Cognitive and Sleep Features of Multiple System Atrophy: Review and Prospective Study

Abstract

Background: The profile and degree of cognitive impairment in Multiple System Atrophy (MSA) and the impact of sleep disorders, REM sleep behavior disorder (RBD) in particular, in parkinsonism-related cognitive deficits are currently being debated. Summary: We reviewed the cognitive, affective and sleep findings in MSA and also carried out a longitudinal investigation of 10 MSA patients. At the first evaluation, 3 patients showed isolated cognitive deficits. After a mean of 16 months, these patients remained unchanged, while 1 patient worsened from a normal condition. No significant differences emerged when the cognitive, affective and video-polysomnographic findings of MSA-P and MSA-C were compared. Depression was present in half of the patients, although it did not influence their cognitive performance. Comparisons between the first and second evaluation data showed significant worsening in visual attention and in ADL/IADL and UMSARS. Key Messages: Isolated cognitive deficits are evidenced in a minority of MSA patients with the absence of a clear-cut diagnosis of dementia in the early stages of the disease. Attention and executive functions are often impaired. This study with a short follow-up period showed that RBD, although present in almost all patients affected by MSA, does not appear a clear early marker of cognitive impairment. Future longer-term studies with a larger patient sample are thus encouraged.

Distinct Parameters in the EEG of the PLP α-SYN Mouse Model for Multiple System Atrophy Reinforce Face Validity

“…This is in agreement with clinical findings on normal amounts of REMS in MSA patients (Vetrugno et al, 2004; Stanzani-Maserati et al, 2014). But old MSA animals spent significantly less amount in REMS than the young MSA mice.…”

“…REMS latency seems to be decreased in MSA patients (Manni et al, 1993; Plazzi et al, 1997; Stanzani-Maserati et al, 2014). We also found decreased REMS latency in our MSA animals, compared to both control groups.…”

Cognition in multiple system atrophy: a single‐center cohort study

“…So far, few studies have addressed the possible differences between patients with predominant cerebellar or parkinsonian presentation, and the focus has been mainly on MSA‐P patients. In this study, a comparison of raw scores revealed lower performance of MSA‐C patients than MSA‐P patients in a screening of executive functions and in phonological verbal fluency.…”

“…In contrast, severe cognitive decline that significantly disrupts daily living is uncommon in MSA; hence, dementia has been regarded as a nonsupporting feature in the current diagnostic criteria . Comparative studies on cognitive impairment in both motor subtypes have been conducted but reported heterogeneous results . Kawai and colleagues observed a multidomain cognitive decline in MSA‐P but not in MSA‐C .…”

Prevalence of REM sleep behavior disorder in multiple system atrophy: a multicenter study and meta-analysis

“…Several studies have reported that RBD is frequent in patients with MSA [8, 10, 16, 22, 26, 27, 32, 33, 36, 37, 40, 41] but its exact prevalence and whether RBD affects all patients with MSA is not known. Moreover, whether questionnaires are sufficient to diagnose RBD or polisomnography showing abnormal muscle activation (i.e., lack of atonia) and vocalizations/motor behavior during REM is required for the diagnosis is unclear.…”