Cognitive and behavioural dysfunctions in a patient with progressive supranuclear palsy (PSP)
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Submission date: 2018-05-21
Final revision date: 2018-12-22
Acceptance date: 2018-12-23
Online publication date: 2019-01-30
Publication date: 2019-01-30
Health Psychology Report 2019;7(1):48–56
The aim of the case study was to describe the profile of cognitive and emotional functioning of a patient with possible progressive supranuclear palsy (PSP) from a longitudinal perspective.

Participants and procedure:
This study involved an 71-year-old male patient diagnosed with PSP, and 9 matched healthy subjects. Neuro-psychological examination of the patient was performed twice with a 6 month interval. A set of neuropsycho-logical tests was used to assess both cognition and behaviour.

Neuropsychological assessment revealed executive dysfunction dominance (planning deficits, reduced cogni-tive flexibility and abstract thinking, impulsiveness), reduced verbal fluency, psychomotor slowness and prob-lems with memory retrieval from the long-term memory storage in contrast to significantly better recognition of the previously learned information. According to emotional functioning, frontal change of personality was ob-served, with apathy, disinhibition, lack of insights, impulsiveness and “utilization behaviours”.

The profile of emotional and cognitive impairments met the criteria for dementia. There was a progression of deficits at visit two in comparison to visit one. The longitudinal perspective allowed the dynamics of emotional, cognitive and behavioural changes to be described over time: from depression related to initially preserved criticism of the illness to apathy and emotional blunting and behavioural frontal syndrome connected with the systematic loss of insight.

Ardila, A. (1992). Luria’s Approach to neuropsychological assessment. International Journal of Neuro-science, 66, 35–43.
Armstrong, R. A. (2011). Visual signs and symptoms of progressive supranuclear palsy. Clinical and Experimental Optometry, 94, 150–160. https://doi.org/10.1111/j.1444....
Aarsland, D., Litvan, I., Salmon, D., Galasko, D., Wentzel-Larsen, T., & Larsen, J. P. (2003). Perfor-mance on the dementia rating scale in Parkinson’s disease with dementia and dementia with Lewy bodies: comparison with progressive supranuclear palsy and Alzheimer’s disease. Journal of Neu-rology, Neurosurgery, and Psychiatry, 74, 1215–1220. https://doi.org/10.1136/jnnp.7....
Albert, M. I., Feldman, R. G., & Willis, A. L. (1974). The “subcortical dementia” of progressive supra-nuclear palsy. Journal of Neurology, Neurosurgery, and Psychiatry, 76, 420–422.
Bower, J. H., Maraganore, D. M., McDonnell, S. K., & Rocca, W. A. (1997). Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990. Neu-rology, 49, 1284–1288.
Burn, D. J., & Lees, A. J. (2002). Progressive supranuclear palsy: where are we now? The Lancet Neu-rology, 1, 359–369.
Crawford, J. R., & Garthwaite, P. H. (2002). Investigation of the single case in neuropsychology: con-fidence limits on the abnormality of test scores and test score differences. Neuropsychologia, 40, 1196–1208.
Crawford, J. R., Howell, D. C., & Garthwaite, P. H. (1998). Payne and Jones Revisited: Estimating the Abnormality of Test Score Differences Using a Modified Paired Samples t Test. Journal of Clinical and Experimental Neuropsychology, 20, 898–905.
Dubois, B., Deewer, B., & Pillon, B. (1996). The cognitive syndrome of progressive palsy. Advances in Neurology, 69, 399–403. https://doi.org/10.1016/j.park....
Enderby, P. M., Wood, V. A., Wade, D. T., & Hewer, R. L. (1986). The Frenchay Aphasia Screening Test: a short, simple test for aphasia appropriate for non-specialists. International Rehabilitation Medicine, 8, 166–170.
Ferrara, J. M., & Litvan, I. (2011). Kobieta z zaburzeniami chodu i trudnościami w czytaniu [A woman with a gait disorder and reading difficulties]. In S. Gauthier & P. Rossa-Neto (Eds.), P. P. Liberski & T. Sobów (Polish Eds.), Demencja. Trafna diagnoza [Dementia. Accurate diagnosis] (pp. 233–242). Warszawa: PZWL.
Folstein, M. F., Folstein, S. E., & McHugh, P. R. (1975). “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research, 12, 189–198.
Gerstenecker, A., Duff, K., Mast, B., & Litvan, I. (2013). Behavioral Abnormalities in Progressive Su-pranuclear Palsy. Psychiatry Research, 210, 1205–1210. https://doi.org/10.1016/j.psyc....
Hauw, J. J., & Agid Y. (2003). Progressive supranuclear palsy (PSP) or Steele-Richardson-Olszewski disease. In D. W. Dickson (Ed.). Neurodegeneration: The molecular pathology of dementia and movement disorders (pp. 103–114). Basel: ISN Neuropath Press.
Hillis, A. E. (2014). Inability to empathize: brain lesions that disrupt sharing and understanding anoth-er’s emotions. Brain, 137, 981–997. https://doi.org/10.1093/brain/....
Houghton, D. J., & Litvan I. (2007). Unraveling progressive supranuclear palsy: from the bedside back to the bench. Parkinsonism & Related Disorders, 13, S341–S346. https://doi.org/10.1016/S1353-....
Jelic, V., Nennesmo, I., Palhagen, S. E., & Winblad B. (2011). Najpierw upadki, potem zapominanie [First falls, then forgetfulness]. In S. Gauthier & P. Rossa-Neto (Eds.), P. P. Liberski & T. Sobów (Polish Eds.), Demencja. Trafna diagnoza [Dementia. Accurate diagnosis] (pp. 259–264). Warszawa: PZWL.
Kertesz, A., Davidson, W., & Fox, H. (1997). Frontal Behavioral Inventory: Diagnostic criteria for frontal lobe dementia. Canadian Journal of Neurological Science, 24, 29–36.
Kertesz, A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. G. (2005). The evolution and pa-thology of frontotemporal dementia. Brain, 128, 1996–2005. https://doi.org/10.1093/brain/....
Kertesz, A., Jesso, S., Harciarek, M., Blair, M., & McMonagle, P. (2010) What Is Semantic Dementia? A Cohort Study of Diagnostic Features and Clinical Boundaries. Archives of Neurology, 67, 483–489.
Kobylecki, C., Jones, M., Thompson, J. C., Richardson, A. M., Neary, D., Mann, D. M., Snowden, J. S., & Gerhard, A. (2015). Cognitive-behavioural features of progressive supranuclear palsy syn-drome overlap with frontotemporal dementia. Journal of Neurology, 262, 916–22. https://doi.org/10.1007/s00415....
Lange, K. W., Tucha, O., Alders, G. L., Preier, M., Csoti, I., Merz, B., Mark, G., Herting, B., For-nadi, F., Reichmann, H., Vieregge, P., Reiners, K., Becker, G., & Naumann, M. (2003). Differentia-tion in parkinsonian syndromes according to differences in executive functions. Journal of Neural Transmission, 110, 983–995. https://doi.org/10.1007/s00702....
Lauterbach, E. C. (2004). The neuropsychiatry of Parkinson’s disease and related disorders. Psychiat-ric Clinics of North America, 27, 801–825.
Lhermitte, F. (1983). Utilization behavior and its relation to lesions of the frontal lobes. Brain, 106, 237–255.
Liberski, P. P., Sobów, T., & Wszołek, Z. K. (2005). Postępujące zwyrodnienie nadjądrowe [Pro-gressive supranuclear degeneration]. In P. P. Liberski & Z. K. Wszołek (Eds.), Otępienia czołowo-skroniowe [Frontotemporal dementia] (pp. 155–172). Lublin: Wydawnictwo Czelej.
Liberski, P. P., Sikorska, B., & Sobów T. (2006). Otępienie w atypowych postaciach parkinsonizmu [Dementia in atypical forms of parkinsonism]. In T. Sobów & J. Sławek (Eds.), Zaburzenia poznaw-cze i psychiczne w chorobie Parkinsona i w innych zespołach parkinsonowskich [Cognitive and men-tal disorders in Parkinson’s disease and other parkinsonian syndromes] (pp. 113–126). Wrocław: Wydawnictwo Continuo.
Liscic, R. M., Srulijes, K., Gröger, A., Maetzler, W., & Berg, D. (2013). Differentiation of Progressive Supranuclear Palsy: clinical, imaging and laboratory tools. Acta Neurologica Scandinavica, 2, 1–9. https://doi.org/10.1111/ane.12....
Litvan, I. (1994). Cognitive disturbances in progressive supranuclear palsy. Journal of Neural Trans-mission, 42, 69–78.
Litvan, I., & Kong, M. (2014). Rate of decline in progressive supranuclear palsy. Movement Disorders, 29, 463–468. https://doi.org/10.1002/mds.25....
Litvan, I., Grimes, D. A., Lang, A. E., Jankovic, J., McKee, A., Verny, M., Jellinger, K., Chaudhuri, K. R., & Pearce, R. K. (1999). Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration. Journal of Neurology, 246, 1–5.
Luria, A. R. (1962). Higher Cortical Functions in Man. New York: Basic Books.
Łuria, A. (1976). Neuropsychologia [Neuropsychology]. Warszawa: PZWL.
Millar, D., Griffiths, P., & Zermansky, A. J. (2006). Characterising behavioral and cognitive dysexecu-tive changes in progressive supranuclear palsy. Movement Disorders, 21, 199–207. https://doi.org/10.1002/mds.20....
Motyl, R. (2007). Otępienie – kryteria diagnostyczne [Dementia – diagnostic criteria]. Polski Przegląd Neurologiczny, 3, 43–60.
Nath, U., Ben-Shlomo, Y., Thomson, R. G., Morris, H. R., Wood, H. R., Lees, A. J., & Burn, J. (2001). The prevalence of progressive supranuclear palsy (Steele–Richardson–Olszewski syndrome) in the UK. Brain, 124, 1438–1449.
Nijboer, H., & Dautzenberg, P. L. (2009). Progressive supranuclear palsy: Acetylcholineeserase-inhibitor a possible therapy. Tijdschrift voor Gerontologie en Geriatrie, 40, 133–137.
Paviour, D. C., Price, S. L., Jahanshahi M., Lees, A. J., & Fox, N. C. (2006). Longitudinal MRI in pro-gressive supranuclear palsy and multiple system atrophy: rates and regions of atrophy. Brain, 129, 1040–1049. https://doi.org/10.1093/brain/....
Pąchalska, M., & MacQueen, B. D. (1998). Kwestionariusz Zachowań Osoby z Zespołem Czołowym. Autoryzowany przekład z języka angielskiego: Frontal Behavioral Inventory (Kertesz i wsp., 1997) [Frontal Behavioural Inventory. Authorized translation from English (Kertesz et al., 1997)]. Kraków: Fundacja na Rzecz Osób z Dysfunkcjami Mózgu.
Pekmezović, T., Ječmenica-Lukić, M., Petrović, I., Špica, V., Tomić, A., & Kostić, V. (2015). Quality of life in patients with progressive supranuclear palsy: one-year follow-up. Journal of Neurology, 262, 2042–2048. https://doi.org/10.1007/s00415....
Piskunowicz, M., Bieliński, M., Zgliński, A., & Borkowska, A. (2013). Testy fluencji słownej – zastoso-wanie w diagnostyce neuropsychologicznej [Verbal fluency tests – application in neuropsychologi-cal assessment]. Psychiatria Polska, 47, 475–485.
Rittman, T., Coyle-Gilchrist, I. T., & Rowe, J. B. (2016). Managing cognition in progressive supranu-clear palsy. Neurodegenerative Disease Management, 6, 499–508. http://doi.org/10.2217/nmt-201....
Rittman, T., Ghosh, B. C., McColgan, P., Breen, D. P., Evans, J., Williams-Gray, C. H., Barker, R. A., & Rowe, J. B. (2013). The Addenbrooke’s Cognitive Examination for the differential diagnosis and longitudinal assessment of patients with parkinsonian disorders. Journal of Neurology, Neurosur-gery, and Psychiatry, 84, 544–551. https://doi.org/10.1136/jnnp-2....
Rohrer, J. D., Paviour, D., Bronstein, A. M., O’Sulli-van, S. S., Lees, A., & Warren, J. D. (2010). Pro-gressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: a neuropsychological and neuroimaging analysis. Movement Disorders, 30, 179–188. https://doi.org/10.1002/mds.22....
Sha, S. J., Hou, C., Viskontas, I. V., & Miller, B. L. (2007). Are frontotemporal lobar degeneration, progressive supranuclear palsy and corticobasal degeneration distinct diseases? Nature Clinical Practice Neurology, 2, 658–665. https://doi.org/10.1038/ncpneu....
Sitburana, O., & Ondo W. G. (2009). Brain magnetic imaging (MRI) in parkinsonian disorders. Parkin-sonism & Related Disorders, 15, 165–174. https://doi.org/10.1016/j.park....
Steele, J. C., Richardson, J. C., & Olszewski, J. (1964). Progressive supranuclear palsy. A heteroge-neous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Archives of Neurology, 10, 333–359. https://doi.org/10.1055/s-0034....
Talarowska, M., Florkowski, A., Orzechowska, A., Zboralski, A., & Gałecki, P. (2011). Auditory-verbal learning test by Łuria in the examination of psychiatric patients. Polski Merkuriusz Lekarski, 31, 41–44.
Walsh, K., & Darby, D. (2008). Płaty czołowe [Frontal lobes]. In K. Walsh & D. Darby (Eds.), Neurop-sychologia kliniczna Walsha [Walsh’s Neuropsychology: A Clinical Approach] (pp. 124–181). Gdańsk: Wydawnictwo GWP.
Williams, D. R., & Lees, A. J. (2009). Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurology, 8, 270–279. https://doi.org/10.1016/S1474-....
Williams, D. R., & Lees A. J. (2010). What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)? Movement Disorders, 25, 357–362. https://doi.org/10.1002/mds.22....