Download e-book for kindle: Amyotrophic Lateral Sclerosis and the Frontotemporal by Michael J. Strong

By Michael J. Strong

ISBN-10: 0199590672

ISBN-13: 9780199590674

During the last ten years, there was an expanding popularity that syndromes of frontotemporal disorder (FTD) are a standard prevalence in sufferers with amyotrophic lateral sclerosis (ALS). Such syndromes should be found in as many as 60% of sufferers with ALS. Conversely, the prevalence of motor neuron disorder in sufferers with clinically natural frontotemporal dementia is more and more well-known. this implies that to some degree there are overlapping syndromes during which either ALS and FTD happen in the comparable person.

This quantity summarizes the advances in our realizing of those problems, in addition to the aptitude courting among the 2. Key issues contain advances in our skill to clinically describe the frontotemporal syndromes, preclinical detection, neuroimaging, and genetics. The exploding box of latest markers in neuropathology is tested, as is the function of latest genetic mutations in DNA/RNA delivery structures. This ebook is the fundamental reference textual content for this subject, and may be of curiosity to neurologists and neurological trainees with a medical or study curiosity within the FTDs or ALS, neuropsychologists, neuropathologists, and researchers.

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Extra resources for Amyotrophic Lateral Sclerosis and the Frontotemporal Dementias

Sample text

Neurofibrillary tangles and abnormal accumulations of tau protein are found in these patients, similar to that found in patients with Alzheimer’s disease. Multiple hypotheses have been offered to link risk factors among the Chammoros and patients in this region. However, a causative toxic or environmental agent has not been definitively identified and this still remains an active area of investigation. In summary, since the early clinical descriptions, much has been added to our understanding of ALS as a complex clinical and pathological condition.

Patients affected solely with bulbar pathology are also often referred as having progressive bulbar palsy (also known as progressive bulbar atrophy), whereas patients affected with bulbar predominant symptoms are referred to as bulbar ALS (13–16). Furthermore, our traditional view of ALS as a disorder restricted to the motor system has been greatly modified by recognition of comorbid syndromes of frontotemporal dysfunction in up to 30–50% of patients (17,18). As discussed elsewhere in this text, patients with ALS are now identified as having primarily cognitive or behavioural pathology versus primary motor symptoms and more subtle cognitive involvement.

19. Plato CC, Cruz MT, Kurland LT. (1969). Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam: further genetic investigations. Am J Genet, 21, 133–141. 20. Spencer PS, Palmer VS, Ludolph AC. (2005). On the decline and etiology of high-incidence motor system disease in West Papua (Southwest New Guinea). Mov Disord, 20 (Suppl 12), S119–S126. 21. Miura K. (1911). Amyotrophische lateralclerose unter dem bilde von sog. bulbaparalyse. Neurol Jpn, 10, 366–36. 22. Kimura K, Yase Y, Higashi Y, et al.

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Amyotrophic Lateral Sclerosis and the Frontotemporal Dementias by Michael J. Strong


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