key: cord-0938913-bowpx6fs
authors: Bombaci, Alessandro; Giusiano, Silvia; Peotta, Laura; Iazzolino, Barbara; Mastro, Enza; Arcari, Martina; Palumbo, Francesca; Torrieri, Maria Claudia; Grassano, Maurizio; Cabras, Sara; Di Pede, Francesca; De Mattei, Filippo; Matteoni, Enrico; Solero, Luca; Daviddi, Margherita; Salamone, Paolina; Fuda, Giuseppe; Manera, Umberto; Canosa, Antonio; Chio, Adriano; Calvo, Andrea; Moglia, Cristina; Vasta, Rosario
title: Amyotrophic lateral sclerosis patients' quality of life and their caregiver burden during COVID-19 pandemic
date: 2021-10-31
journal: Journal of the Neurological Sciences
DOI: 10.1016/j.jns.2021.117701
sha: da03950ee1e1c2896e481a43492b311fb8e0b938
doc_id: 938913
cord_uid: bowpx6fs

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had a profile of amnesic mild cognitive impairment and 5 (1 VUS) had cognitively-normal profile. At follow up, three ALSbi patients (one with VUS) developed frank frontotemporal dementia (FTD) and one ALSbi developed cognitive impairment (ALScbi). The most common behavioural changes were apathy, mental rigidity and irritability.

Our findings demonstrate that SOD1 patients have early behavioural impairment more commonly than previously reported, which might increase the risk to develop FTD. 

The two-month lockdown period during COVID-19 pandemic had a general impact on health treatments and care assistance. We wanted to assess Quality of life (QoL) of ALS patients and the burden of their caregivers during that period.

Methods 60 patients and 59 caregivers, visited in telemedicine during March 2020, underwent the assessment of patients' QoL (McGill QoL Questionnaire), general health status (EQ-5D-5L) and caregiver burden (Zarit Burden Interview [ZBI]). These phone scales were administered in April 2020 (T1) and repeated one month after the end of lockdown (T2), with the addition of a qualitative questionnaire (COVID-QoL-Questionnaire), exploring family reorganization and personal perception of lockdown. Wilcoxon signed-rank test and the chi-squared test were used.

QoL and perceived health status did not worsen during lockdown, while caregiver burden increased (p = 0.01). Patient's QoL and caregiver burden were mildly inversely correlated at T1 (p b 0.05, rho = −0.265); no significant correlations were found at T2. According to the COVID-QoL-Questionnaire, both patients and caregivers were able to consult their physicians while at home (60% and 66.1% respectively). Moreover, caregivers perceived lower family help compared to patients (p b 0.001).

In our cohort of ALS patients, QoL and caregiver burden were not compromised by restriction measures during COVID-19 pandemic, while caregiver burden significantly increased. Probably, this is due to the motor impairment in ALS patients, which did not perceive changing in life conditions. Instead, the reported increased burden for primary caregivers could be explained by the restriction of family help, reflecting the importance of a wide social support in ALS patients ' management. doi:10.1016/j.jns.2021.117701 Background and aims Robust preclinical findings demonstrated that guanabenz selectively inhibits ER stress-induced eIF2α-phosphatase allowing misfolded protein clearance, reduces neuronal death and prolongs survival in in vitro and in vivo ALS models.

In this multicentre RCT with futility design, ALS patients with onset b18 months were randomly assigned to receive in a 1:1:1:1 ratio guanabenz 64 mg, 32 mg, 16 mg or placebo daily for 6 months as add-on-therapy to riluzole. Primary outcome was the proportion of patients progressing to higher stages of disease measured by ALS-MITOs compared to a historical cohort of 200 patients. Secondary Abstracts / Journal of the Neurological Sciences 429 (2021) 117638 

IRCCS FONDAZIONE ISTITUTO NEUROLOGICO CARLO BESTA, Scientific Directorate