Functional impairment in progressive supranuclear palsy

Department of Psychological and Brain Sciences (A.G.), University of Louisville, Louisville, KY
Neurology (Impact Factor: 8.29). 01/2013; 80(4). DOI: 10.1212/WNL.0b013e31827f0859
Source: PubMed


The current study sought to describe the functional profiles of patients with early-stage progressive supranuclear palsy (PSP) in a large prospective, multisite study.

Using data from 202 individuals meeting criteria for clinically definite or probable PSP, 3 functional scales were examined. Functional scores were then compared to measures of motor, cognition, and psychiatric symptoms.

Functional disability was high in early-stage PSP, with 100% of patients having less than perfect scores on all functional scales. Whereas functional scores tended not to be related to cognition or psychiatric symptoms, they were strongly related to motoric ratings.

Both clinically and in research settings, the definition of functional intactness/impairment has important implications. Future studies should examine if functional impairment is this high in PSP or if new scales of functional abilities need to be developed for this condition.

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    • "PSP patients display impairments in executive functioning, memory, and other cognitive abilities (Gerstenecker et al., 2013). Functionally, when diagnosed, they usually have difficulties with most daily activities (Duff et al., 2013). Finally, PSP patients present with notable behavioral disturbances, particularly apathy, impulsivity, and irritability. "
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    ABSTRACT: Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder in which, classically, patients present with postural instability and falls, parkinsonism, and slowing of vertical saccades. PSP patients typically have deficits in cognitive functioning, difficulties with most daily activities, and present with notable behavioral disturbances—particularly apathy, impulsivity, and irritability. Using data from 154 patients meeting criteria for clinically probable PSP, domain and total scores of the Neuropsychiatric Inventory were examined and compared to demographics, disease severity, cognition, and motor features. Behavioral abnormalities were common in this cohort of PSP patients, with more than half experiencing apathy, depression, and sleeping problems, and approximately one third displaying agitation, irritability, disinhibition, and eating problems. Few clinical correlates of neuropsychiatric symptoms were observed in this cohort. Given the prevalence of neuropsychiatric symptoms in PSP, these patients are expected to be frequently seen by psychiatrists and other mental health professionals for symptom management and increased quality of life. Clinical trials are clearly needed to address the neuropsychiatric morbidity in these patients.
    Psychiatry Research 09/2013; 210(3). DOI:10.1016/j.psychres.2013.08.045 · 2.47 Impact Factor
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    ABSTRACT: Objectives To investigate the frequency and the different types of psychiatric disturbances in PSPpatients using validated psychiatric instruments. Methods We conducted a case-control study using the Structured Clinical Interview (SCID-I) for DSM-IV to investigate psychiatric disorders in 28 PSP patients and 28 age and gender matched healthy controls. PSP severity was scored using the PSP rating scale; cognitive functions were assessed using the Montreal Cognitive Assessment scale and the Frontal Assessment Battery scale. Results SCID-I disclosed that psychiatric disturbances were more frequent in PSP patients (53 %) than in healthy controls (17.8%) (p =0.005). Psychiatric disorders in PSP patients were characterized by mood disorders. Depression due to PSP was the most frequent diagnosis and was found in 8 of the 15 patients with depressive disorders. None of the PSP patients had a diagnosis of other SCID-I disorders. No clinical or demographic differences were found when comparing PSP patients with and without psychiatric disturbances. Conclusions Psychiatric disturbances, namely depressive disorders, were more frequent in PSP patients than in controls. A thorough evaluation of psychiatric disorders is important to insure appropriate treatment of PSP patients.
    Parkinsonism & Related Disorders 09/2014; 20(9). DOI:10.1016/j.parkreldis.2014.05.015 · 3.97 Impact Factor
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    ABSTRACT: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are characterized by rapid deterioration and a fatal outcome. Admission triggers, treatment efficacy, and care patterns. Retrospective analysis of patients with PSP/CBD admitted to an inpatient specialized palliative care service. In 38 patients, there were 63 admissions for swallowing difficulties, falls, pain, impaired communication, cognitive/mood disturbances, respiratory symptoms, and infection. Mean length of stay was 11.6 days. Treatment response was variable. In 68%, of admission episodes there was stabilization or improvement, 75% were discharged home. In case of readmission, the mean interval has been 9.7 months. Time since diagnosis and admission triggers were not associated with outcome or death. Patients showed high symptom load contrasting with discharge rates and subsequent health care utilization. Brief multidisciplinary interventions might be helpful to preserve autonomy. © The Author(s) 2014.
    The American journal of hospice & palliative care 12/2014; DOI:10.1177/1049909114565110 · 1.38 Impact Factor
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