scholarly journals Morphological Abnormalities in the Basal Ganglia of Dystonia Patients

2021 ◽  
pp. 1-12
Author(s):  
Xi Bai ◽  
Peter Vajkoczy ◽  
Katharina Faust
Keyword(s):  
Basal Ganglia ◽  
Globus Pallidus ◽  
Trajectory Planning ◽  
Matched Control ◽  
Young Patients ◽  
Control Population ◽  
Pathogenic Role ◽  
Target Region ◽  
Morphological Abnormalities ◽  
Primary Dystonia

<b><i>Objective:</i></b> The pathophysiology of dystonia is poorly understood. As opposed to secondary forms of dystonia, primary dystonia has long been believed to lack any neuroanatomical substrate. During trajectory planning for DBS, however, conspicuous T2-hyperinstensive signal alterations (SA) were registered within the target region, even in young patients, where ischemia is rare. <b><i>Methods:</i></b> Fifty MRIs of primary dystonia patients scheduled for DBS were analyzed. Total basal ganglia (BG) volumes, as well as proportionate SA volumes, were measured and compared to 50 age-matched control patients. <b><i>Results:</i></b> There was a 10-fold preponderance of percentaged SA within the globus pallidus (GP) in dystonia patients. The greatest disparity was in young patients &#x3c;25 years. Also, total BG volume differences were observed with larger GP and markedly smaller putamen and caudate in the dystonia group. <b><i>Conclusions:</i></b> BG morphology in primary dystonia differed from a control population. Volume reductions of the putamen and caudate may reflect functional degeneration, while volume increases of the GP may indicate overactivity. T2-hyperintensive SA in the GP of young primary dystonia patients, where microvascular lesions are highly unlikely, are striking. Their pathogenic role remains unclear.

scholarly journals The Subthalamic Nucleus in Primary Dystonia: Single-Unit Discharge Characteristics

2009 ◽  
Vol 102 (6) ◽  
pp. 3740-3752 ◽  
Author(s):  
Lauren E. Schrock ◽  
Jill L. Ostrem ◽  
Robert S. Turner ◽  
Shoichi A. Shimamoto ◽  
Philip A. Starr
Keyword(s):  
Basal Ganglia ◽  
Globus Pallidus ◽  
Subthalamic Nucleus ◽  
Single Unit ◽  
Discharge Rate ◽  
Receptive Fields ◽  
Principal Component ◽  
Oscillatory Activity ◽  
Inhibitory Input ◽  
Primary Dystonia

Most models of dystonia pathophysiology predict alterations of activity in the basal ganglia thalamocortical motor circuit. The globus pallidus interna (GPi) shows bursting and oscillatory neuronal discharge in both human dystonia and in animal models, but it is not clear which intrinsic basal ganglia pathways are implicated in this abnormal output. The subthalamic nucleus (STN) receives prominent excitatory input directly from cortical areas implicated in dystonia pathogenesis and inhibitory input from the external globus pallidus. The goal of this study was to elucidate the role of the STN in dystonia by analyzing STN neuronal discharge in patients with idiopathic dystonia. Data were collected in awake patients undergoing microelectrode recording for implantation of STN deep brain stimulation electrodes. We recorded 62 STN neurons in 9 patients with primary dystonia. As a comparison group, we recorded 143 STN neurons in 20 patients with Parkinson's disease (PD). Single-unit activity was discriminated off-line by principal component analysis and evaluated with respect to discharge rate, bursting, and oscillatory activity. The mean STN discharge rate in dystonia patients was 26.3 Hz (SD 13.6), which was lower than that in the PD patients (35.6 Hz, SD 15.2), but higher than published values for subjects without basal ganglia dysfunction. Oscillatory activity was found in both disorders, with a higher proportion of units oscillating in the beta range in PD. Bursting discharge was a prominent feature of both dystonia and PD, whereas sensory receptive fields were expanded in PD compared with dystonia. The STN firing characteristics, in conjunction with those previously published for GPi, suggest that bursting and oscillatory discharge in basal ganglia output may be transmitted via pathways involving the STN and provide a pathophysiologic rationale for STN as a surgical target in dystonia.

Germ cell tumors in the basal ganglia: problems of early diagnosis and treatment

2008 ◽  
Vol 2 (2) ◽  
pp. 118-124 ◽  
Author(s):  
Yukihiko Sonoda ◽  
Toshihiro Kumabe ◽  
Shin-Ichiro Sugiyama ◽  
Masayuki Kanamori ◽  
Yoji Yamashita ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Early Diagnosis ◽  
Germ Cell ◽  
Precocious Puberty ◽  
Germ Cell Tumors ◽  
Young Patients ◽  
Early Recurrence ◽  
Initial Therapy ◽  
Motor Weakness ◽  
Delay In Diagnosis

Object Intracranial germ cell tumors (GCTs) originating in the basal ganglia are rare. The authors investigated factors related to the diagnosis of these lesions as well as outcome in order to help decrease the time to diagnosis and improve treatment efficacy. Methods The authors reviewed the clinical features of 142 cases of intracranial GCT in their institute. Fourteen cases of basal ganglia GCT were identified. The symptoms, neuroimaging findings, delay between symptom onset and diagnosis or treatment, initial and further treatment, and outcome were investigated. Results Major symptoms were motor weakness and precocious puberty. Gadolinium-enhanced T1-weighted MR images showed enhancement in 8 of 11 patients examined, but only slight hyperintensity without enhancement in 2 patients. Ipsilateral peduncle and hemispheric atrophy were found in 3 and 4 patients, respectively. Cases of basal ganglia GCT were characterized by a longer delay from the initial neuroimaging examination to diagnosis compared with GCT in other regions. Five patients had aggravated hemiparesis in the extremities due to the delay in diagnosis. Despite good response to the initial therapy, 5 patients experienced recurrence; 2 of these 5 had malignant GCTs, and 3 had been treated only with chemotherapy or radiochemotherapy with insufficient radiation dose and field. Finally, the 2 patients with malignant GCTs died of the disease, and 1 died of aspiration pneumonia due to dissemination around the brainstem. Conclusions Early diagnosis requires MR imaging with administration of contrast medium in young patients presenting with motor weakness and/or precocious puberty. Serial neuroimaging studies should be performed if any tiny lesion is detected in the basal ganglia. Since insufficient treatment resulted in early recurrence, radiation therapy with adequate dose and field is essential.

scholarly journals Use of antidepressants among Finnish family caregivers: a nationwide register-based study

2021 ◽  
Author(s):  
Tuija M. Mikkola ◽  
Hannu Kautiainen ◽  
Minna Mänty ◽  
Mikaela B. von Bonsdorff ◽  
Hannu Koponen ◽  
...  
Keyword(s):  
Relative Risk ◽  
Family Caregivers ◽  
High Intensity ◽  
Matched Control ◽  
Age Groups ◽  
Control Population ◽  
Defined Daily Doses ◽  
Male Caregivers ◽  
And Gender

Abstract Purpose The purpose of this study was to compare the use of antidepressants over 6 years between family caregivers providing high-intensity care and a matched control population using register-based data. Methods The study includes all individuals, who received family caregiver’s allowance in Finland in 2012 (n = 29,846 females, mean age 66 years; n = 12,410 males, mean age 71 years) and a control population matched for age, sex, and municipality of residence (n = 59,141 females; n = 24,477 males). Information on purchases of antidepressants, including the number of defined daily doses (DDD) purchased, between 2012 and 2017 was obtained from the national drugs reimbursement register. Results During the follow-up, 28.5% of female caregivers and 23.5% of the female controls used antidepressants, while the numbers for males were 21.1% and 16.4%, respectively. Adjusted for socioeconomic status, female caregivers used 43.7 (95% confidence interval 42.4–45.0) and their controls used 36.2 (35.3–37.2) DDDs of antidepressants per person-year. Male caregivers used 29.6 (27.6–31.6) and their controls used 21.6 (20.2–23.0) DDDs of antidepressants per person-year. Among female caregivers, the relative risk for use of antidepressants was similar (about 1.3) from 20 to 70 years, after which the relative risk declined. In male caregivers, the relative risk was highest (about 1.4–1.5) between 45 and 65 years. Conclusions Family caregivers providing high-intensity care use more antidepressants and hence, are likely to have poorer mental health than the age-matched general population in virtually all age groups. However, the magnitude of the higher use varies as a function of age and gender.

Abstract P156: Assessing the Effect of the Heart of New Ulm Project: a Population-Based Program to Reduce Cardiovascular Disease

Circulation ◽  
2017 ◽  
Vol 135 (suppl_1) ◽  
Author(s):  
Abbey C Sidebottom ◽  
Arthur Sillah ◽  
David M Vock ◽  
Michael M Miedema ◽  
Raquel Pereira ◽  
...  
Keyword(s):  
Risk Factors ◽  
Blood Pressure ◽  
Cardiovascular Disease ◽  
Health System ◽  
Matched Control ◽  
Population Based ◽  
Control Population ◽  
Cvd Risk Factors ◽  
Cvd Risk ◽  
Matched Control Population

Background: Despite a highly recognized priority for public health and healthcare to implement population-level strategies to reduce the burden of cardiovascular disease (CVD), limited evidence exists on the most effective strategies. Data collection and evaluation of large scale, community based-prevention programs can be challenging and costly to achieve. The Heart of New Ulm (HONU) Project, begun in 2009, is a population-based initiative with healthcare, community, and workplace interventions addressing multiple levels of the social-ecological model designed to reduce modifiable CVD risk factors in rural New Ulm, MN. The community is served by one health system, enabling the use of electronic health record (EHR) data for surveillance. Objective: To assess trends for CVD risk factors, events, and healthcare utilization for New Ulm residents compared to a matched control population. Methods: We matched New Ulm residents (n = 4,077) with controls (n = 4,077) from a regional community served by the same health system using refined covariate balance techniques to match on baseline demographics, CVD risk factors, and health care utilization. Mixed effects longitudinal models with adjustment for age and gender, and an interaction for time by community, were run. Model based estimates were constructed for the entire cohort at each time period. Results: Over the first 6 years of the HONU Project,blood pressure, LDL, total cholesterol, and triglycerides were managed better in New Ulm than the matched comparison community. The proportion of New Ulm residents with controlled blood pressure increased by 6.2 percentage points while the control group increased by 2 points. 10-year ASCVD risk scores showed less decline for New Ulm residents than controls (16 vs. 18.4). The intervention and control groups did not differ with regard to inpatient stays, CVD events, smoking, or glucose. Conclusions: Compared to a matched control population, we found improved control of CVD risk factors in the New Ulm Population exposed to the HONU Project.

Regionalization within the mammalian telencephalon is mediated by changes in responsiveness to Sonic Hedgehog

Development ◽  
1998 ◽  
Vol 125 (24) ◽  
pp. 5079-5089 ◽  
Author(s):  
J.D. Kohtz ◽  
D.P. Baker ◽  
G. Corte ◽  
G. Fishell
Keyword(s):  
Basal Ganglia ◽  
Globus Pallidus ◽  
Sonic Hedgehog ◽  
Adult Brain ◽  
Ganglionic Eminence ◽  
Lateral Ganglionic Eminence ◽  
Expression Characteristic ◽  
Sonic Hedgehog Gene ◽  
Embryonic Structure ◽  
Sequential Induction

The cortex and basal ganglia are the major structures of the adult brain derived from the embryonic telencephalon. Two morphologically distinct regions of the basal ganglia are evident within the mature ventral telencephalon, the globus pallidus medially, and the striatum, which is positioned between the globus pallidus and the cortex. Deletion of the Sonic Hedgehog gene in mice indicates that this secreted signaling molecule is vital for the generation of both these ventral telencephalic regions. Previous experiments showed that Sonic Hedgehog induces differentiation of ventral neurons characteristic of the medial ganglionic eminence, the embryonic structure which gives rise to the globus pallidus. In this paper, we show that later in development, Sonic Hedgehog induces ventral neurons with patterns of gene expression characteristic of the lateral ganglionic eminence. This is the embryonic structure from which the striatum is derived. These results suggest that temporally regulated changes in Sonic Hedgehog responsiveness are integral in the sequential induction of basal telencephalic structures.

scholarly journals Disrupted basal ganglia—thalamocortical loops in focal to bilateral tonic-clonic seizures

2019 ◽  
Author(s):  
Xiaosong He ◽  
Ganne Chaitanya ◽  
Burcu Asma ◽  
Lorenzo Caciagli ◽  
Danielle S. Bassett ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Temporal Lobe Epilepsy ◽  
Globus Pallidus ◽  
Temporal Lobe ◽  
Functional Organization ◽  
Globus Pallidus Internus ◽  
Effective Control ◽  
Thalamocortical Connections ◽  
Clonic Seizures ◽  
Current History

AbstractFocal to bilateral tonic-clonic seizures are associated with lower quality of life, higher risk of seizure-related injuries, increased chance of sudden unexpected death, as well as unfavorable treatment outcomes. Achieving greater understanding of its underlying circuitry offers better opportunity to control these particularly serious seizures. Towards this goal, we provide a network science perspective of the interactive pathways among basal ganglia, thalamus and the cortex, to explore the imprinting of secondary seizure generalization on the mesoscale brain network in temporal lobe epilepsy. Specifically, we parameterized the functional organization of both the thalamocortical network and the basal ganglia—thalamus network with resting-state functional magnetic resonance imaging in three groups of patients with different focal to bilateral tonic-clonic seizure histories. Using the participation coefficient to describe the pattern of thalamocortical connections among different cortical networks, we showed that, compared to patients with no previous history, those with positive histories of focal to bilateral tonic-clonic seizures, including both remote (none for over one year) and current (within the past year) histories, presented more uniform distribution patterns of thalamocortical connections in the ipsilateral medial-dorsal thalamic nuclei. As a sign of greater thalamus mediated cortico-cortical communication, this result comports with greater susceptibility to secondary seizure generalization from the epileptogenic temporal lobe to broader brain networks in these patients. Using interregional integration to characterize the functional interaction between basal ganglia and thalamus, we demonstrated that patients with current history presented increased interaction between putamen and globus pallidus internus, and decreased interaction between the latter and the thalamus, compared to the other two patient groups. Importantly, through a series of “disconnection” simulations, we showed that these changes in interactive profiles of the basal ganglia—thalamus network in the current history group mainly depended upon the direct but not the indirect basal ganglia pathway. It is intuitively plausible that such disruption in the striatum modulated tonic inhibition of the thalamus from the globus pallidus internus could lead to an under-suppressed thalamus, which in turn may account for their greater vulnerability to secondary seizure generalization. Collectively, these findings suggest that the broken balance between the basal ganglia inhibition and thalamus synchronization can inform the presence and effective control of focal to bilateral tonic-clonic seizures. The mechanistic underpinnings we uncover may shed light on the development of new treatment strategies for patients with temporal lobe epilepsy.

scholarly journals Effects of Dopamine Depletion on Network Entropy in the External Globus Pallidus

2009 ◽  
Vol 102 (2) ◽  
pp. 1092-1102 ◽  
Author(s):  
Ana V. Cruz ◽  
Nicolas Mallet ◽  
Peter J. Magill ◽  
Peter Brown ◽  
Bruno B. Averbeck
Keyword(s):  
Basal Ganglia ◽  
Globus Pallidus ◽  
Dopamine Depletion ◽  
Information Coding ◽  
Logistic Regression Models ◽  
Firing Rates ◽  
Before And After ◽  
Network Entropy ◽  
Coding Capacity

Dopamine depletion in cortical-basal ganglia circuits in Parkinson's disease (PD) grossly disturbs movement and cognition. Classic models relate Parkinsonian dysfunction to changes in firing rates of basal ganglia neurons. However, disturbances in other dynamics of neural activity are also common. Taking both inappropriate firing rates and other dynamics into account and determining how changes in the properties of these neural circuits that occur during PD impact on information coding are thus imperative. Here, we examined in vivo network dynamics in the external globus pallidus (GPe) of rats before and after chronic dopamine depletion. Dopamine depletion led to decreases in the firing rates of GPe neurons and increases in synchronized network oscillations in the β frequency (13–30 Hz) band. Using logistic regression models, we determined the combined and separate impacts of these factors on network entropy, a measure of the upper bound of information coding capacity. Importantly, changes in these features in dopamine-depleted rats led to a significant decrease in GPe network entropy. Changes in firing rates had the largest impact on entropy, with changes in synchrony also decreasing entropy at the network level. Changes in autocorrelations tended to offset these effects because autocorrelations decreased entropy more in the control animals. Thus it is possible that reduced information coding capacity within basal ganglia networks may contribute to the behavioral deficits accompanying PD.

Subthalamo-Pallidal Circuit

2017 ◽  
pp. 143-150
Author(s):  
Charles J. Wilson
Keyword(s):  
Basal Ganglia ◽  
Substantia Nigra ◽  
Globus Pallidus ◽  
Subthalamic Nucleus ◽  
Substantia Nigra Pars Reticulata ◽  
Modern Methods

The subthalamo-pallidal system constitutes the second layer of circuitry in the basal ganglia, downstream of the striatum. It consists of four nuclei. Two of them, the external segment of the globus pallidus (GPe) and subthalamic nucleus (STN), make their connections primarily within the basal ganglia. The others, the internal segment of the globus pallidus (GPi) and the substantia nigra pars reticulata (SNr), are the output nuclei of the basal ganglia. Collectively, their axons distribute collaterals to all the targets of the basal ganglia. Rare interneurons have been reported in each of them from studies of Golgi-stained preparations, but they have not so far been confirmed using more modern methods. The circuit as described here is based primarily on studies of the axonal arborizations of neurons stained individually by intracellular or juxtacellular labeling.

Sign in / Sign up

Export Citation Format

Share Document