Word list learning in patients with polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy
- PMID: 23569454
- PMCID: PMC3618099
- DOI: 10.1159/000346857
Word list learning in patients with polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy
Abstract
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 with PLOSL and 23 control subjects were examined with a version of the 10-word list-learning task. Learning curves were compared between the patients and the matched control subjects.
Results: Compared with the control subjects, PLOSL patients with moderate or severe dementia were impaired in both learning trials and delayed recall on the 10-word list-learning test.
Conclusion: Progressive degeneration of brain structures affecting the hippocampus and the medial temporal lobe with advanced PLOSL disease contributes to an inefficient verbal learning process.
Keywords: Dementia; Frontal lobe degeneration; Nasu-Hakola disease; Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy.
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