
On the other hand, mutations three main genes (MAPT, GRN and C9ORF72) are found in 60% of familial FTD cases, whereas rare mutations (<5%) have been found in other 11 genes. At present, the four major ALS-associated genes are the chromosome 9 open reading frame 72 ( C9ORF72), Cu-Zn superoxide dismutase 1 ( SOD1), TAR DNA-binding protein 43 ( TARDBP), and fusion in malignant liposarcoma/translocation in liposarcoma ( FUS/TLS), in addition to at least other 40 genes. Mutations in at least 44 loci have been linked to ALS and, correspondingly, at least 14 loci have been linked to FTD. Over this time period, more than 50 genes have been associated with ALS and FTD. The outlook of ALS and FTD as distinct nosological entities has progressively changed in the last decade due to genetic breakthroughs. However, it is increasingly recognized that ALS is a multisystem disorder in which other non-motor (and in particular, cognitive and behavioral) impairments can be observed, whereas, on the other side, FTD can be associated to signs of motor neuron disease (FTD-MND). Indeed, along this line, recent studies have outlined how these two diseases seem to be also characterized by different inflammatory profiles, with predominance of macrophage/microglia activation in ALS and astrocyte malfunctioning in FTD. These pathologies were initially considered two independent clinical entities, because of heterogeneity at clinical and neuropathological levels.

More specifically, there is a general consensus that FTD consists of three distinct clinical syndromes: behavioral variant frontotemporal degeneration (bvFTD), non-fluent variant primary progressive aphasia (nfvPPA) and semantic variant primary progressive aphasia (svPPA). On the other hand, FTD, whose incidence in Europe is 3.00 person-years, is a type of dementia characterized by atrophy of the frontal and temporal lobes, with composite clinical signs ranging from alterations in behavior and language to motor and cognitive dysfunctions. ALS, whose incidence in Europe is 2.00 person-years, is a progressive and ultimately fatal disease caused by progressive loss of motor neurons controlling voluntary muscles. Not available with lease and some other offers.In principle, Amyotrophic lateral sclerosis (ALS) and Frontotemporal Degeneration (FTD) are two very different neurodegenerative diseases. 4Monthly payment is $13.89 for every $1,000 you finance.Įxample down payment: 4.4%. Not available with special finance, lease, or some other offers. 2Not available with special financing, lease and some other offers. See dealer for details.ġNot available with special financing, lease and some other offers. Not available with lease and some other offers. Monthly payment is $13.89 for every $1,000 you finance. See dealer for details.ĢNot available with special financing, lease and some other offers. 1Not available with special financing, lease and some other offers. 7Monthly payment is $13.89 for every $1,000 you finance. Residency restrictions apply.ĦMust qualify through GM Financial. Take delivery.ĥNot available with lease and some other offers. See Parsons for details.ĤNot available with special finance or lease offers. Please call Parsons of Eagle River for complete details 3plus tax, title, license, and dealer service fee. Please call Parsons of Eagle River for complete details 2Plus Tax, Title, License, and Dealer Service Fee. May be eligible for select New Vehicle Rebates. 1Driven as a Courtesy Transportation Vehicle.

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