Cognitive impairment in cerebellar phenotype of multiple system atrophy and its association with hot-cross bun sign

Abstract

BACKGROUND: Cognitive impairment has been reported in patients with cerebellar phenotype of multiple system atrophy (MSA-C), yet this area remains largely unexplored. OBJECTIVE: We evaluated the cognitive profile of MSA-C patients and assessed its association with neuroimaging features, specifically the hot-cross bun (HCB) sign. METHODS: This retrospective study encompassed 210 patients diagnosed with MSA-C between 2019 and 2023. Each patient underwent a thorough clinical evaluation, a comprehensive neuropsychological assessment and a magnetic resonance imaging (MRI) scan. RESULTS: MSA-C patients demonstrated a high burden of comorbidities, including a high frequency of motor symptoms, autonomic disorders, and various non-motor symptoms such as cognitive deficits, mood disorders and sleep disturbances. Notably, 24.8% of MSA-C patients exhibited cognitive impairment, primarily affecting language, memory and processing speed. The presence of the HCB sign was independently associated with cognitive impairment, whereas other clinical features, such as orthostatic hypotension, were not. MSA-C patients with the HCB sign demonstrated significantly poorer performance in language, visuospatial function, and processing speed. CONCLUSIONS: MSA-C patients suffer a substantial burden of cognitive impairment, primarily affecting language, memory and processing speed. Notably, the HCB sign is a significant indicator for cognitive dysfunction in patients with MSA-C.