Caregiver strain in progressive supranuclear palsy and corticobasal syndromes

Lukas Kellermair,Gerhard Ransmayr,Franz Fellner,Petra Schwingenschuh,Stephanie Mangesius,Alexandra Fuchs,Michael Guger,Thomas Forstner,Christian Eggers,Mariella Kögl

doi:10.1007/s00702-021-02379-z

Abstract

Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) progress relentlessly and lead to a need for care. Caregiving is often burdensome. Little is known about the course of caregiver burden (CB) in PSP and CBS patients. Longitudinal analysis of CB in family members caring for PSP and CBS patients. Single-center longitudinal pilot study in 68 newly diagnosed patients with probable PSP and CBS (52 Richardson’s syndrome; 1 progressive gait freezing of PSP; 15 CBS). Demographic, educational, occupational parameters, family status, motor functions (UPDRSIII, Hoehn and Yahr Score, Tinetti) and neuropsychological performance (CERAD Plus, Frontal Assessment Battery) were assessed, as well as behavioral and neuropsychiatric impairments (Frontal Behavioral Inventory, Neuropsychiatric Inventory), activities of daily living (ADL) and caregiver burden using the Caregiver Strain Index (CSI), in most patients also the Zarit Burden Interview (ZBI). Patients were followed up every 6 months for up to 2 years. Caregivers reported mild to moderate CB at baseline, which increased by 25–30% in 2 years and was significantly greater in PSP than in CBS. Risk for mental health problems increased over time, especially in female caregivers (depression). Important patient-related factors were apathy, aspontaneity, depression, irritability, disorganization, poor judgment, impairment of language, impairments in ADL, a high educational level of the patient and close family relationship. Behavioral symptoms and impaired ADL are the main patient-related factors of CB in PSP and CBS. CB can be severe and needs to be assessed repeatedly from the time of diagnosis to provide comprehensive support.

Highlights

From 2009 to 2018, we recruited 52 consecutive patients with newly diagnosed probable Progressive supranuclear palsy (PSP)-Richardson syndrome (RS) and one patient with PSP-progressive gait freezing according to the NINDS-SPSP criteria (Litvan et al 1996a), updated by Williams and Lees (Williams and Lees 2009), and 15 patients with probable corticobasal syndrome (CBS) according to Litvan et al.1997, after 2013 according to Armstrong et al (2013a,b)
Little is known about the course of the burden of care for patients with PSP and CBS, in particular during the first years after diagnosis (Uttl et al 1998; Schmotz et al 2017; Bukki et al 2016; Knutson et al 2008; Pillas et al 2016; Armstrong et al 2013b; Jabbari et al 2020)
Because of clinical and presumed neuropathological overlaps between PSP and CBI in our collective and in agreement with the literature, PSP and CBS patients were merged for an analysis of factors underlying caregiver burden (Bukki et al 2016; Armstrong et al 2013b; Jabbari et al 2020)

Summary

Introduction

A similar syndrome comprising asymmetric rigidity, akinesia, dystonia and apraxia was observed, from which the concepts of corticobasal degeneration and later corticobasal syndrome emerged (CBS; Ali and Josephs 2018; Rebeiz et al 1968; Armstrong et al 2013a). Cognitive and neuropsychiatric and behavioral symptoms, such as irritability, disinhibition, and depression, as well as neuropathological features overlap in PSP and CBS

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