Congenital myopathies are a heterogeneous group of inherited muscle disorders, characterized by the predominance of particular histopathological features on muscle biopsy, such as cores (central core disease) or rods (nemaline myopathy). Clinically, early onset of the disease, stable or slowly progressive muscle weakness, hypotonia and delayed motor development are common in most forms. As a result, the diagnosis of a subtype of congenital myopathy is largely based on the presence of specific structural abnormalities in the skeletal muscle detected by enzyme-histochemistry and electron microscopy studies. During the last decades there have been significant advances in the identification of the genetic basis of most congenital myopathies. However, there is significant genetic heterogeneity within the main groups of congenital myopathies, and mutations in one particular gene may also cause diverse clinical and morphological phenotypes. Thus, the nosography and nosology in this field is still evolving.
The go-to intelligence expert and author of MI6 has “provided the clearest review of GCHQ and its predecessors in a publicly available book” (Firetrench). Signal intelligence is the most secret, and most misunderstood, weapon in the modern espionage arsenal. As a reliable source of information, it is unequalled, which is why Government Communications Headquarters, almost universally known as GCHQ, is several times larger than the two smaller, but more familiar, organizations, MI5 and MI6. Because of its extreme sensitivity, and the ease with which its methods can be compromised, GCHQ’s activities remain cloaked in secrecy. In GCHQ: The Secret Wireless War 1900-1986, the renowned expert Nigel West traces GCHQ’s origins back to the early days of wireless and gives a detailed account of its development since that time. From the moment that Marconi succeeded in transmitting a radio signal across the Channel, Britain has been engaged in a secret wireless war, first against the Kaiser, then Hitler and the Soviet Union. Following painstaking research, Nigel West is able to describe all GCHQ’s disciplines, including direction-finding, interception and traffic analysis, and code-breaking. Also explained is the work of several lesser known units such as the wartime Special Wireless Groups and the top-secret Radio Security Service. Laced with some truly remarkable anecdotes, this edition of this important book will intrigue historians, intelligence professionals and general readers alike. “Nigel West is an acknowledged expert in this field of literature and his latest book is fascinating and intriguing.” —Books Monthly “Rich in the kind of detail from which all students of radio and military history can learn.” —The Spectrum Monitor
J. Frank Adams had a profound influence on algebraic topology, and his works continue to shape its development. The International Symposium on Algebraic Topology held in Manchester during July 1990 was dedicated to his memory, and virtually all of the world's leading experts took part. This two volume work constitutes the proceedings of the symposium; the articles contained here range from overviews to reports of work still in progress, as well as a survey and complete bibliography of Adams' own work. These proceedings form an important compendium of current research in algebraic topology, and.
The Historical Dictionary of Naval Intelligence relates the long and fascinating history of naval intelligence through a chronology, an introduction, a bibliography, and hundreds of cross-referenced dictionary entries on the organizations, operations, and events that made Naval intelligence what it is today.
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