Date: Sunday, October 7, 2018
Session Title: Parkinsonism, MSA, PSP (Secondary and Parkinsonism-Plus)
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: Explore cognitive and behavioural disturbances between subtypes of progressive supranuclear palsy (PSP).
Background: Although clinical diagnosis of PSP is based on specific motor symptoms, cognitive and behavioural disturbances are frequent and it is unknown whether PSP phenotypes differ in terms of cognition or behaviour.
Methods: Prospective recruitment of 30 early PSP patients (3.8±1.9y). According to current PSP-MDS criteria, Richardson´s(RS;n=18), progressive gait freezing(PGF;n=6), speech/language disorder(SL;n=4) and parkinsonism (PSP-P;n=2)). Patients did not differ in age, education and disease duration. All patients underwent a neuropsychological battery, including tests of global cognition(FAB) and all 5 domains. Behaviour was assessed by Neuropsychiatric Inventory(NPI), Starkstein Apathy Scale(SAS), Frontal Behaviour Inventory(FBI), Geriatric Depression Scale(GDS) and specific irritability(IQ) and social cognition checklists.
Results: The analyses showed predominant frontal and dysexecutive dysfunction in all phenotypes, with major impairment in FAB (9.6±3.7), TMTA (SS=5.5±3.2), TMTB (SS=4.6±3.3), phonetic (SS=6.8±2.6)/semantic fluencies (SS=4.9±2.3), and Stroop test (SS=5.7±1.7). Deficits were also found in memory (Buschke;immediate free recall SS=7.2±2.8) and visuospatial abilities (VOSP-Number Location SS=6.3±2.7). Comparative analysis showed greater cognitive impairment in RS and PGF phenotypes, with worse performance in FAB (total p=0.008,interference p=0.021,inhibition p=0.026), TMTB (p=0.027), Rey-Osterrieth copy (p=0.008), and clock drawing (p= 0.02). SL was the phenotype with more preserved cognition. Conversely, no clear differences in behaviour were found between phenotypes, apart from more impaired social cognition (p=0.027) in both the RS and PGF groups. In all phenotypes,apathy (SAS=17.2±10.1) showed the highest impairment, followed by depression (GDS=7.1±4.3) and irritability (IQ=4.5±3.6). The association of depression and apathy was present in 61.5%. FBI showed worse scores on negative items (13.0±7.8) than on deshinibition items (4.2±3.2).
Conclusions: -Cognitive deficits involve mental flexibility and rigidity since the early stages of the disease in all PSP phenotypes. -RS and PGF phenotypes exhibit more cognitive deterioration, and SL appears as the phenotype with more preserved cognition. -Apathy, depression and irritability are the most common co-existing behavioural symptoms in all PSP subtypes. -RS and PGF phenotypes showed more impairment in social cognition.
References: R.G. Brown et al. Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain 2010: 133; 2382–2393. Gerstenecker et al. Behavioral abnormalities in progressive supranuclear palsy. Psychiatry Res. 2013 December 30; 210(3).
To cite this abstract in AMA style:A. Horta-Barba, J. Pagonabarraga, S. Martinez-Horta, I. Illán, I. Aracil, J. Pérez-Pérez, B. Pascual, H. Bejr-Kasem, J. Marin, F. Sampedro, M. Botí, C. Izquierdo, A. Campolongo, J. Kulisevsky. Comparative study of the cognitive and behavioral disturbances between progressive supranuclear palsy phenotypes [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/comparative-study-of-the-cognitive-and-behavioral-disturbances-between-progressive-supranuclear-palsy-phenotypes/. Accessed December 11, 2023.
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