Objective: To establish the cognitive profile of MSA-P in a Tunisian cohort and to discuss its association with motor signs.

Background: It has been previously admitted that cognition remained preserved in the Parkinsonian variant of multiple system atrophy (MSA-P). Dementia was considered a non supportive feature in consensus diagnostic criteria. Currently, cognitive disorders are increasingly recognized in MSA even at early stages of the disease.

Methods: We conducted a retrospective cross-sectional descriptive study at the Neurology department of Razi Hospital of 12 years (from January 2003 to December 2014), including all patients with MSA-P according to consensus criteria of Gilman (2007). We analyzed clinical and neuropsychological data.

Results: We included 24 patients (sex-ratio=0.8, mean age: 62 years, mean age at onset:56 years[37-74], median MMSE=26, median BREF=14). Dementia was present in 17%, at a mild or moderate stage. Main cognitive domain impaired were attention (79%) and language and speech (71%). Memory impairment was found in 54% of cases and was hippocampal-dependant in 38%. Executive functions were also altered in 54% of cases. Apraxia was found in a quarter of patients. All patients with dementia had parkinsonism at stage 3 of Hoehn and Yahr. No association between the severity of Parkinsonian syndrome and the stage of dementia was established. Behavioral disorders were associated particularly with dystonia.

Conclusions: In our series, dementia was found in the same range of previous studies (17% versus 5-26% in the literature). Frontal-executive dysfunction is the most common presentation. Memory (mainly working memory) and visuospatial functions also may be impaired. Executive and memory functions were equally disturbed; the latter being more frequent than in literature (54% in our series versus 10-48% in literature). Apraxia was also more frequently found than previously reported (8%-10% of MSA-P). Unlike literature, motor signs were not more severe in MSA with dementia in our series. These cognitive impairments may originate from striatofrontal deafferentation. Cognitive disturbances in MSA-P occur across a wide spectrum, from mild single domain deficits to severe impairment in multiple domains, without correlation to motor severity. Dementia, apraxia or memory impairment should not lead to reconsider the diagnosis.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cognitive-profile-in-the-parkinsonian-variant-of-multiple-system-atrophy-findings-from-a-tunisian-cohort/