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Handbook of Clinical Neurology

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https://www.readbyqxmd.com/read/29903454/physiology-of-the-cerebellum
#1
Egidio D'Angelo
The cerebellum is a central brain structure deeply integrated into major loops with the cerebral cortex, brainstem, and spinal cord. The cerebellum shows a complex regional organization consisting of modules with sagittal orientation. The cerebellum takes part in motor control and its lesions cause a movement incoordination syndrome called ataxia. Recent observations also imply involvement of the cerebellum in cognition and executive control, with an impact on pathologies like dyslexia and autism. The cerebellum operates as a forward controller learning to predict the precise timing of correlated events...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903453/topography-of-the-cerebellum-in-relation-to-social-brain-regions-and-emotions
#2
Maria Leggio, Giusy Olivito
In the last few decades, an increasing number of studies have focused on better characterizing the cerebellar functions beyond motor control, including emotional and social domains. Anatomic and functional evidence strongly contributes to delineating the cerebellar functional subdivisions and their integration with cerebral functional networks strictly related to emotional regulation and social functioning, thus suggesting a model of cerebellar organization that resembles that of the cerebral cortex. Overcoming the traditional segregation of cerebrocerebellar networks in sensorimotor/cognitive functional modules, during emotional/social processes, the cerebellar activity reflects a domain-specific mentalizing functionality that is strongly connected with corresponding mentalizing networks in the cerebrum...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903452/functional-topography-of-the-human-cerebellum
#3
Catherine J Stoodley, Jeremy D Schmahmann
Accumulating evidence points to a critical role for the human cerebellum in both motor and nonmotor behaviors. A core tenet of this new understanding of cerebellar function is the existence of functional subregions within the cerebellum that differentially support motor, cognitive, and affective behaviors. This cerebellar functional topography - based on converging evidence from neuroanatomic, neuroimaging, and clinical studies - is evident in both adult and pediatric populations. The sensorimotor homunculi in the anterior lobe and lobule VIII established in early tract tracing and electrophysiologic studies are evident in both task-based and resting-state human functional imaging studies...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903451/principles-of-organization-of-the-human-cerebellum-macro-and-microanatomy
#4
Reiko Ashida, Nadia L Cerminara, Jon Brooks, Richard Apps
In this chapter, we compare current understanding of the anatomy and functional compartmentation of the human cerebellum with detailed knowledge in nonhuman species. The anatomy of the cerebellum is highly conserved across mammals and comparison of functional data suggests that similar principles of organization also hold true for somatotopy. In particular, there is a dual representation of the limbs in the cerebellar cortex in rat, ferret, cat, monkey, and human. In animals, a key organizing principle of the cerebellum is its division into a series of longitudinally oriented olivocorticonuclear modules that are narrow in the mediolateral axis but extend across multiple cerebellar lobules in the rostrocaudal plane...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903450/scales-for-the-clinical-evaluation-of-cerebellar-disorders
#5
Katrin Bürk, Deborah A Sival
Clinical scales represent an important tool not only for the initial grading/scoring of disease and assessment of progression, but also for the quantification of therapeutic effects in clinical trials. There are several scales available for the clinical evaluation of cerebellar symptoms. While some scales have been developed and evaluated for specific cerebellar disorders such as Friedreich ataxia, others reliably capture cerebellar symptoms with no respect to the underlying etiology. Each scale has its strengths and weaknesses...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903449/neuro-ophthalmologic-assessment-and-investigations-in-children-and-adults-with-cerebellar-diseases
#6
Alexander A Tarnutzer, Dominik Straumann, Michael S Salman
In this chapter, we present the bedside assessment and laboratory tests that are available for the neuro-ophthalmologic evaluation of children and adults with cerebellar diseases. In the evaluation of a patient with cerebellar dysfunction, recognizing the pattern of ocular motor and / or vestibular impairment is often a key step to the correct diagnosis. The cerebellum is very important in processing a wide range of different eye movements, including angular vestibulo-ocular reflexes, otolith-ocular reflexes, fixation and gaze holding, smooth pursuit eye movements, saccadic eye movements, optokinetic response, ocular alignment, and vergence...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903448/historic-notes-on-anatomic-physiologic-and-clinical-research-on-the-cerebellum
#7
Jan Voogd, Peter J Koehler
This chapter is concerned with ideas on the function, structure, and pathology that shaped our present knowledge of the cerebellum. One of the main themes in its early history is its localization subtentorially, leading to misattributions due to clinical observations in trauma and lesion experiments that caused collateral damage to the brainstem. Improvement of techniques led to the insight that it plays a role in movement control (Rolando) or coordination (Flourens). Purkinje initiated the histology of the cerebellar cortex in 1837...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903447/neurophysiology-of-gait
#8
Mariano Serrao, Alberto Ranavolo, Carlo Casali
Beyond the classic clinical description, recent studies have quantitatively evaluated gait and balance dysfunction in cerebellar ataxias by means of modern motion analysis systems. These systems have the aim of clearly and quantitatively describing the differences, with respect to healthy subjects, in kinematic, kinetic, and surface electromyography variables, establishing the basis for a rehabilitation strategy and assessing its efficacy. The main findings which characterize the gait pattern of cerebellar patients are: increased step width, reduced ankle joint range of motion with increased coactivation of the antagonist muscles, and increased stride-to-stride variability...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903446/embryology
#9
Parthiv Haldipur, Derek Dang, Kathleen J Millen
With the growing recognition of the extent and prevalence of human cerebellar disorders, an understanding of developmental programs that build the mature cerebellum is necessary. In this chapter we present an overview of the basic epochs and key molecular regulators of the developmental programs of cerebellar development. These include early patterning of the cerebellar territory, the genesis of cerebellar cells from multiple spatially distinct germinal zones, and the extensive migration and coordinated cellular rearrangements that result in the formation of the exquisitely foliated and laminated mature cerebellum...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903445/laboratory-investigations
#10
Eugen Boltshauser, Konrad P Weber
This chapter deals with chemical and hematologic investigations which are often considered in the diagnostic workup of subacute to chronic cerebellar ataxias. Relevant investigations in blood (serum, plasma), urine, and cerebrospinal fluid are discussed. Particular attention is paid to early diagnosis of treatable metabolic ataxias (such as abetalipoproteinemia, coenzyme Q10 deficiency, cerebrotendinous xanthomatosis, glucose transporter type 1 deficiency, Refsum disease, and vitamin E deficiency), but autoimmune ataxias, other vitamin deficiencies, and endocrine disorders should also be kept in mind...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903444/genetics-of-cerebellar-disorders
#11
Enza Maria Valente, Sara Nuovo, Dan Doherty
The approach to identifying a genetic cause in patients with cerebellar disorders relies on history, examination, consultation, and testing, combined with specialized expertise because they are rare and genetically diverse. Cerebellar disorders can be caused by a variety of DNA alterations including single-nucleotide changes, small insertions or deletions, larger copy number variants, and nucleotide repeat expansions, exhibiting autosomal-recessive, autosomal-dominant (inherited and de novo), X-linked, and mitochondrial modes of inheritance...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903443/nuclear-medicine-of-the-cerebellum
#12
Alessandra Vella, Mario Mascalchi
Single-photon emission computed tomography (SPECT) and positron emission tomography (PET) with different radiotracers enable regional evaluation of blood flow and glucose metabolism, of receptors and transporters of several molecules, and of abnormal deposition of peptides and proteins in the brain. The cerebellum has been used as a reference region for different radiotracers in several disease conditions. Whole-brain voxel-wise analysis is not affected by a priori knowledge bias and should be preferred. SPECT and PET have contributed to establishing the cerebellum role in motion, cognition, and emotion control in physiologic and pathophysiologic conditions...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903442/probing-the-neuroanatomy-of-the-cerebellum-using-tractography
#13
Christophe Habas, Mario Manto
Diffusion tensor imaging (DTI) is a noninvasive neuroimaging tool assessing the organization of white-matter tracts and brain microstructure in vivo. The technique takes into account the three-dimensional (3D) direction of diffusion of water in space, the brownian movements of water being constrained by the brain microstructure. The main direction of diffusion in the brain is extracted to obtain the principal direction of axonal projection within a given voxel. Overall, the diffusion tensor is a mathematic analysis of the magnitude/directionality (anisotropy) of the movement of water molecules in 3D space...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903441/conventional-mri
#14
Filippo Arrigoni, Sonia Calloni, Thierry A G M Huisman, Luisa Chiapparini
Conventional magnetic resonance imaging (MRI) allows for a detailed noninvasive visualization/examination of posterior fossa structures and represents a fundamental step in the diagnostic workup of many cerebellar disorders. In the first part of this chapter methodologic issues, like the correct choice of hardware (magnets, coils), pro and cons of the different MRI sequences, and patient management during the examination are discussed. In the second part, the MRI anatomy of the cerebellum, as noted on the various conventional MRI sequences, as well as a detailed description of cerebellar maturational processes from birth to childhood and into adulthood, are reported...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903440/ultrasound-and-ct-of-the-posterior-fossa-in-neonates
#15
Elizabeth Snyder, Misun Hwang, Bruno P Soares, Aylin Tekes
Ultrasound, CT and MRI may all be used in the evaluation of the posterior fossa in neonates depending on the clinical scenario. Ultrasonography is particularly valuable for the evaluation of the neonatal brain because of the lack of ionizing radiation and the ability to perform exams at the bedside and, importantly, advancements in ultrasound technology now allow for diagnostic-quality imaging. While CT is still the initial imaging modality of choice in most neurologic emergencies, in the neonate, ultrasound is the first line in nontraumatic emergencies...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903439/language-and-the-cerebellum
#16
Peter Mariën, Renato Borgatti
During the past decades neuroanatomic, neuroimaging, and clinical studies have substantially changed the long-standing view of the role of the cerebellum as a sole coordinator of sensorimotor function. Currently, the cerebellum is considered to be crucially implicated in a variety of cognitive, affective, social, and behavioral processes as well. In this chapter we aim to summarize a number of critical insights from different research areas (neuroanatomy, functional neuroimaging, clinical practice) that provide evidence for a role of the cerebellum in motor speech and nonmotor language processing in both adults and children...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903438/cognitive-aspects-sequencing-behavior-and-executive-functions
#17
Marco Molinari, Marcella Masciullo, Sara Bulgheroni, Stefano D'Arrigo, Daria Riva
The question posed today is not whether the cerebellum plays a role in cognition, but instead, how the cerebellum contributes to cognitive processes, even in the developmental age. The central role of the cerebellum in many areas of human abilities, motor as well as cognitive, in childhood as well as in adulthood, is well established but cerebellar basic functioning is still not clear and is much debated. Of particular interest is the changing face of cerebellar influence on motor, higher cognitive, and behavioral functioning when adult and developmental lesions are compared...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903437/cerebellar-motor-syndrome-from-children-to-the-elderly
#18
Mario Manto
More than a century after the description of its cardinal components, the cerebellar motor syndrome (CMS) remains a cornerstone of daily clinical ataxiology, in both children and adults. Anatomically, motor cerebellum involves lobules I-V, VI, and VIII. CMS is typically associated with errors in the metrics of voluntary movements and a lack of coordination. Symptoms and motor signs consist of speech deficits, impairments of limb movements, and abnormalities of posture/gait. Ataxic dysarthria has a typical scanning (explosive with staccato) feature, voice has a nasal character, and speech is slurred...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903436/the-neuropathology-of-the-adult-cerebellum
#19
Arnulf H Koeppen
This chapter summarizes the neuropathologic features of nonneoplastic disorders of the adult cerebellum. Gait ataxia and extremity dysmetria are clinical manifestations of diseases that interrupt the complex cerebellar circuitry between the neurons of the cerebellar cortex, the cerebellar nuclei (especially the dentate nuclei), and the inferior olivary nuclei. The cerebellum is a prominent target of several sporadic and hereditary neurodegenerative diseases, including multiple system atrophy, spinocerebellar ataxia, and Friedreich ataxia...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29903435/cerebellar-networks-and-neuropathology-of-cerebellar-developmental-disorders
#20
Harvey B Sarnat
The cerebellar system is a series of axonal projections and synaptic circuits as networks, similar to those of the limbic system and those subserving the propagation and spread of seizures. Three principal cerebellar networks are identified and cerebellar disease often affects components of the networks other than just the cerebellar cortex. Contemporary developmental neuropathology of the cerebellum is best considered in the context of alterations of developmental processes: embryonic segmentation and genetic gradients along the three axes of the neural tube, individual neuronal and glial cell differentiation, migration, synaptogenesis, and myelination...
2018: Handbook of Clinical Neurology
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