Hostname: page-component-cd9895bd7-q99xh Total loading time: 0 Render date: 2024-12-27T15:19:26.411Z Has data issue: false hasContentIssue false
  • English
  • Français

Rorschach assessment of personality functioning in patients with polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

Published online by Cambridge University Press:  24 June 2014

Tuula Ilonen ,
Panu Hakola ,
Matti Vanhanen  and
Jari Tiihonen
Tuula Ilonen*
Affiliation:
Department of Psychiatry, University of Turku, Turku, Finland
Panu Hakola
Affiliation:
Department of Forensic Psychiatry, University of Eastern Finland, Kuopio, Finland
Matti Vanhanen
Affiliation:
Niuvanniemi Hospital, Kuopio, Finland
Jari Tiihonen
Affiliation:
Department of Forensic Psychiatry, University of Eastern Finland, Kuopio, Finland Department of Clinical Physiology, Kuopio University Hospital, Kuopio, Finland
*
Tuula Ilonen, Department of Psychiatry, University of Turku, Turku, Finland. Tel: +358 2 266 2526; Fax: +358 2 266 2528; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Extract

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.

Keywords

dementiapersonalitypolycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathyRorschach CS
Type
Research Article
Information
Acta Neuropsychiatrica , Volume 24 , Issue 4 , August 2012 , pp. 236 - 244
Copyright
Copyright © Cambridge University Press 2011

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Hakola, HPA.Neuropsychiatric and genetic aspects of a new hereditary disease characterized by progressive dementia and lipomembranous polycystic osteodysplasia. Acta Psychiatr Scand 1972;232 (Suppl.):1–173.Google ScholarPubMed
2.Nasu, T, Tsukahara, Y, Terayama, K.A libid metabolic disease – Membranous lipodystrophy”. Acta Pathol Jpn 1973;23:539–558.Google Scholar
3.Paloneva, J, Autti, T, Raininko, R et al. CNS manifestations of Nasu-Hakola disease. A frontal dementia with bone cysts. Neurology 2001;56:1552–1556.CrossRefGoogle ScholarPubMed
4.Hakola, HPA.Polycystic libomembranous osteodysplasia with schlerosing leukoencephalopathy (membranous lipodystrophy): a neuropsychiatric follow-up study. Monographs of Psychiatrica Fennica 1990;17:1–114.Google Scholar
5.Pekkarinen, P, Kestilä, M, Paloneva, J et al. Fine-scale mapping of a novel dementia gene, PLOSL, by linkage disequilibrium. Genomics 1998;54:307–315.Google Scholar
6.Paloneva, J, Kestilä, M, Wu, J et al. Loss-of-function mutations in TYROBP (DAP12) result in a presenile dementia with bone cysts. Nat Genet 2000;25:357–361.CrossRefGoogle Scholar
7.Paloneva, J, Mandelin, J, Kiialainen, A et al. DAP12/ TREM2 deficiency results in impaired osteoclast differentiation and osteoporotic features. J Exp Med 2003;192: 669–675.CrossRefGoogle Scholar
8.Paloneva, J, Manninen, T, Christman, G et al. Mutations in two genes encoding different subunits of a receptor signaling complex result in an identical disease phenotype. Am J Human Gen 2002;71:656–662.CrossRefGoogle Scholar
9.Kiialainen, A, Hovanes, K, Paloneva, J, Kopra, O, Peltonen, L.Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS. Neurobiol Dis 2005;18:314–322.CrossRefGoogle ScholarPubMed
10.Kiialainen, A, Veckman, V, Saharinen, J et al. Transcript profiles of dendritic cells of PLOSL patients link demyelinating CNS disorders with abnormalities in pathways of actin bundling and immune response. J Mol Med 2007;85: 971–983.CrossRefGoogle ScholarPubMed
11.Kiialainen, A, Linturi, H, Saharinen, J, Lanier, LL, Kopra, O, Peltonen, L. DAP 12 (Tyrobp) deficient mice show defects in microglial cell function and abnormal synaptic properties. In: Kiialainen, A, ed (Academic dissertation). Pathogenic mechanism of polycystic lipomembranous osteodysplacia with sclerosing leukoencephalopathy (PLOSL). Helsinki: Publications of theNational Public Health Institute, 2007, 14/207, 1–17 in Part III.Google Scholar
12.Virtama, PEJ, Hakola, MT, Hakola, HPA.“Bone radiography of PLO-SL cases”. Helsinki: Foundation for Psychiatric Research in Finland, 1990.Google Scholar
13.Akai, M, Tateishi, A, Cheng, CH et al. Mempranous lipodystrophy, a clinicopathological study of six cases. J Bone Joint Surg 1977;59:802–809.Google Scholar
14.Harada, K.Ein Fall von “Membranöser Lipodystrophy (Nasu) unter besonder Berückzichtigung des psychiatrischen und neuropathologischen Befundes. Folia Psychiatr Neurol Jpn 1975;29:169–177.Google Scholar
15.Kitajima, I, Kuriyama, M, Usuki, F et al. Nasu-Hakola disease (membranous lipodystrophy). Clinical, histopathological and biochemical studies of three cases. J Neurol Sci 1989;91:35–52.CrossRefGoogle ScholarPubMed
16.Matsushita, M, Oyanagi, S, Hanawa, S, Shiraki, H, Kosaka, K.Nasu-Hakola's disease (membranous libodystrophy). A case-report. Acta Neuropathol 1981;54:89–93.CrossRefGoogle ScholarPubMed
17.Sourander, P, Järvi, O, Hakola, P, Kalimo, H, Nevalainen, T.Neuropathological aspects of polycystic lipomempranous osteodysplasia with sclerosing leucoencephalopathy (membranous lipodystrophy). International Symposium on the Leucodystrophy and Allied Diseases, Okazaki Kyoto. The Japanise Soc Neuropath 1981; (Suppl. 1):87–101.Google Scholar
18.Tanaka, J.Leucoencephalopathic alteration in membranous lipodystrophy. Acta Neuropathol 1980;50:193–197.CrossRefGoogle Scholar
19.Hakola, HPA, Iivanainen, M.A new hereditary disease with progressive dementia and polycystic osteodysplasia. Neuroradiological analysis of seven cases. Neuroradiography 1973;6:162–168.Google Scholar
20.Hakola, HPA, Puranen, M.Neuropsychiatric and brain CT findings in polycystic lipomembranous osteodysplasia with sclerosing leucoencephalopathy. Acta Neurol Scand 1993;88:370–375.CrossRefGoogle Scholar
21.Iivanainen, M, Hakola, P, Erkinjuntti, T et al. Cerebral MR and CT imaging in polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy. J Comput Assist Tomogr 1984;8:940–943.CrossRefGoogle ScholarPubMed
22.Hakola, HPA, Partanen, I.Neurophysiological findings in progressive dementia with lipomembranous polycystic osteodysplasia. J Neurol Neurosurg Psychiatry 1983;46:515–520.CrossRefGoogle Scholar
23.Hakola, HPA.Benton's Visual Retention Test in patients with polycystic lipomembranous dysplasia with sclerosing leukoencephalopathy. Dement Geriatr Cogn Disord 1998;9:39–43.CrossRefGoogle ScholarPubMed
24.Vanhanen, M, Hakola, P, Ilonen, T, Tiihonen, J. The 10-word test in PLOSL patients (manuscript).Google Scholar
25.Rorschach, H.Psychodiagnostics: a diagnostic test based on perception. Bern: Hans Huber, 1942 (original work published in 1921).Google Scholar
26.Piotrowski, Z.The Rorschach Inkblot method in organic disturbances of the central nervous system. J Nerv Ment Dis 1937;86:525–537.CrossRefGoogle Scholar
27.Dorken, H, Kral, VA.Psychological investigation of senile dementia. Geriatrics 1951;6:151–163.Google ScholarPubMed
28.Insua, AM, Loza, SM.Psychometric patterns on the Rorschach of healthy elderly persons and patients with suspected dementia. Percept Mot Skills 1986;63:931–936.CrossRefGoogle ScholarPubMed
29.Perry, W, Potterat, E, Auslander, L, Kaplan, E, Jeste, D.A neuropsychological approach to the Rorschach in patients with dementia of the Alzheimer type. Assessment 1996;3:351–363.CrossRefGoogle Scholar
30.Stuss, DT, Cow, CA, Hetherington, CR.“No longer gage”: frontal lobe dysfunction and emotional changes. J Consult Clin Psychol 1992;3:349–359.CrossRefGoogle Scholar
31.Lezak, MD.Neuropsychological assessment, 3rd edn.Oxford: Oxford University Press, 1995.Google Scholar
32.Exner, JE. The Rorschach: a comprehensive system. In: Basic foundations and principles of interpretation, Vol. 1, 4th edn.New York: Wiley, 2003.Google Scholar
33.Exner, JE. The Rorschach: a comprehensive system. In: Basic foundations, Vol. 1, 3rd edn.New York: Wiley, 1993.Google Scholar
34.Viglione, DJ.A review of recent research addressing the utility of the Rorschach. Psychol Assess 1999;11: 251–265.CrossRefGoogle Scholar
35.Viglione, DJ, Taylor, N.Empirical support for interrater reliability of Rorschach comprehensive system coding. J Clin Psychol 2003;59:111–121.CrossRefGoogle ScholarPubMed
36.Knight, RT.Decreased response to novel stimuli after prefrontal lesions in man. Electroencephalogr Clin Neurophysiol 1984;59:9–20.Google Scholar
37.Ota, M, Obu, S, Sato, N, Asada, T.Neuroimaging study in subjects at high risk of psychosis revealed by the Rorschach test and first-episode schizophrenia. Acta Neuropsychiatrica 2011;23:125–131.CrossRefGoogle ScholarPubMed
38.Mclellan, T, Johnston, L, Dalrymple-alford, J, Porter, R.The recognition of facial expressions of emotion in Alzheimer's disease: a review of findings. Acta Neuropsychiatrica 2008;20:236–250.CrossRefGoogle ScholarPubMed
39.Grattan, LM, Eslinger, PJ.The psychological basis of introversion-extraversion. Behav Res Ther 1990;8:249–266.Google Scholar