A - 110 Characterization of Mild Cognitive Impairment in Multiple System Atrophy

Abstract

Abstract Objective Multiple system atrophy (MSA) is a rare neurodegenerative disorder that primarily impacts movement and balance, as well as the autonomic nervous system. While traditionally not associated with cognitive decline, recent evidence indicates cognitive impairment is part of the disease process, typically manifesting as attentional and executive function deficits, alongside learning/memory impairments. This case contributes to MSA literature by describing mild cognitive changes in a patient with predominant autonomic dysfunction. Method A 68-year-old Caucasian man exhibited progressive cognitive changes over 2–3 years, with concerns emerging 5–10 years prior to neurological consultation (e.g., forgetfulness, word-finding difficulties, decline in ability to follow complex instructions). Predominant sensorimotor/physiological changes included chronic constipation, vasovagal episodes, hearing loss, orthostatic hypotension, transient vision loss, and mild dysphagia. Neuroimaging showed nonspecific mild pontine T2/FLAIR hyperintensity. Significant family history of dementia. Results Evaluation showed mostly normal cognitive function with some decline in executive functioning (slowed processing, reduced problem-solving, set loss, task rigidity) and mild memory decline. Despite scores within normal limits, performance likely reflects cognitive change consistent with his subjective experience, possibly masked by strong cognitive reserve (high educational and occupational attainment, research interests, social engagement). Conclusions Although lacking frank impairment, the patient’s history suggests a neurological process (i.e., synucleinopathy such as MSA) including history of REM sleep behavior disorder, autonomic symptoms/conditions, and neuroimaging findings suggestive of brain stem involvement. Neuropsychological evaluation is critical in differentiating MSA from other Parkinsons’s plus syndromes, as it typically presents with fewer cognitive impairments.