Titlebook: Neurodegenerative Diseases; Shamim I. Ahmad Book 2012 The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer

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Epilepsy and Epileptic Syndrome,ficant number of patients, especially in the setting of medically-intractable epilepsies, may experience different degrees of memory or cognitive impairment, behavioral abnormalities or psychiatric symptoms, which may limit their daily functioning. As a result, in many patients, epilepsy may resembl

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Frontotemporal Lobar Degeneration,ed with degeneration of the frontal and temporal lobes. Three different clinical presentations have been described, namely behavioural variant Frontotemporal Dementia (bvFTD), Semantic Dementia (SD) and Progressive Non-Fluent Aphasia (PNFA). The associated histopathology includes different neuropath

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,Gerstmann-Str?ussler-Scheinker Disease,ongiform encephalopathy (TSE) in which a mutation in a gene encoding for prion protein (PrP) was discovered. The first “H” family had been known by the Viennese neuropsychiatrists since the XXth century and was reported by Gerstmann, Str?ussler and Scheinker in 1936. In this chapter we present the c

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Kuru: The First Prion Disease, to chimpanzees and subsequently classified as a transmissible spongiform encephalopathy (TSE), or slow unconventional virus disease. It was first reported to Western world in 1957 by Gajdusek and Vincent Zigas,. and in 1975 a complete bibliography of kuru was published by Alpers et al.. “Kuru” in t

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Leukodystrophies,malcy with a variety of neurologic problems. Though the ultimate diagnosis is not found in many patients with leukodystrophies, distinctive features unique to them aid in diagnosis, treatment and prognostication. The clinical characteristics, etiologies, diagnostic testing and treatment options are

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Machado-Joseph Disease and other Rare Spinocerebellar Ataxias,disability. SCAs show high clinical, genetic, molecular and epidemiological variability. In the last one decade, the intensive scientific research devoted to the SCAs is resulting in clear advances and a better understanding on the genetic and nongenetic factors contributing to their pathogenesis wh

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