Abstract
Multiple system atrophy (MSA) is characterized by a variable combination of cerebellar ataxia, parkinsonism and pyramidal signs associated with autonomic failure. Classically, cognitive impairment was not considered a clinical feature of MSA and dementia was pointed out as an exclusion diagnostic criteria. Based on comprehensive neuropsychological assessment, cognitive impairment was found to be a frequent feature in MSA, and clinically-defined dementia is now reported in 14-16% of cases. This article reviews the current data on cognitive impairment in MSA along with its neuropsychological profile and pathophysiology.
Highlights
Multiple system atrophy (MSA) is characterized by a variable combination of cerebellar ataxia, parkinsonism and pyramidal signs associated with autonomic failure
Robust data shows that cognitive impairment in MSA is a frequent feature and should be actively pursued
MMSE and DSM-IV criteria for dementia, common tools used in initial clinical evaluation, are not sensitive enough to detect these abnormalities in MSA patients
Summary
Multiple system atrophy (MSA) is characterized by a variable combination of cerebellar ataxia, parkinsonism and pyramidal signs associated with autonomic failure. The density of GCIs containing α-synuclein correlates significantly with neuronal deterioration and disease duration. The earliest description of sporadic cases presenting with cerebellar ataxia and urinary incontinence dates from 1900 and was reported by Dejerine and Thomas They introduced the term olivopontocerebellar atrophy for these neurological conditions.[4] In the years that followed, the concepts of postural hypotension and dysautonomia were incorporated by Shy and Drager, who described a syndrome characterized by orthostatic hypotension, anhidrosis, urinary and fecal incontinence, as well as sexual impotence associated with variable neurological involvement.[5,6] In the 1960s, cases presenting predominantly with parkinsonism were described as striatonigral degeneration.[7,8] the term MSA was proposed by Graham and Oppenheimer to encompass the three conditions above.[9]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
