A “hot crossed buns” sign, orthostatic syncope & gait ataxia point to probable multiple systems atrophy with dysarthria and slowed fluency suspicious for associated cognitive impairment

Abstract

Multiple systems atrophy (MSA) describes a group of rare neurodegenerative disorders classified as Parkinsonism predominant (MSA-P) or cerebellar ataxia (MSA-C) subtypes. Parkinsonism or cerebellar ataxia is frequently the initial motor symptom; it is often accompanied by autonomic dysregulation presenting as orthostatic hypotension. MSA is a clinical diagnosis. Three levels of diagnostic certainty are recognized. A diagnosis of “definite MSA” requires autopsy confirmation. A “probable” diagnosis has a sporadic and progressive adult-onset with prominent dysautonomia and either motor symptoms which are poorly responsive to levodopa, or cerebellar ataxia. A diagnosis of “possible” MSA requires at least one feature suggesting autonomic dysfunction plus another feature, which is either determined on clinical grounds or via neuroimaging. The case described here is a case of adult onset recurrent syncope, unambiguous orthostatic hypotension, progressively worsening gait ataxia, slowed fluency, and dysarthria with cognition remaining grossly intact. Axial T2-weighted imaging depicted hyper-intense enhancement forming a cross, indicative of degeneration of pontocerebellar tracts (“hot cross bun sign”).

Keywords

• hot crossed bun sign
• multiple systems atrophy
• dysarthria
• slowed fluency
• cognitive impairment
• orthostatic hypotension