Genetically engineered systems revealed the roles of basal ganglia in sleep-wake regulation

The basal ganglia(BG) act as a cohesive functional unit that regulates motor function,habit formation,and reward/addictive behaviors. However,it is still not well understood how the BG maintains wakefulness and suppresses sleep to achieve al these fundamental functions until genetical y engineered systems developed these years. Significant research efforts have recently been directed at developing genetic-molecular tools to achieve reversible and cell-type specific in vivo silencing or activation of neurons in behaving animals. Optogenetic tools can be used both to specifically activate or inhibit neurons of interest and identify functional synaptic connectivity between specific neuronal populations,both in vivo and in brain slices. Another recently developed system by Roth and colleagues permits the selective and ″remote″ manipulation(activation and silencing) of neuronal activity via all 3 major GPCR signaling pathways(G_i,G_s and G_q). These so-called ″ designer receptors exclusively activated by designer drugs″(DREADD) involve mutant GPCRs that do not respond to their endogenous ligands but are responsive to otherwise inert biological compounds. Recently,we demonstrated the essential roles and the neural pathways of the neurons expressing adenosine A_(2A) receptors or dopamine D_1 receptors in the BG for sleep-wake regulation using the genetically engineered systems including optogenetics and DREADD. We proposed a plausible model in which the caudate-putamen and the nucleus accumbens integrates behavioral processes with sleep/wakefulness through adenosine and dopamine receptors.

Emergence of lesions outside of the basal ganglia and irreversible damage to the basal ganglia with severeβ-ketothiolase deficiency:A case report

BACKGROUNDβ-ketothiolase deficiency(β-KTD)is an inherited disease,and insufficient attention has been paid to imageology due to its lower morbidity.Therefore,few lesions outside the basal ganglia have been found before,and the persistent pathological changes have rarely been reported.CASE SUMMARY A 10-mo-old Chinese female patient with a free previous medical history but with poor physical and athletic development had received the haemophilus influenzae vaccine and then developed a low fever 2 d prior.She was initially diagnosed with severe brain injury,central respiratory failure,metabolic acidosis complicated with respiratory alkalosis,hyper-IgE,etc.With further examination,a definite diagnosis ofβ-KTD was made.Symptomatic treatment was adopted.Ten days later,the dyspnea was improved evidently and the ventilator was removed,but there were still obvious abnormalities on magnetic resonance imaging(MRI).The lesions mainly invaded the corpus striatum but were not limited to the basal ganglia.Then,the patient’s disease improved and discharged approximately 1 mo later,and the abnormal lesions on MRI had partially improved.However,for about 1 year,the residual irreversible lesions were observed on MRI,the mental and physical development of the patient was obviously regressive,and extra rehabilitation training was needed.CONCLUSION The case highlights the critical importance of one view that the range of lesions in some patients may be more extensive than previously thought in someβ-KTD patients.In addition to biochemical tests,genetic tests and magnetic resonance imaging are not only conducive to quickly diagnosingβ-KTD but also to partially evaluating the short-and long-term outcomes.Moreover,more attention should be paid to the two mutations(c.478 C>G;c.951 C>T)that may be associated with severeβ-KTD.

Relationship between abnormal vagus nerve tension and basal ganglia cerebral infarction induced paroxysmal atrial fibrillation

Objective: To investigate the relationship between basal ganglia cerebral infarction and paroxysmal atrial fibrillation(PAF) caused by abnormal vagus nerve tension.Methods: A total of 1 483 cases of elder patients with cerebral infarction who received head CT or MRI examination during the period were enrolled, including 830 male and613 female, with the average age as 78 years. These cases were divided into basal infarction ganglia group(n = 1 045) and non-basal ganglia infarction group(n = 438)according to the anatomic site of cerebral infarction. The differences of the incidence of PAF, left atrial diameter and heart rate variability were compared between the two groups.Results: In basal ganglia infarction group, the incidence rate of PAF was significantly higher than that of non-basal ganglia infarction group(P < 0.05). The incidence trend of cerebral infarction in basal ganglia was age-related, in the >79 years basal ganglia cerebral infarction group, the incidence of PAF was significantly higher than that of nonbasal ganglia infarction group(P < 0.05). There was no significant difference in the left atrial diameter between the basal ganglia infarction group and non-basal ganglia infarction group. Basal ganglia cerebral infarction patients with high PAF had higher heart rate variability than non-basal ganglia infarction group.Conclusion: Elderly patients with basal ganglia infarction have high incidence of PAF.Sympathetic nerve damage in cerebral basal ganglia, increased vagal tension and cardiac vagal tension are the direct causes of PAF. The results indicates that the increased central vagal nerve tension mediated PAF probably is an indication of supplying sympathetic neurotransmitter or cardiac vagal denervation treatment.

Resuscitating acupuncture therapy for glucose metabolism in acute cerebral infarction of basal ganglia

BACKGROUND： Acupuncture can improve motor function in patients with cerebral infarction, and activate brain glucose metabolism in relevant brain areas. However, the association between encephalic region activation and acupuncture, as well as the clinical significance of activation remain unclear. OBJECTIVE： Through the use of positron emission tomography-computed tomography （PET-CT）, acute cerebral infarction patients were analyzed for global cerebral metabolism, cerebral infarction focus, peripheral edema, and pyramidal tract pathway changes, which were directly related to clinical symptoms. The influence of resuscitating acupuncture on cerebral glucose metabolism was analyzed in patients with acute cerebral infarction in basal ganglia. DESIGN, TIME AND SETTNG： Randomized, controlled, clinical trials were performed from March 2007 to October 2008 at the PET-CT Center of the General Hospital of Tianjin Medical University, China. PARTICIPANTS： Twelve patients with acute basal ganglia infarction were recruited from the First Affiliated Hospital of Tianjin University of Traditional Chinese Medicine, the Second Affiliated Hospital of Tianjin University of Traditional Chinese Medicine, Tianjin Chinese Medicine Hospital, and Affiliated Hospital of Medical College of Chinese People＇s Armed Police Force. METHODS： The cerebral infarcted patients were randomly assigned to acupuncture and control groups. In addition to routine treatment, the acupuncture group was treated by acupuncture at the main acupoints for resuscitation [Neiguan （PC 6）, Renzhong （DU 26）, and Sanyinjiao （SP 6）], while the control group received routine treatment. MAIN OUTCOME MEASURES： Before and after treatment, patients with acute cerebral infarction were evaluated for global brain, cerebral infarction focus, and surrounding edema and glucose metabolism in encephalic region of pyramidal tract conduction by 18-labeled fluorodeoxyglucose for PET-CT imaging. RESULTS： The resuscitating acupuncture therapy can significantly activate the metabolism of global brain, infarction center and surrounding edema in patients with cerebral infarction in basal ganglia, also has effects on the activation of glucose metabolism in the encephalic regions of pyramidal tract pathway （P 〈 0.05）. CONCLUSION： Resuscitating acupuncture was superior to routine treatment for significantly activating glucose metabolism in patients with acute cerebral basal ganglia infarction.

Germinomas of the basal ganglia and thalamus: Four case reports

BACKGROUND The early diagnosis of basal ganglia and thalamus germinomas is often difficult due to the absence of elevated tumor markers,and atypical clinical symptoms and neuroimaging features.CASE SUMMARY Four male children aged 8 to 15 years were diagnosed with germinomas in the basal ganglia and thalamus by stereotactic biopsy from 2017 to 2019.All patients developed hemiplegia except patient 4 who also had cognitive decline,speech disturbance,nocturnal enuresis,polydipsia,polyuria,precocious puberty and abnormalities of thermoregulation.All four cases were alpha-fetoprotein and beta-human chorionic gonadotrophin(p-HCG)negative except patient 3 who had slightly elevated p-HCG in cerebrospinal fluid(CSF).No malignant cells were detected in the patients'CSF.Brain magnetic resonance imaging findings were diverse in these patients with the exception of the unique and common characteristics of ipsilateral hemisphere atrophy,especially in the cerebral peduncle.All patients were diagnosed with germinomas of the basal ganglia and thalamus by stereotactic brain biopsy.CONCLUSION Stereotactic brain biopsy is necessary to confirm the diagnosis of ectopic germinomas.Serial neuroimaging studies can not only differentiate disease but also determine the biopsy site.

Idiopathic basal ganglia calcification associated with new MYORG mutation site:A case report

BACKGROUND Idiopathic basal ganglia calcification(IBGC)is a neurodegenerative disease characterized by symmetrical calcification of basal ganglia and other brain region,also known as Fahr’s disease.It can be sporadic or familial,and there is no definite etiology at present.With the development of neuroimaging,the number of reports of IBGC has increased in recent years.However,due to its hidden onset,diverse clinical manifestations,and low incidence,it is likely to be misdiagnosed or ignored by potential patients and their family.CASE SUMMARY We report a case of a 61-year-old man who presented with symptoms of dysphagia and alalia.His computed tomography scan of the brain revealed bilateral symmetric calcifications of basal ganglia,cerebellum,thalamus,and periventricular area.The genetic test showed a new mutation sites of MYORG,c.1438T>G mutation and c.1271_1272 TGGTGCGC insertion mutation.He was finally diagnosed with IBGC.CONCLUSION It is important to detect MYORG mutation when IBGC is suspected,especially in those without an obvious family history,for better understanding of the underlying mechanism and identifying potential treatments.

Abnormal Basal Ganglia Functional Connectivity in Idiopathic Generalized Epilepsy

The basal ganglia have been implicated in a modulation role in idiopathic generalized epilepsy （IGE） by an invasive electrophysioigic means. This paper investigates the basal ganglia functional connectivity by using the region-wise functional connection analysis in resting-state functional magnetic resonance imaging （fMRI） in IGE. The increased functional connectivity within basal ganglia, and between the basal ganglia and the thalamus, and decreased functional connectivity between basal ganglia and motor cortex are found in IGE compared with the controls. These findings not only implicate dysfunctional integration in the motor loop in IGE and the enhanced interaction in the modulated loop, but also suggest that the basal ganglia modulate the generalized epileptic discharges with the influence over thalamus in the corticothalamus network.

DISTRIBUTION OF PARVALBUMIN, CALBINDIN-D28 AND CALRETININ IMMUNOREACTIVE NEURONS AND FIBERS IN THE MONKEY BASAL GANGLIA

Objective To investigate the cellular localization of parvalbumin (PV), calbindin-D28k (CB) and calretinin (CR) in the monkey basal ganglia.Methods Immunocytochemical technique was used to detect PV,CB and CR immunoreactivity in the basal ganglia. Results In the striatum, CB labeled medium-sized spiny projection neurons whereas PV and CR marked two separate classes of aspiny interneurons. The striatal matrix compartment was markedly enriched with CB while striatal patches displayed a CR-rich neuropil. In the pallidum, virtually all neurons contained PV but none express CB. CR occured only in a small subpopulation of large and small pallidal neurons. In the subthalamic nucleus, there existed a multitude of PV-positive cells and fibers but the number of CR and CB-positive neuronal elements was small. In the substantia nigra / ventral tegmental area complex, CB and CR occured principally in dopaminergic neurons of the dorsal tier of the pars compacta and in those of the ventral tegmental area. PV was strickly confined to the GABAergic neurons of the pars reticular and lateralis. CB-rich fibers abounded in the pars reticular and lateralis, while CR-positive axons were confined to the pars compacta. Conclusion CB and PV were distributed according to a strikingly complementary pattern in primate basal ganglia, and the use of CB and PV immunocytochemistry may be considered as an excellent tool to define distinct chemoarchitectonic and functional domains within the complex organization of the basal ganglia. CR was less ubiquitous but occured in small basal ganglia components where it labeled distinct subsets of neurons. Such highly specific patterns of distribution indicate that CB, PV and CR may work in synery within primate basal ganglia.

Characteristics of basal ganglia aphasia after stroke and the rehabilitative interventions

OBJECTIVE: To introduce the characteristics of basal ganglia aphasia after stroke and the rehabilitative interventions. DATA SOURCES: Articles related to stroke, subcortical aphasia, basal ganglia aphasia and language rehabilitation published in Chinese from January 1988 to December 2005 were searched in Chinese journal full-text database (CJFD) using the Keywords of 'stroke, basal ganglia aphasia, language rehabilitation' in Chinese. Meanwhile, English articles about aphasia published from January 1982 to December 2005 were searched in and Pubmed database. Besides, several books associated with the contents were looked through manually. STUDY SELECTION: The data were checked primarily, the articles about the pathomechanism and neurolinguistic characteristics of basal ganglia aphasia, diagnostic methods of aphasia and language rehabilitation were selected, and those had no obvious relation with the above contents were excluded. Inclusive criteria: literatures explain the clinical characteristics of basal ganglia aphasia, neurolinguistic pathogenesis and methods of rehabilitation therapy in details. The repetitive studies were excluded. DATA EXTRACTION: Totally 95 literatures about basal ganglia aphasia were collected, including 31 about the clinical characteristics of basal ganglia aphasia, 45 about its neurolinguistic pathogenesis, 5 about the evaluation and classification of aphasia, and 14 about its rehabilitation therapy. Thirty accorded with the inclusive criteria were used for review, and the other 65 were excluded. DATA SYNTHESIS: Concisely introduced the definition, past investigation of basal ganglia aphasia after stroke, then dwelled on the multiplicity neurolinguistics characteristics. Aphasia evaluation was dependent upon clinical aphasic symptoms. The relationship between symptom and focus of infection was explored, and the mechanism of pathosis language behavior on basal ganglia aphasia patients was understood to provide consequence data that could authenticate the processing of language in brain. On the other hand, the method of rehabilitation on basal ganglia aphasia after stroke was explained. CONCLUSION: Basal ganglia aphasia is manifested as atypical aphasic symptom, the mechanism for the structure of basal ganglia in the speech formation should be further confirmed. It is effective to select pertinent language rehabilitation for basal ganglia aphasia after stroke.

Analysis of correlation between MSCT classification and prognosis of basal ganglia hypertensive intracerebral hemorrhage

Objective:To investigate the correlation between MSCT grading and the prognosis of basal ganglia hypertensive intracerebral hemorrhage.Methods:A total of 86 patients with basal ganglia hypertensive cerebral hemorrhage admitted to our hospital from May 2017 to March 2018 were selected.The clinical data and imaging data were collected from 86 patients.The MSCT images were observed and summarized by two radiologists.Based on the morphology of cisterna ambiens and brain stem,the correlation of MSCT grading to Glasgow Coma Scale(GCS)and Glasgow Outcome Scale(GOS)grading was analyzed by use of Spearman correlation analysis.Results:Among 86 patients,the number of MSCT gradeⅠ,Ⅱ,ⅢandⅣwere 29 cases,38 cases,9 cases and 10 cases respectively.There was no significant correlation between MSCT grading and age,sex as well as types of combined underlying diseases in the patients with basal ganglia hypertensive intracerebral hemorrhage(p>0.05).Spearman correlation analysis showed that there was a positive correlation between MSCT grading and GCS score of basal ganglia hypertensive intracerebral hemorrhage.The higher MSCT grade was,the higher GCS score was(r=0.719,p<0.001).There was a positive correlation between MSCT grading and GOS grading of basal ganglia hypertensive intracerebral hemorrhage.The higher MSCT grade was,the higher GOS grade was,leading to a poor prognosis(r=0.734,p<0.001).86 cases of basal ganglia hypertensive intracerebral hemorrhage showed round or quasi-circular high-density shadows in MSCT images,the CT value ranged from 50 Hu to 80 Hu.Low-density bands surrounded the hematoma in the acute stage,and space-occupying effect could be seen in some patients,which resulted in the compression of ventricular sulcus and cistern and the displacement of midline structure.Subacute basal ganglia hypertensive intracerebral hemorrhage patients showed a relative decrease in the density of hematoma,with the extent of edema gradually reduced,the focus showed a high-density shadow in the center;chronic basal ganglia hypertensive intracerebral hemorrhage focus showed a cystic low-density shadow.Conclusion:MSCT grading of basal ganglia hypertensive intracerebral hemorrhage is positively correlated to GCS and GOS grading.MSCT grading can contribute to the prognostic evaluation to the patients.

Longitudinal observation of ten family members with idiopathic basal ganglia calcification: A case report

BACKGROUND Familial idiopathic basal ganglia calcification (FIBGC) is a rare autosomal dominant disorder that causes bilateral calcification of the basal ganglia and/or cerebellar dentate nucleus, among other locations. CASE SUMMARY The aim of this study is to report 10 cases of FIBGC observed in a single family. Seven patients showed calcification on their computed tomography scan, and all of these patients carried the SLC20A2 mutation. However, individuals without the mutation did not show calcification. Three patients among the 7 with calcification were symptomatic, while the remaining 4 patients were asymptomatic. Additionally, we longitudinally observed 10 subjects for ten years. In this paper, we mainly focus on the clinical course and neuroradiological findings in the proband and her son.CONCLUSION The accumulation of more case reports and further studies related to the manifestation of FIBGC are needed.

The History of the Basal Ganglia: The Contribution of Karl Friedrich Burdach

It took many centuries for the basal ganglia (BG) to be recognized as specific brain entities involved in the control of psychomotor behavior. Andreas Vesalius (1514-1564) was the first to delineate this set of structures, but he did not name them nor payany attention to their functional significance. This was left to the English physician Thomas Willis (1621-1675), who used the term corpus striatum (striated or chamfered body) to designate the largest BG constituent, which he considered a major sensorimotor integration center. Willis’s pioneering description influenced markedly some 18th and 19th centuries scholars, particularly the German physician and anatomist Karl Friedrich Burdach (1776-1847). Burdach’s insightful studies of the human brain are summarized in a three-volume treatise entitled Vom Baue und Leben des Gehirns (1819-1826). This landmark opus provides a description of the BGwhose originality has largely been overlooked. Burdach’s careful investigation allowed him to differentiate the caudate nucleus from the putamen, which he respectively termed Streifenhügel (elongated hillock) and Schale (shell). He also called the putamen Linsenkern (lens-shaped nucleus), a term that he admittedly borrowed from his compatriot Johann Christian Reil (1759-1813). He further identified a paler structure (blasser Klumpen) within the inner portion of the lentiform nucleus that he called globus pallidus, and correctly identified its inner and outer segments (innern und ?ussern Theil). He aptly pointed out that the major BG nuclei are separated from one another by fibers fascicles that he termed inner and ?ussre Capsel (internal and external capsules). Burdach also referred to the substantia nigra (schwarzgraue Schicht or stratum nigrum) and claustrum (Vormauer), but gave full credit to the French anatomist Félix Vicq-d’Azyr (1748-1794) for their discovery. Although Burdach did not comment much on BG function, his anatomical description was sufficiently cogent to be still in use two centuries after its inception.

Attention deficit hyperactivity disorder secondary to lesion of the basal ganglia

Attention Deficit Hyperactivity Disorder (ADHD) is a childhood onset autoregulation disorder characterized by attention deficit, hyperactivity and/or impul- siveness, which results in social and academic functional impairment. ADHD has a complex aetiology: along with genetic factors, anomalies in several cere-bral districts have been reported. We describe the case of a 9 year old boy with glucose-6-phosphate dehydrogenase deficiency in association with memory, behavioural and attentive disabilities. The patient’s clinical history is characterized by cerebral stroke at 3 years of age during a febrile episode, resulted in slight hemiparesis. Neuroimaging revealed a cystic lesion in the anterior portion of the right lenticular nucleus. Neuropsychological and psychiatric assess- ment evidenced alterations of executive functions. Diagnosis of ADHD secondary to lesion of the basal ganglia was made. We report the patient’s clinical profile in the light of current evidence pointing towards dysfunction of the basal ganglia as a crucial aetiological factor in memory and executive function impairment.

Streptococcal Antibody Probe Crosses the Blood Brain Barrier and Interacts within the Basal Ganglia

Within the brain, the basal ganglia (basal nuclei) regulates wanted movement and inhibits unwanted movement. This area of the brain is intertwined with capillary beds that bring nutrients to the brain and form the blood brain barrier. During disease state, antibodies are increased in circulation and movement of these antibodies into the basal ganglia can occur. Streptococcal infection can lead to the generation of antibodies that have autoimmune activity within the brain. These antibodies have been implicated in neurological disorders. In our laboratory, an in vitro study of a monoclonal mouse antibody generated against the class 1 epitope of the M6 protein has demonstrated binding within the basal ganglia of Lewis rat brains. Here we present an in vivo study using Lewis rats injected with either the streptococcal antibody or an anti-myosin positive control. The interaction and movement of the antibody from blood vessels into the tissues of the basal ganglia was determined through the use of immunofluorescence and fluorescent microscopy and is contrasted with IgG injected and uninjected controls. Our data demonstrates that the streptococcal antibody penetrates the blood brain barrier within 24 hours (as determined by the presence of immunofluorescence outside of blood vessels) and remains significantly elevated above control values even 72 hours after injection (p < 0.05). In contrast, the anti-myosin positive control was not visualized in the interstitial fluid until 48 hours post injection and was no longer significantly above control levels by 72 hours. IgG injected controls did not display movement of antibody into the brain. Therefore, the streptococcal antibody is capable of crossing the blood brain barrier and interacting with tissues of the basal ganglia.

Motor Learning Based on the Cooperation of Cerebellum and Basal Ganglia for a Self-Balancing Two-Wheeled Robot

A novel motor learning method is present based on the cooperation of the cerebellum and basal ganglia for the behavior learning of agent. The motor learning method derives from the principle of CNS and operant learning mechanism and it depends on the interactions between the basal ganglia and cerebellum. The whole learning system is composed of evaluation mechanism, action selection mechanism, tropism mechanism. The learning signals come from not only the Inferior Olive but also the Substantia Nigra in the beginning. The speed of learning is increased as well as the failure time is reduced with the cerebellum as a supervisor. Convergence can be guaranteed in the sense of entropy. With the proposed motor learning method, a motor learning system for the self-balancing two-wheeled robot has been built using the RBF neural networks as the actor and evaluation function approximator. The simulation experiments showed that the proposed motor learning system achieved a better learning effect, so the motor learning based on the coordination of cerebellum and basal ganglia is effective.

A Bio-Inspired Integration Model of Basal Ganglia and Cerebellum for Motion Learning of a Musculoskeletal Robot

It is a significant research direction for highly complex musculoskeletal robots that how to develop the ability of motion learning and generalization.The cooperations of multiple brain regions are crucial to improving motion performance.Inspired by the neural mechanisms of structures,functions,and interconnections of basal ganglia and cerebellum,a biologically inspired integration model for motor learning of musculoskeletal robots is proposed.Based on the neural characteristics of the basal ganglia,the basal ganglia actor network,which mainly simulates the dorsal striatum,outputs motion commands,and the basal ganglia critic network,which simulates the ventral striatum,estimates actionstate values.Their network parameters are updated using the soft actor-critic method.Based on the sensorimotor prediction mechanism of the cerebellum,the cerebellum network evaluates the state feature extraction quality of the basal ganglia actor network and then updates the weights of its feature layer.This learning method is proven to converge to the optimal policy.Furthermore,drawing on the mechanism of dopaminergic dynamic regulation in the basal ganglia,the adaptive adjustment of target entropy and the dopaminergic experience replay are proposed to further improve the integration model,which contributes to the exploration-exploitation trade-off of motor learning.The bio-inspired integration model is validated on a musculoskeletal system.Experimental results indicate that this model can effectively control the musculoskeletal robot to accomplish the motion task from random starting locations to random target positions with high precision and robustness.

The right inferior frontal gyrus as pivotal node and effective regulator of the basal ganglia-thalamocortical response inhibition circuit

Background:The involvement of specific basal ganglia-thalamocortical circuits in response inhibition has been extensively mapped in animal models.However,the pivotal nodes and directed causal regulation within this inhibitory circuit in humans remains con-troversial.Objective:The main aim of the present study was to determine the causal information flow and critical nodes in the basal ganglia-thalamocortical inhibitory circuits and also to examine whether these are modulated by biological factors(i.e.sex)and behavioral performance.Methods:Here,we capitalize on the recent progress in robust and biologically plausible directed causal modeling(DCM-PEB)and a large response inhibition dataset(n=250)acquired with concomitant functional magnetic resonance imaging to determine key nodes,their causal regulation and modulation via biological variables(sex)and inhibitory performance in the inhibitory circuit encompassing the right inferior frontal gyrus(rIFG),caudate nucleus(rCau),globus pallidum(rGP),and thalamus(rThal).Results:The entire neural circuit exhibited high intrinsic connectivity and response inhibition critically increased causal projections from the rIFG to both rCau and rThal.Direct comparison further demonstrated that response inhibition induced an increasing rIFG inflow and increased the causal regulation of this region over the rCau and rThal.In addition,sex and performance influenced the functional architecture of the regulatory circuits such that women displayed increased rThal self-inhibition and decreased rThal to GP modulation,while better inhibitory performance was associated with stronger rThal to rIFG communication.Furthermore,control analyses did not reveal a similar key communication in a left lateralized model.Conclusions:Together,these findings indicate a pivotal role of the rIFG as input and causal regulator of subcortical response inhibition nodes.

Sonographic Alteration of Basal Ganglia in Different Forms of Primary Focal Dystonia： A Cross-sectional Study

Background：Few studies have addressed whether abnormalities in the lenticular nucleus （LN） are characteristic transcranial sonography （TCS） echo features in patients with primary dystonia.This study aimed to explore alterations in the basal ganglia in different forms of primary focal dystonia.Methods：A cross-sectional observational study was performed between December 2013 and December 20 1 4 in 80 patients with different forms of primary focal dystonia and 55 neurologically normal control subjects.TCS was performed in patients and control subjects.Multiple comparisons of multiple rates were used to compare LN hyperechogenicity ratios between control and patient groups.Results：Thirteen individuals were excluded due to poor temporal bone windows,and two subjects were excluded due to disagreement in evaluation by sonologists.Totally,70 patients （cervical dystonia,n =30;blepharospasm,n =30;oromandibular dystonia,n =10） and 50 normal controls were included in the final analysis.LN hyperechogenicity was observed in 51% （36/70） of patients with primary focal dystonia,compared with 12% （6/50） of controls （P 〈 0.001）.Substantia nigra hyperechogenicity did not differ between the two groups.LN hyperechogenicity was observed in 73% （22/30） of patients with cervical dystonia,a greater prevalence than in patients withblepharospasm （33%,10/30,P =0.002） and oromandibular dystonia （40%,4/10,P =0.126）.LN hyperechogenicity was more frequently observed in patients with cervical dystonia compared with controls （73% vs.12%,P 〈 0.001）;however,no significant difference was detected in patients with blepharospasm （33% vs.12%,P =0.021） or oromandibular dystonia （40% vs.12%,P =0.088）.Conclusions：LN hyperechogenicity is more frequently observed in patients with primary focal dystonia than in controls.It does not appear to be a characteristic TCS echo feature in patients with blepharospasm or oromandibular dystonia.

Properties of oscillatory neuronal activity in the basal ganglia and thalamus in patients with Parkinson’s disease

Background:The cardinal features of Parkinson’s disease(PD)are bradykinesia,rigidity and rest tremor.Abnormal activity in the basal ganglia is predicted to underlie the mechanism of motor symptoms.This study aims to characterize properties of oscillatory activity in the basal ganglia and motor thalamus in patients with PD.Methods:Twenty-nine patients with PD who underwent bilateral or unilateral electrode implantation for subthalamic nucleus(STN)DBS(n=11),unilateral pallidotomy(n=9)and unilateral thalamotomy(n=9)were studied.Microelectrode recordings in the STN,globus pallidus internus(GPi)and ventral oral posterior/ventral intermediate of thalamus(Vop/Vim)were performed.Electromyography of the contralateral limbs was recorded.Single unit characteristics including interspike intervals were analyzed.Spectral and coherence analyses were assessed.Mean spontaneous firing rate(MSFR)of neurons was calculated.Analysis of variance and χ^(2) test were performed.Results:Of 76 STN neurons,39.5% were 4–6 Hz band oscillatory neurons and 28.9% were β frequency band(βFB)oscillatory neurons.The MSFR was 44.2±7.6 Hz.Of 62 GPi neurons,37.1% were 4–6 Hz band oscillatory neurons and 27.4% were βFB neurons.The MSFR was 80.9±9.6 Hz.Of 44 Vop neurons,65.9% were 4–6 Hz band oscillatory neurons and 9%were βFB neurons.The MSFR was 24.4±4.2 Hz.Of 30 Vim oscillatory neurons,70% were 4–6 Hz band oscillatory neurons and 13.3% were β FB neurons.The MSFR was 30.3±3.6 Hz.Further analysis indicated that proportion of βFB oscillatory neurons in STN and GPi was higher than that of similar neurons in the Vop and Vim(P<0.05).Conversely,the proportion of 4–6 Hz band oscillatory neurons and tremor related neurons in the Vim and Vop was higher than that of STN and GPi(P<0.05).The highest MSFR was for GPi oscillatory neurons whereas the lowest MSFR was for Vop oscillatory neurons(P<0.005).Conclusion:The alterations in neuronal activity in basal ganglia play a critical role in generation of parkinsonism.β oscillatory activity is more prominent in basal ganglia than in thalamus suggesting that the activity likely results from dopaminergic depletion.While both basal ganglia and thalamus have tremor activity,the thalamus appears to play a more important role in tremor production,and basal ganglia β oscillatory activity might be the trigger.

Idiopathic Basal Ganglia Calcification Presented with Progressive Supranuclear Palsy-like Features

Fahr＇s disease, or idiopathic basal ganglia calcification （IBGC）, is a rare neurological syndrome characterized by abnormal calcified deposits located mostly in bilateral basal ganglia and dentate nucleus, and also in cerebral cortex, thalamus, hippocampus, cerebellar, and subcortical white matter. Clinically, it presents various symptoms, including parkinsonism （presented in 57% of the patientsL chorea （ 19%）, tremors （8%）, dystonia （8%）, athetosis （5%）~ and orofacial dyskinesia （3%）.