Cognitive impairment in multiple system atrophy: A position statement by the neuropsychology task force of the MDS multiple system atrophy (MODIMSA) study group

Abstract

Consensus diagnostic criteria for multiple system atrophy consider dementia as a non-supporting feature, despite emerging evidence demonstrating that cognitive impairments are an integral part of the disease. Cognitive disturbances in multiple system atrophy occur across a wide spectrum from mild single domain deficits to impairments in multiple domains and even to frank dementia in some cases. Frontal-executive dysfunction is the most common presentation, while memory and visuospatial functions may also be impaired. Imaging and neuropathological findings support the concept that cognitive impairments in MSA originate from striatofrontal deafferentation with additional contributions from intrinsic cortical degeneration and cerebellar pathology. Based on a comprehensive evidence-based review we here propose future avenues of research that may ultimately lead to diagnostic criteria for cognitive impairment and dementia associated with multiple system atrophy.

Diagnosing dementia in multiple system atrophy by applying Movement Disorder Society diagnostic criteria for Parkinson's disease dementia

“…Cerebellar atrophy in MSA may also contribute to cognitive impairment through deafferentiation of fronto-cerebellar loops [6]. Finally, abnormalities in frontal, temporal and parietal processing due to important neuronal loss and abundant glial cytoplasmic inclusions may also contribute to cognitive impairment [6,12].…”

“…Moreover, dementia has been reported in MSA patients with a prevalence ranging between 14 and 18.2% [4,5]. In view of the increasing body of evidence for cognitive impairment in MSA, the Neuropsychology Task Force of the International Parkinson and Movement Disorder Society (MDS)-sponsored MSA Study Group has called in a recent position statement for diagnosis criteria for cognitive impairment and dementia associated with MSA [6].…”

Cognitive Impairment and Its Structural Correlates in the Parkinsonian Subtype of Multiple System Atrophy

“…Several hypotheses for the background mechanism of cognitive dysfunction in MSA have been proposed according to neuropathological considerations [29]. With prominent nigrostriatal degeneration and secondary disruption of striatocortical circuits [30], the concept of subcortical dementia may explain the cognitive features of MSA.…”

Recent advances in neuropathology, biomarkers and therapeutic approach of multiple system atrophy

“…According to consensus criteria, dementia (as defined by DSM-IV criteria) is a non-supporting feature for a clinical diagnosis of MSA2; however, a position statement by the Neuropsychology Task Force of the Movement Disorders Society MSA study group indicates that cognitive impairment may be an under-recognised feature in MSA (reviewed in ref 62). The frequency of cognitive impairment varies depending on the study design and sample size, but ranges from 14% to 37% 1 18 34 63 64.…”