Word List Learning in Patients with Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy
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Word List Learning in Patients with Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy
BACKGROUND/AIMS Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a rare hereditary disease that is characterized by a combination of progressive presenile dementia and sclerosing leukoencephalopathy with bone cysts. No quantitative information on verbal memory functioning in PLOSL patients compared with control subjects is available. METHODS 23 patients ...
متن کامل[Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy].
BACKGROUND Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (Nasu-Hakola syndrome) (PLO-SL) is an unusual cause of early dementia in which the decline in cognitive function is associated with multiple bone cysts, in particular in hands and feet. About 165 cases of this autosomal recessive hereditary disease have been reported worldwide, mostly from Finland and Japan....
متن کاملPathogenic Mechanisms of Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy (PLOSL)
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), also known as Nasu-Hakola disease, is a recessively inherited disease of brain and bone. PLOSL manifests as early-onset progressive dementia and bone fractures. Mutations in the TYROBP (DAP12) and TREM2 genes have been identified as the primary cause of PLOSL. DAP12 and TREM2 encode important signalling molecu...
متن کاملNeurophysiological findings in the hereditary presenile dementia characterised by polycystic lipomembranous osteodysplasia and sclerosing leukoencephalopathy.
17 patients suffering from the presenile dementia characterised by polycystic lipomenbranous osteodysplasia and sclerosing leukoencephalopathy were studied neurophysiologically. Noteworthy findings were diffuse slowing, the accentuation of 6-8 Hz parasagittal activity, slowing of the dominant occipital rhythm and, in the late phase when 13 of patients got epileptic seizures, there was paroxysma...
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Takeshi Nagasaka, Hideyuki Mishima, Akira Sawaki, Mototsugu Shimokawa, Michio Inukai, Katsunori Shinozaki, Hiroaki Tanioka, Junichiro Nasu, Tomohiro Nishina, Shoichi Hazama, Masazumi Okajima, Yoshiyuki Yamaguchi To cite: Nagasaka T, Mishima H, Sawaki A, et al. Protocol of a randomised phase III clinical trial of sequential capecitabine or 5fluorouracil plus bevacizumab (Cape/5-FU-Bmab) to capec...
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ژورنال
عنوان ژورنال: Dementia and Geriatric Cognitive Disorders Extra
سال: 2013
ISSN: 1664-5464
DOI: 10.1159/000346857