Archicad 16 Portablelkjh
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Primary lateral sclerosis in Japan: A multicenter study. Primary lateral sclerosis (PLS) is a rare subtype of amyotrophic lateral sclerosis (ALS), and there are few reports of Japanese patients with PLS. We performed a retrospective analysis of all the patients with ALS who were diagnosed at one of the nine neurological institutes in Japan between January 2009 and June 2014. This is the first report of a nationwide retrospective study of PLS in Japan. Our study included 62 patients with ALS who were diagnosed using the revised El Escorial criteria. Of these patients, nine were diagnosed with PLS. The mean age at onset was 64.3 years in the PLS group and 57.6 years in the control group (P = 0.83). The mean interval from onset to diagnosis was 3.9 years in the PLS group and 1.9 years in the control group (P = 0.12). The level of serum neuron-specific enolase and the rate of cognitive impairment were significantly higher in the PLS group than in the control group. Ataxia was observed in all nine patients with PLS. None of the patients with PLS were found to have positive mutations for SOD1, C9orf72, TDP-43, or FUS. The frequency of a D90A mutation in the Cu/Zn superoxide dismutase gene (SOD1) was observed to be as high as that in the control group. The frequency of cognitive impairment was higher in the PLS group than in the control group. The mean age at onset was higher in the PLS group than in the control group. We also found a higher frequency of ataxia in the PLS group than in the control group. Our study suggests that some factors that are not associated with ALS may influence the occurrence of PLS.
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