功能性运动障碍(functional movement disorders,FMDs)是一类常见的分离/转换性障碍,已成为运动障碍疾病的重要分支,但多数临床医师对此疾病的认知不足。基于此,本文综述了FMDs的临床表现、辅助检查、诊断及鉴别诊断、治疗及预后,以期...功能性运动障碍(functional movement disorders,FMDs)是一类常见的分离/转换性障碍,已成为运动障碍疾病的重要分支,但多数临床医师对此疾病的认知不足。基于此,本文综述了FMDs的临床表现、辅助检查、诊断及鉴别诊断、治疗及预后,以期提高临床医生对FMDs的认识,提高临床诊断正确率,为准确治疗提供可能性。展开更多
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 ...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.展开更多
There has been a growing appreciation for freezing of gait as a disabling symptom that causes a significant burden in Parkinson’s disease. Previous research has highlighted some of the key components that underlie th...There has been a growing appreciation for freezing of gait as a disabling symptom that causes a significant burden in Parkinson’s disease. Previous research has highlighted some of the key components that underlie the phenomenon, but these reductionist approaches have yet to lead to a paradigm shift resulting in the development of novel treatment strategies. Addressing this issue will require greater integration of multi-modal data with complex computational modeling, but there are a number of critical aspects that need to be considered before embarking on such an approach. This paper highlights where the field needs to address current gaps and shortcomings including the standardization of definitions and measurement, phenomenology and pathophysiology, as well as considering what available data exist and how future studies should be constructed to achieve the greatest potential to better understand and treat this devastating symptom.展开更多
Photophobia is a common symptom seen in many neurologic disorders,however,its pathophysiology remains unclear.Even the term is ambiguous.In this paper,we review the epidemiology and clinical manifestations of photopho...Photophobia is a common symptom seen in many neurologic disorders,however,its pathophysiology remains unclear.Even the term is ambiguous.In this paper,we review the epidemiology and clinical manifestations of photophobia in neurological disorders,including primary headache,blepharospasm,progressive supranuclear palsy,and traumatic brain injury,discuss the definition,etiology and pathogenesis,and summarize practical methods of diagnosis and treatment.展开更多
Thousands of resting state functional magnetic resonance imaging(RS-f MRI)articles have been published on brain disorders.For precise localization of abnormal brain activity,a voxel-level comparison is needed.Because ...Thousands of resting state functional magnetic resonance imaging(RS-f MRI)articles have been published on brain disorders.For precise localization of abnormal brain activity,a voxel-level comparison is needed.Because of the large number of voxels in the brain,multiple comparison correction(MCC)must be performed to reduce false positive rates,and a smaller P value(usually including either liberal or stringent MCC)is widely recommended[1].展开更多
Background:Focal dystonia is a neurological disorder characterized by unwanted muscle spasms.Blepharospasm is a focal dystonia producing an involuntary closure of the eyelid.Its etiology is unknown.Objective:To invest...Background:Focal dystonia is a neurological disorder characterized by unwanted muscle spasms.Blepharospasm is a focal dystonia producing an involuntary closure of the eyelid.Its etiology is unknown.Objective:To investigate if there are structural changes in the white and grey matter of blepharospasm patients,and if the changes are related to disease features.Methods:T1 and diffusion-weighted magnetic resonance imaging scans were collected from 14 female blepharospasm patients and 14 healthy matched controls.Grey matter volumes,fractional anisotropy(FA),and mean diffusivity maps were compared between the groups.Based on grey matter differences within the facial portion of the primary motor cortex,the corticobulbar tract was traced and compared between groups.Results:Changes in grey matter in patients included the facial portion of the sensorimotor area and anterior cingulate gyrus.These changes did not correlate with disease duration.Corticobulbar tract volume and peak tract connectivity were decreased in patients compared with controls.There were no significant differences in FA or mean diffusivity between groups.Conclusions:Grey matter changes within the primary sensorimotor and the anterior cingulate cortices in blepharospasm patients may help explain involuntary eyelid closure and the abnormal sensations often reported in this condition.展开更多
Te content of the rectifed motor evoked potential(MEP)induced by transcranial magnetic stimulation(TMS)has ambiguously been assessed without the precision that energy calculation deserves.Tis fact has misled data inte...Te content of the rectifed motor evoked potential(MEP)induced by transcranial magnetic stimulation(TMS)has ambiguously been assessed without the precision that energy calculation deserves.Tis fact has misled data interpretation and misguided biomedical interventions.To defnitively fll the gap that exits in the neurophysics processing of these signals,we computed,in Walls(̂�),the bioenergy within the rectifed MEP recorded from the human frst digitorum index(FDI)muscle at rest and under isometric contraction.We also gauged the biowork exerted by this muscle.Here we show that bioenergy and biowork can accurately and successfully be assessed,validated,and determined in̂�from MEP signals induced by TMS,regardless of knowing the mathematical expression of the function of the signal.Our novel neurophysics approach represents a dramatic paradigm shif in analysis and interpretation of the content of the MEP and will give a true meaning to the content of rectifed signals.Importantly,this innovative approach allowed unveiling that women exerted more bioenergy than men at the magnetic stimulations used in this study.Revisitation of conclusions drawn from studies published elsewhere assessing rectifed EMG signals that have used ambiguous units is strongly recommended.展开更多
Prevention The best treatment is to prevent the illness in the first place or to retard its progression. There is considerable new information about Parkinson's disease genetics and pathophysiology that hopefu... Prevention The best treatment is to prevent the illness in the first place or to retard its progression. There is considerable new information about Parkinson's disease genetics and pathophysiology that hopefully will lead to therapy in this regard, but there is still nothing definitive. The only medication for which there are some reasonable data is selegiline, a monoamine oxidase (MAO)-B inhibitor. This drug actually has a symptomatic effect since it prevents the breakdown ofdopamine in the brain. Some trials have shown a beneficial effect separate from its symptomatic effect. The situation is rather controversial, however, and in one study there was an excess of mortality. {4}There are no supportive data for antioxidants such as vitamin E, and a trial of the neurotrophic factor GDNF was not positive.
……展开更多
The Movement Disorder Society (MDS) is the international not-for-profit organization representing and serving clinicians, other health professionals, researchers and policy makers interested in the area of movemen... The Movement Disorder Society (MDS) is the international not-for-profit organization representing and serving clinicians, other health professionals, researchers and policy makers interested in the area of movement disorders. The Society is represented in 68 countries by approximately 1,500 members. The Society has developed regional sections and welcomes affiliation of regional Movement Disorder groups.
……展开更多
文摘功能性运动障碍(functional movement disorders,FMDs)是一类常见的分离/转换性障碍,已成为运动障碍疾病的重要分支,但多数临床医师对此疾病的认知不足。基于此,本文综述了FMDs的临床表现、辅助检查、诊断及鉴别诊断、治疗及预后,以期提高临床医生对FMDs的认识,提高临床诊断正确率,为准确治疗提供可能性。
基金This work was supported by the National Natural Science Foundation of China(NSFC)(No.81371256,81171061,81361128012)Ministry of Education of Republic of China(BIBD-PXM2013-014226-07-000084)Seed Grant of International Alliance of Translational Neuroscience(PXM2014-014226-000015).
文摘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.
基金a National Health and Medical Research Council Leadership Fellowship(1195830)has received research funding from The Michael J.Fox Foundation and the Australian Research Council.S.A.F.was supported by The Sartain Lanier Family Foundation.He has been a consultant for Lundbeck,Sunovion,Biogen,Impel,Acorda,and CereSpir.He has received education and research grants from Medtronic,Boston Scientific,Sun Pharmaceuticals Advanced Research Company,Biohaven,Impax,Lilly,US World Meds,Sunovion Therapeutics,Neurocrine,Vaccinex,Voyager,Jazz Pharmaceuticals,CHDI Foundation,The Michael J.Fox Foundation,National Institutes of Health(NIH),and Parkinson’s Foundation.He receives royalties from Demos,Blackwell Futura,Springer for textbooks,and UpToDate.Other support was from Signant(Bracket Global LLC)and CNS Ratings LLC.N.G.serves as consultant to Sionara,NeuroDerm,Pharma2B,Denali,Neuron23,Sanofi-Genzyme,Biogen,and AbbVie.He receives royalties from Lysosomal Therapeutics(LTI)and payment for lectures at AbbVie,Sanofi-Genzyme,and Movement Disorder Society.He received research support from The Michael J.Fox Foundation,the National Parkinson Foundation,European Union,and Israel Science Foundation,as well as from Teva NNE program,Biogen,and Ionis.He receives support from the Sieratzki Family Foundation and the Aufzien Academic Center in Tel-Aviv University.A.N.received funding from the European Commission,Research Foundation Flanders,King Baudouin Foundation,The Michael J.Fox Foundation,Jacques and Gloria Gossweiler Foundation,and KU Leuven Internal Research Funds.M.H.is an inventor of patents held by the NIH for an immunotoxin for the treatment of focal movement disorders and the H-coil for magnetic stimulationin relation to the latter,he has received license-fee payments from the NIH(from Brainsway).He is on the Medical Advisory Boards of CALA Health and Brainsway(both unpaid positions).He is on the editorial board of approximately 15 journals and receives royalties and/or honoraria from publishing from Cambridge University Press,Oxford University Press,Springer,Wiley,Wolters Kluwer,and Elsevier.He has research grants from Medtronic,Inc.for a study of deep brain stimulation for dystonia and CALA Health for studies of a device to suppress tremor.
文摘There has been a growing appreciation for freezing of gait as a disabling symptom that causes a significant burden in Parkinson’s disease. Previous research has highlighted some of the key components that underlie the phenomenon, but these reductionist approaches have yet to lead to a paradigm shift resulting in the development of novel treatment strategies. Addressing this issue will require greater integration of multi-modal data with complex computational modeling, but there are a number of critical aspects that need to be considered before embarking on such an approach. This paper highlights where the field needs to address current gaps and shortcomings including the standardization of definitions and measurement, phenomenology and pathophysiology, as well as considering what available data exist and how future studies should be constructed to achieve the greatest potential to better understand and treat this devastating symptom.
基金The work was supported by the National Natural Science Foundation of China(No.81200981,81,371,407)the Innovation Program of Shanghai Municipal Education Commission(No.13YZ026)the Intramural Program of the National Institute of Neurological Disorders and Stroke.
文摘Photophobia is a common symptom seen in many neurologic disorders,however,its pathophysiology remains unclear.Even the term is ambiguous.In this paper,we review the epidemiology and clinical manifestations of photophobia in neurological disorders,including primary headache,blepharospasm,progressive supranuclear palsy,and traumatic brain injury,discuss the definition,etiology and pathogenesis,and summarize practical methods of diagnosis and treatment.
基金the National Natural Science Foundation of China(81520108016,81661148045,and 31471084 to Yu-Feng Zang81671774 and 81630031 to Chao-Gan Yan+11 种基金81571228 to Tao Wu61571047 to Xia Wu81701664 to Jian Wang,81471654 to Biao Huang81701671 to Wei-Guo Liu82001898 to Xi-Ze Jia81771820,81371519 and 81571654 to Wei Luo)Henry G Leong Endowed Professorship in Neurology to Shu-Leong Ho and Shirley YY Pang,BRC for Mental Health at South London and Maudsley NHS Foundation Trust and by the Sackler Institute to Grainne McAlonan,NIH(2R01AG006457 to Fay B.Horak1RC4NS073008-01 and P50NS062684 to Tara Madhyastha)NINDS Intramural Research Program to Mark HallettStart-up Funds for Leading Talents at Beijing Normal UniversityNational Basic Science Data Center‘‘Chinese Data-sharing Warehouse for In-vivo Imaging Brain”(NBSDC-DB-15)to Xi-Nian ZuoGrant NU20-04-00294 of the Agency for Health Research,Czech Republic to Lenka Krajcovicova and Irena Rektorova。
文摘Thousands of resting state functional magnetic resonance imaging(RS-f MRI)articles have been published on brain disorders.For precise localization of abnormal brain activity,a voxel-level comparison is needed.Because of the large number of voxels in the brain,multiple comparison correction(MCC)must be performed to reduce false positive rates,and a smaller P value(usually including either liberal or stringent MCC)is widely recommended[1].
基金This research was supported by the Intramural Research Program of the National Institute of Neurological Disorders and Stroke,National Institutes of Health.
文摘Background:Focal dystonia is a neurological disorder characterized by unwanted muscle spasms.Blepharospasm is a focal dystonia producing an involuntary closure of the eyelid.Its etiology is unknown.Objective:To investigate if there are structural changes in the white and grey matter of blepharospasm patients,and if the changes are related to disease features.Methods:T1 and diffusion-weighted magnetic resonance imaging scans were collected from 14 female blepharospasm patients and 14 healthy matched controls.Grey matter volumes,fractional anisotropy(FA),and mean diffusivity maps were compared between the groups.Based on grey matter differences within the facial portion of the primary motor cortex,the corticobulbar tract was traced and compared between groups.Results:Changes in grey matter in patients included the facial portion of the sensorimotor area and anterior cingulate gyrus.These changes did not correlate with disease duration.Corticobulbar tract volume and peak tract connectivity were decreased in patients compared with controls.There were no significant differences in FA or mean diffusivity between groups.Conclusions:Grey matter changes within the primary sensorimotor and the anterior cingulate cortices in blepharospasm patients may help explain involuntary eyelid closure and the abnormal sensations often reported in this condition.
基金The authors would like to thank Robert Reed for reviewing the English grammar of this article and Mayra A.Gualdron for graphical design.Dr.Fidias E.Leon-Sarmiento was supported by the US National Institutes of Health(RO1DA019055-04)the Department of Defense(USAMRAA W81XWH-09-1-0467)+1 种基金the Michael J.Fox Foundation(Grant no.11805)Alexander Gonzalez-Castano,MSc.,was supported by the Corporacion Universitaria Minuto de Dios-UNIMINUTO.
文摘Te content of the rectifed motor evoked potential(MEP)induced by transcranial magnetic stimulation(TMS)has ambiguously been assessed without the precision that energy calculation deserves.Tis fact has misled data interpretation and misguided biomedical interventions.To defnitively fll the gap that exits in the neurophysics processing of these signals,we computed,in Walls(̂�),the bioenergy within the rectifed MEP recorded from the human frst digitorum index(FDI)muscle at rest and under isometric contraction.We also gauged the biowork exerted by this muscle.Here we show that bioenergy and biowork can accurately and successfully be assessed,validated,and determined in̂�from MEP signals induced by TMS,regardless of knowing the mathematical expression of the function of the signal.Our novel neurophysics approach represents a dramatic paradigm shif in analysis and interpretation of the content of the MEP and will give a true meaning to the content of rectifed signals.Importantly,this innovative approach allowed unveiling that women exerted more bioenergy than men at the magnetic stimulations used in this study.Revisitation of conclusions drawn from studies published elsewhere assessing rectifed EMG signals that have used ambiguous units is strongly recommended.
文摘 Prevention The best treatment is to prevent the illness in the first place or to retard its progression. There is considerable new information about Parkinson's disease genetics and pathophysiology that hopefully will lead to therapy in this regard, but there is still nothing definitive. The only medication for which there are some reasonable data is selegiline, a monoamine oxidase (MAO)-B inhibitor. This drug actually has a symptomatic effect since it prevents the breakdown ofdopamine in the brain. Some trials have shown a beneficial effect separate from its symptomatic effect. The situation is rather controversial, however, and in one study there was an excess of mortality. {4}There are no supportive data for antioxidants such as vitamin E, and a trial of the neurotrophic factor GDNF was not positive.
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文摘 The Movement Disorder Society (MDS) is the international not-for-profit organization representing and serving clinicians, other health professionals, researchers and policy makers interested in the area of movement disorders. The Society is represented in 68 countries by approximately 1,500 members. The Society has developed regional sections and welcomes affiliation of regional Movement Disorder groups.
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