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

• Nasu-Hakola disease
• Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy
• Dementia
• Frontal lobe degeneration