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  • 1
    Online Resource
    Online Resource
    Trials for Cerebellar Ataxias : From Cellular Models to Human Therapies (2023)
    Cham :Springer International Publishing :
    Details
    Keywords: Neurosciences. ; Neurology . ; Pharmaceutical chemistry. ; Cognitive neuroscience. ; Neuroscience. ; Neurology. ; Pharmaceutics. ; Cognitive Neuroscience.
    Description / Table of Contents: Part I: Basic Science of Cerebellum and Ataxias -- Functional anatomy of the cerebellum -- Cerebellar physiology -- Cerebellar biochemistry/pharmacology -- Genetics of dominant ataxias -- Autosomal and X-linked degenerative ataxias: from genetics to promising therapeutics -- Seeking therapies for Spinocerebellar Ataxia: from gene silencing to systems-based approaches -- Ion Channel Genes and Ataxia -- Part II: Biomarkers and tools of trials -- How to Design a Therapeutic Trial in SCAs -- Therapy development for spinocerebellar ataxia: Rating Scales and Biomarkers -- Clinical rating scales for ataxia -- Scale for Ocular motor Disorders in Ataxia (SODA): Procedures and Basic Understanding -- Cerebellar learning in the prism adaptation task -- Blood and CSF biomarkers in autosomal dominant cerebellar ataxias -- Part III: Autosomal Dominant Cerebellar Ataxias -- Riluzole in progressive cerebellar ataxias -- ASOs against ATXN2 in preclinical and phase 1 trials -- Antisense oligonucleotide therapy against SCA3 -- Spinocerebellar ataxia type 7: From mechanistic pathways to therapeutic opportunities -- Experimental neurotransplantation for cerebellar ataxias -- Development of mesenchymal stem cells therapy for the treatment of polyglutamine SCA: from bench to bedside -- Cerebello-Spinal tDCS as Rehabilitative Intervention in Neurodegenerative Ataxia -- Cerebellar Transcranial Magnetic Stimulation in Cerebellar Ataxias -- Physical therapy in Cerebellar Ataxia -- Part IV: Autosomal Recessive Cerebellar Ataxias -- Recent advances on therapeutic approaches for Friedreich’s Ataxia -- Therapeutic use of interferon gamma in Friedreich ataxia -- Metabolic treatments of cerebellar ataxia -- Clinical Trials in Fragile X-Associated Tremor/Ataxia Syndrome -- Part V: Sporadic Ataxias -- Therapeutic strategies in immune-mediated cerebellar ataxias -- Coenzyme Q10 in MSA -- State of the Art and History of Therapeutics in Ataxias.
    Abstract: Despite the critical importance of the cerebellum in brain functions, the scientific community still lacks effective treatments for most cerebellar ataxias. This book provides a link between the pathogenesis and therapies of cerebellar ataxias while also providing a comprehensive assessment of the preclinical and clinical trials dedicated to cerebellar ataxias over the past 20 years of progress. This is the first book fully dedicated to the trials and therapies of these disorders. It is a truly authoritative and comprehensive reference and comes at a time of major advances in genetic tools and neuroimaging assessments. The coverage begins by laying a foundation of the basic science of the cerebellum and ataxias, proceeds to discuss biomarkers and the tools of trials, offers guidelines on conducting trials, and then explores the full range of therapeutics and their trials, including gene therapy and cell transplantation. The authors are top experts on cerebellar research and the contributing authors have all made seminal contributions in the field.
    Type of Medium: Online Resource
    Pages: XI, 727 p. 1 illus. , online resource.
    Edition: 1st ed. 2023.
    ISBN: 9783031243455
    Series Statement: Contemporary Clinical Neuroscience,
    URL: https://doi.org/10.1007/978-3-031-24345-5
    DOI: 10.1007/978-3-031-24345-5
    DDC: 612.8
    Language: English
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  • 2
    Online Resource
    Online Resource
    Mechanisms and Emerging Therapies in Tremor Disorders (2023)
    Cham :Springer International Publishing :
    Details
    Keywords: Medicine Research. ; Biology Research. ; Neurology . ; Neurosciences. ; Nervous system Diseases. ; Biomedical Research. ; Neurology. ; Neuroscience. ; Neurological Disorders.
    Description / Table of Contents: PART I: Fundamental Aspects -- Chapter 1. Definition of Tremor -- Chapter 2. Membrane Mechanisms of Tremor -- Chapter 3 Advances in the Genetics of Human Tremor -- Chapter 4. Two origins of tremors related to the Guillain-Mollaret triangle: the forward model-related tremor and the inferior olive oscillation-related tremor -- PART II:Tremor in Clinical Practice -- Chapter 5. Physiologic Tremor -- Chapter 6. Rest Tremor -- Chapter 7. Postural Tremors -- Chapter 8. Isometric Tremor -- Chapter 9. Essential Tremor and Other Forms of Kinetic Tremor -- Chapter 10. Cerebellar lesions and tremor -- Chapter 11. Orthostatic Tremor -- Chapter 12. Posttraumatic Tremor and Other Posttraumatic Movement Disorders -- Chapter 13 Tremor in Childhood -- Chapter 14. Metabolic Causes of Tremor -- Chapter 15. Drug-Induced Tremor -- Chapter 16. Tremor: The Clinical Approach to Reach the Diagnosis -- PART III: Assessment of tremor -- Chapter 17. Signal Processing -- Chapter 18. Diffusion imaging in Tremor -- Chapter 19. The role of the noradrenergic system in tremor pathogenesis -- Chapter 20. Metabolic Networks in Parkinson’s Disease -- PART IV: Therapies -- Chapter 21. Deep brain stimulation for tremor -- Chapter 22 Mechatronic Devices for Upper Limb Tremor.
    Abstract: Tremor is intimately linked to the numerous interactions of the central and peripheral nervous system components tuning motor control, from the cerebral cortex to the peripheral effectors. Activities of central generators, reflex loop delays, inertia, stiffness, and damping are all factors that influence the features of tremor. This completely updated new edition discusses the pathophysiology of tremor, including membrane mechanisms and rodent models, the advances in genetics, and the musculoskeletal models pertinent to body oscillations. The main forms of tremor encountered during clinical practice are considered, taking into account neuroimaging aspects. The book covers recent advances in methodologies and techniques of assessment and provides practical information for daily management. This new edition is informed by the guidelines of the Tremor Task Force of the International Parkinson and Movement Disorders Society. New chapters include Classification of Tremors, Medically Induced Tremors, Resting State fMRI, and Gabaergic Pathways. In addition to pharmacological treatments, neurosurgical approaches such as deep brain stimulation (DBS) and thalamotomy are discussed. Emerging techniques under development are also introduced.
    Type of Medium: Online Resource
    Pages: X, 558 p. 86 illus., 59 illus. in color. , online resource.
    Edition: 2nd ed. 2023.
    ISBN: 9783031261282
    Series Statement: Contemporary Clinical Neuroscience,
    URL: https://doi.org/10.1007/978-3-031-26128-2
    DOI: 10.1007/978-3-031-26128-2
    DDC: 610.72
    Language: English
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  • 3
    Online Resource
    Online Resource
    Essentials of Cerebellum and Cerebellar Disorders : A Primer For Graduate Students (2023)
    Cham :Springer International Publishing :
    Details
    Keywords: Neurosciences. ; Neuroanatomy. ; Neurophysiology. ; Developmental neurobiology. ; Cognitive neuroscience. ; Neuroscience. ; Neuroanatomy. ; Neurophysiology. ; Development of the Nervous System. ; Cognitive Neuroscience.
    Abstract: This monograph, now in its 2nd edition with 31 new chapters and significant updates, is the first book of its kind written specifically for graduate students and clinicians. The monograph is based on the 4-volume treatise, Handbook of the Cerebellum and Cerebellar Disorders (Springer, 2013; 2nd edition: 2022), the definitive reference for scientists and neurologists in the field of cerebellar neurobiology and related areas. There have been fundamental advances in the basic science and clinical neurology of the cerebellum and its role in sensorimotor function and cognition. Essentials of the Cerebellum and Cerebellar Disorders makes this large and expanding body of knowledge readily accessible to trainees and clinicians alike. It is organized into easy to read and short chapters that are ideal for students and clinicians. The most common cerebellar disorders encountered in the clinic are covered. The editors are world leaders in the field, and the chapters are authored by an international panel of experts drawn from cerebellar laboratories and ataxia clinics throughout North America, Europe and Asia. Essentials provides a solid grounding in the field of cerebellar research and ataxiology from cerebellar cellular biology and circuity to clinical practice, and it serves as a springboard to a deeper appreciation of both the principles and the complexities of cerebellar neurobiology. Clinicians are expected to have a deep appreciation of cerebellar disorders, not only in specialized ataxia clinics but also in adult and pediatric neurology, neurosurgery, psychiatry and neuropsychology practices, and in outpatient and inpatient rehabilitation settings. This book is an indispensable resource for students and practitioners navigating the evolving field of cerebellar motor and cognitive neurology. It also links to the more expansive Handbook for those who need to explore the topics in this monograph in greater depth.
    Type of Medium: Online Resource
    Pages: XIV, 742 p. 182 illus., 127 illus. in color. , online resource.
    Edition: 2nd ed. 2023.
    ISBN: 9783031150708
    URL: https://doi.org/10.1007/978-3-031-15070-8
    DOI: 10.1007/978-3-031-15070-8
    DDC: 612.8
    Language: English
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  • 4
    Online Resource
    Online Resource
    The Emotional Cerebellum (2022)
    Cham :Springer International Publishing :
    Details
    Keywords: Neurosciences. ; Emotions. ; Neuropsychology. ; Neuroscience. ; Emotion. ; Neuropsychology.
    Description / Table of Contents: Part I. Basic Principles of Cerebellar Function in Emotion -- Chapter 1. Introduction into the Role of the Cerebellum in Emotion -- Chapter 2. Principles of Brain and Emotion: Beyond the Cortico-Centric Bias -- Chapter 3. Cerebellum and Emotion Processing -- Chapter 4. Cerebellum and Emotion Recognition -- Chapter 5. Cerebellum and Emotion Memory -- Part II. Cells and Molecules of Emotions in the Cerebellum -- Chapter 6. Topography of Emotions in Cerebellum as Appraised by Functional Imaging -- Chapter 7. The Neurophysiology of the Cerebellum in Emotion -- Chapter 8. Non-invasive Brain Stimulation of the Cerebellum in Emotion -- Part III. Cerebellum and Emotion in High Order Domains -- Chapter 9. Reward-Based Learning and Emotional Habit Formation in the Cerebellum -- Chapter 10. Cerebellar Contribution to Emotional Body Language Perception -- Chapter 11. Influence of Pain on Cognitive Dysfunction and Emotion Dysregulation in Chiari Malformation Type I -- Chapter 12. Cerebellum and Emotion in Morality -- Chapter 13. Music and the Cerebellum -- Chapter 14. The Cerebellum and Beauty: The Impact of the Cerebellum in Art Experience and Creativity -- Chapter 15. Cerebellum and Emotion in Social Behavior -- Chapter 16. Cerebellum, Embodied Emotions, and Psychological Traits -- Part IV. Cerebellum and Emotion Disorders -- Chapter 17. The Cerebellum and Disorders of Emotion -- Chapter 18. Cerebellum and Neurorehabilitation in Emotion with a Focus on Neuromodulation -- Part V. Perspectives -- Chapter 19. Current and Future Perspectives of the Cerebellum in Affective Neurosciences.
    Abstract: Emotions represent a critical aspect of daily life in humans. Our understanding of the mechanisms of regulation of emotions has increased exponentially these last two decades. This book evaluates the contribution of the cerebellum to emotion. It outlines the current clinical, imaging and neurophysiological findings on the role of the cerebellum in key aspects of emotional processing and its influence on motor and cognitive function and social behavior. In the first section, the reader is introduced to the contributions of the cerebellum to various emotion domains, from emotion perception and recognition to transmission and encoding. Subsequent chapters provide a comprehensive picture of the neurophysiology and topography of emotion in the cerebellum and illustrate the convergence of theoretical and empirical research. Additional chapters address the cerebellum's involvement in emotional learning, emotional pain, emotional aspects of body language and perception, and its relations to social cognition including morality, music, and art. Finally, neuropsychiatric aspects of the cerebellum's influence on mood disorders and the current state of therapeutic options, including noninvasive stimulation approaches, complete the overview. This is the first book summarizing the current state of knowledge on the contribution of the cerebellum to important aspects of emotion. It is an essential reference for students, trainees, neuroscientists, researchers, and clinicians in neuroscience, neurology, neurosurgery and psychology involved in the study of emotions. The authors are renowned scientists in the field of cerebellar research.
    Type of Medium: Online Resource
    Pages: XII, 313 p. 42 illus., 36 illus. in color. , online resource.
    Edition: 1st ed. 2022.
    ISBN: 9783030995508
    Series Statement: Advances in Experimental Medicine and Biology, 1378
    URL: https://doi.org/10.1007/978-3-030-99550-8
    DOI: 10.1007/978-3-030-99550-8
    DDC: 612.8
    Language: English
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  • 5
    Online Resource
    Online Resource
    New Rapid-acting Antidepressants (2021)
    Cham :Springer International Publishing :
    Details
    Keywords: Neurosciences. ; Neuroscience.
    Description / Table of Contents: (R)-ketamine: A new rapid-acting antidepressant -- (2R,6R)-hydroxynorketamine, a metabolite of ketamine: the antidepressant actions and the mechanisms -- Predictable biomarkers for rapid-acting antidepressant response to ketamine -- Nitrous Oxide: an old compound with emerging psychotropic properties -- Novel AMPA receptor potentiators TAK-137 and TAK-653 as potential rapid-acting antidepressants -- AMPA receptor potentiators as potential rapid-acting antidepressants -- mGlu2/3 receptor antagonists as rapid-acting antidepressants -- Antidepressant effects of the muscarinic receptor antagonist scopolamine: Clinical and preclinical review.
    Abstract: This book discusses new candidates for rapid-acting antidepressants, such as (R)-ketamine, (2R,6R)-hydroxynorketamine, scopolamine, mGluR2/3 antagonists and AMPA receptor agonists. There are serious limitations to currently available antidepressants, such as delayed onset and low rates of efficacy. The discovery that a single dose of ketamine, an NMDAR antagonist, can produce rapid antidepressant effects that are sustained has led to new research in this area. In this volume, a variety of novel pharmaceutical treatments are examined. This volume would be useful to both researchers and clinicians who work in the field of pharmacology, specifically CNS drug treatments. Discusses new candidate for rapid-acting antidepressants Provides information for those who work in the field of CNS drugs Ideal for pharmacologists, psychiatrists, and those who want to learn about CNS drugs at the graduate level.
    Type of Medium: Online Resource
    Pages: VII, 149 p. 14 illus., 9 illus. in color. , online resource.
    Edition: 1st ed. 2021.
    ISBN: 9783030797904
    Series Statement: Contemporary Clinical Neuroscience,
    URL: https://doi.org/10.1007/978-3-030-79790-4
    DOI: 10.1007/978-3-030-79790-4
    DDC: 612.8
    Language: English
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  • 6
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    Evaluation of a wearable orthosis and an associated algorithm for tremor suppression (2007)
    Institute of Physics
    Details
    Publication Date: 2007-03-20
    Print ISSN: 0967-3334
    Electronic ISSN: 1361-6579
    Topics: Medicine , Physics
    Published by Institute of Physics
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  • 7
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    Celsr3 is required in motor neurons to steer their axons in the hindlimb (2014)
    Springer Nature
    Details
    Publication Date: 2014-08-10
    Print ISSN: 1097-6256
    Electronic ISSN: 1546-1726
    Topics: Biology , Medicine
    Published by Springer Nature
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  • 8
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    Dysmetria and Errors in Predictions: The Role of Internal Forward Model (2020)
    Molecular Diversity Preservation International
    Details
    Publication Date: 2020-09-20
    Description: The terminology of cerebellar dysmetria embraces a ubiquitous symptom in motor deficits, oculomotor symptoms, and cognitive/emotional symptoms occurring in cerebellar ataxias. Patients with episodic ataxia exhibit recurrent episodes of ataxia, including motor dysmetria. Despite the consensus that cerebellar dysmetria is a cardinal symptom, there is still no agreement on its pathophysiological mechanisms to date since its first clinical description by Babinski. We argue that impairment in the predictive computation for voluntary movements explains a range of characteristics accompanied by dysmetria. Within this framework, the cerebellum acquires and maintains an internal forward model, which predicts current and future states of the body by integrating an estimate of the previous state and a given efference copy of motor commands. Two of our recent studies experimentally support the internal-forward-model hypothesis of the cerebellar circuitry. First, the cerebellar outputs (firing rates of dentate nucleus cells) contain predictive information for the future cerebellar inputs (firing rates of mossy fibers). Second, a component of movement kinematics is predictive for target motions in control subjects. In cerebellar patients, the predictive component lags behind a target motion and is compensated with a feedback component. Furthermore, a clinical analysis has examined kinematic and electromyography (EMG) features using a task of elbow flexion goal-directed movements, which mimics the finger-to-nose test. Consistent with the hypothesis of the internal forward model, the predictive activations in the triceps muscles are impaired, and the impaired predictive activations result in hypermetria (overshoot). Dysmetria stems from deficits in the predictive computation of the internal forward model in the cerebellum. Errors in this fundamental mechanism result in undershoot (hypometria) and overshoot during voluntary motor actions. The predictive computation of the forward model affords error-based motor learning, coordination of multiple degrees of freedom, and adequate timing of muscle activities. Both the timing and synergy theory fit with the internal forward model, microzones being the elemental computational unit, and the anatomical organization of converging inputs to the Purkinje neurons providing them the unique property of a perceptron in the brain. We propose that motor dysmetria observed in attacks of ataxia occurs as a result of impaired predictive computation of the internal forward model in the cerebellum.
    Print ISSN: 1661-6596
    Electronic ISSN: 1422-0067
    Topics: Chemistry and Pharmacology
    Published by Molecular Diversity Preservation International
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  • 9
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    Abnormal Copper Homeostasis: Mechanisms and Roles in Neurodegeneration (2014)
    Molecular Diversity Preservation International
    Details
    Publication Date: 2014-06-18
    Electronic ISSN: 2305-6304
    Topics: Chemistry and Pharmacology
    Published by Molecular Diversity Preservation International
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  • 10
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    Fundamental Mechanisms of Autoantibody-Induced Impairments on Ion Channels and Synapses in Immune-Mediated Cerebellar Ataxias (2020)
    Molecular Diversity Preservation International
    Details
    Publication Date: 2020-07-13
    Description: In the last years, different kinds of limbic encephalitis associated with autoantibodies against ion channels and synaptic receptors have been described. Many studies have demonstrated that such autoantibodies induce channel or receptor dysfunction. The same mechanism is discussed in immune-mediated cerebellar ataxias (IMCAs), but the pathogenesis has been less investigated. The aim of the present review is to evaluate what kind of cerebellar ion channels, their related proteins, and the synaptic machinery proteins that are preferably impaired by autoantibodies so as to develop cerebellar ataxias (CAs). The cerebellum predictively coordinates motor and cognitive functions through a continuous update of an internal model. These controls are relayed by cerebellum-specific functions such as precise neuronal discharges with potassium channels, synaptic plasticity through calcium signaling pathways coupled with voltage-gated calcium channels (VGCC) and metabotropic glutamate receptors 1 (mGluR1), a synaptic organization with glutamate receptor delta (GluRδ), and output signal formation through chained GABAergic neurons. Consistently, the association of CAs with anti-potassium channel-related proteins, anti-VGCC, anti-mGluR1, and GluRδ, and anti-glutamate decarboxylase 65 antibodies is observed in IMCAs. Despite ample distributions of AMPA and GABA receptors, however, CAs are rare in conditions with autoantibodies against these receptors. Notably, when the autoantibodies impair synaptic transmission, the autoimmune targets are commonly classified into three categories: release machinery proteins, synaptic adhesion molecules, and receptors. This physiopathological categorization impacts on both our understanding of the pathophysiology and clinical prognosis.
    Print ISSN: 1661-6596
    Electronic ISSN: 1422-0067
    Topics: Chemistry and Pharmacology
    Published by Molecular Diversity Preservation International
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