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This monograph describes the progress in neuropathological HD research made during the last century, the neuropathological hallmarks of HD and their pathogenic relevance. Starting with the initial descriptions of the progressive degeneration of the striatum as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating neuropathological data with results on the functional neuroanatomy of the human brain, subsequent chapters will highlight recent HD findings: the neuronal loss in the cerebral neo-and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and nuclei, the involvement of select brainstem nuclei, as well as the pathophysiological relevance of these pathologies for the clinical picture of HD. Finally, the potential pathophysiological role of neuronal huntingtin aggregations and the most important and enduring challenges of neuropathological HD research are discussed.
This book constitutes the refereed proceedings of the 26th IFIP WG 6.1 International Conference on Formal Techniques for Networked and Distributed Systems, FORTE 2006, held in Paris, France, in September 2006. The 26 revised full papers and 4 short papers presented together with 3 invited lectures were carefully reviewed and selected from 177 submissions. The papers focus on the construction of middleware and services using formalised and verified approaches.
The cooperation test [Apt, Francez & de Roever] was originally conceived to capture the proof theoretical analogue of distributed message exchange between disjoint processes, as opposed to the interference freedom test [Owicki & Gries], being the proof theoretical analogue of concurrent communication by means of interference through jointly shared variables. Some authors ([Levin & Gries, Lamport & Schneider, Schlichting and Schneider]) stress that both forms of communication can be proof theoretically characterized using interference freedom only, since proofs for both ultimately amount to an invariance proof of a big global assertion [Ashcroft], invariance of whose parts amounts to interfer...
Data is at the center of many challenges in system design today. Difficult issues need to be figured out, such as scalability, consistency, reliability, efficiency, and maintainability. In addition, we have an overwhelming variety of tools, including relational databases, NoSQL datastores, stream or batch processors, and message brokers. What are the right choices for your application? How do you make sense of all these buzzwords? In this practical and comprehensive guide, author Martin Kleppmann helps you navigate this diverse landscape by examining the pros and cons of various technologies for processing and storing data. Software keeps changing, but the fundamental principles remain the s...