Objective: To date no studies have investigated the personality functioning underlying patients diagnosed with polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) using the performance-based Rorschach test.

Methods: We scored and interpreted the Rorschach protocols of eight carefully diagnosed PLOSL patients according to Exner's Comprehensive System. The structural variables in the Rorschach are organised around the seven dimensions of personality functioning that they assess: coping style and resources, organising information, perceiving events, forming concepts and ideas, handling of emotions, self-perception and interpersonal perception.

Results: As a group PLOSL patients had many personality liabilities when contrasted with typical avoidant non-patient adults. A majority of patients showed an avoidant coping style (Lambda > 0.99), low productivity and poor verbal output in the low number of responses and few Blends. Also, they showed limited available resources to cope with problem-solving test. Problems in organising information efficiently, and perceiving events realistically as well as signs of disturbed thinking and concept formation were observed in many patients. Handling of emotions was characterised by avoidance of emotional stimuli but at the same time poor affect modulation. Capacities to view themselves and others were thoroughly limited.

Conclusions: PLOSL tends to have repercussions throughout the personality functioning. In line with clinical findings and later neuroradiological and neuropathological examinations the Rorschach revealed personality features typical for frontal type of dementia.