index. The following predefined inclusion criteria were applied to the full-text versions of the selected articles: i) recruiting and monitoring of PD subjects that were previously treated with DBS and ii) investigating the electrophysiological, biochemical, epigenetic, or neuroimaging effects of DBS. Studies focusing exclusively on motor and clinical changes were excluded. Reviews, case reports, studies on animal models, and computational studies were also not considered. Out of 2,960 records screened, 43 studies met the inclusion criteria. Only three studies described a potential disease-modifying effect of DBS. However, a wide heterogeneity was observed in the investigated biomarkers, and the design and methodological issues of several studies limited their ability to find potential disease-modifying features. Specifically, 60.4% of the trials followed-up subjects for no more than 1 year from the surgical intervention, and 67.4% observed patients with PD only once after DBS. Moreover, 64.2% of the studies enrolled late-stage PD patients. Most of the studies (88.4%) reported that DBS only had a symptomatic effect, with several of them showing some limitations in the study design and recruitment of patients. Further studies using shared biomarkers are encouraged to assess if and how DBS might affect the progression of PD. Based on the existing preclinical literature, prospective clinical trials examining the course of PD in early-stage patients are needed.

scholarly journals Pacemaker and Defibrillator Implantation and Programming in Patients with Deep Brain Stimulation

2019 ◽  
Vol 8 (2) ◽  
pp. 138-142 ◽  
Author(s):  
Mark Elliott ◽  
Sheikh Momin ◽  
Barnaby Fiddes ◽  
Fahad Farooqi ◽  
SM Afzal Sohaib
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Case Reports ◽  
Cardiac Pacemaker ◽  
Cardiac Device ◽  
Deep Brain Stimulator ◽  
Device Implantation ◽  
Cardiac Devices ◽  
Potential Interactions ◽  
Deep Brain

The need for cardiac device implantation in patients receiving deep brain stimulation (DBS) is increasing. Despite the theoretical risk of the two systems interacting, there are no clear guidelines for cardiologists carrying out cardiac device implantation in this population. We performed a review of the literature and describe 13 case reports in which patients have both DBS and a cardiac pacemaker or ICD implanted. Except for one early study, in which an ICD shock reset the deep brain stimulator, no significant interactions have been reported. We discuss the potential interactions between DBS and cardiac devices, and provide practical advice for implanting cardiologists. We conclude that, provided that specific precautions are taken, cardiac device implantation is likely to be safe in patients with DBS.

scholarly journals Deep brain stimulation for orthostatic tremor

2019 ◽  
Vol 10 (4) ◽  
pp. 324-332 ◽  
Author(s):  
Angela L. Hewitt ◽  
Bryan T. Klassen ◽  
Kendall H. Lee ◽  
Jamie J. Van Gompel ◽  
Anhar Hassan
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Case Reports ◽  
Weight Bearing ◽  
Clinical Electrophysiology ◽  
Onset Latency ◽  
Meaningful Improvement ◽  
Standing Time ◽  
Orthostatic Tremor ◽  
Deep Brain

BackgroundOrthostatic tremor (OT) is a high-frequency weight-bearing tremor of the legs and trunk associated with progressive disability and is often refractory to medications. Case reports suggest that thalamic deep brain stimulation (DBS) is effective. We report 5 female patients with medication-refractory OT who underwent bilateral thalamic DBS at the Mayo Clinic and assess factors associated with a successful DBS outcome.MethodsDemographic, clinical, electrophysiology, and DBS data were abstracted. Outcomes were change in tremor-onset latency, standing time, standing ADLs, and patient and clinician global impression of change (PGIC; CGIC).ResultsAll 5 patients had improved standing time (72 vs 408 seconds, p ≤ 0.001) and improved standing ADLs after surgery, without change in tremor-onset latency (16 vs 75 seconds, p = 0.14). Maximal benefit was reached up to 3 years after surgery and sustained for up to 6 years. CGIC was “much improved” in all; PGIC was “much improved” in 4 and “minimally improved” in 1. There were no major complications. Postoperative electrophysiology (n = 1) showed lower tremor amplitude and slower tremor ramp-up on vs off stimulation.ConclusionsBilateral thalamic DBS improved OT symptoms with benefit lasting up to 6 years. A modest increase in standing time of several minutes was associated with meaningful improvement in standing ADLs. Microlesional effect and bilateral stimulation are likely favorable features, while baseline standing time of several minutes may be unfavorable. These findings may inform clinician and patient counseling and require confirmation in larger studies.

Deep Brain Stimulation Generator Failure due to External Defibrillation in a Patient with Essential Tremor

2020 ◽  
pp. 1-2
Author(s):  
Gregory Davis ◽  
Zachary Levine
Keyword(s):  
Cardiac Arrest ◽  
Deep Brain Stimulation ◽  
Essential Tremor ◽  
Brain Stimulation ◽  
Case Reports ◽  
The Other ◽  
Deep Brain Stimulator ◽  
Rf Transmission ◽  
Deep Brain

There exist only two case reports to date of open cardiac defibrillation with deep brain stimulator system (DBS) implantation. We report a 64-year-old male with DBS system in place for essential tremor who underwent cardiac defibrillation after cardiac arrest. Afterwards, his device impedances were all high and his tremor symptoms returned. Both problems resolved with implantation of a new generator and required no changes to the intracranial leads or extension cables. This is significantly different from the two previous reports. One included a significantly different DBS system relying on transcutaneous RF transmission and reported a lesioning effect after cardioversion. The other utilized a modern DBS system but reported damage to the generator and intracranial leads. We report that only the generator sustained damage, and that there were no intracranial changes that occurred.

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