Multiple system atrophy (MSA) is a rare, fatal, rapidly progressing neurodegenerative disorder of uncertain etiology that is clinically characterized by a variable combination of parkinsonism, cerebellar impairment, autonomic dysfunction and pyramidal tract signs. The mean age of disease onset is 56±9 years with poor prognosis and a mean survival of 9.5 years. The prevalence is 1.9 to 4.9 cases...

OBJECTIVE Can dysautonomic symptoms occurring within a year of developing motor symptoms distinguish Multiple system atrophy-Parkinsonian (MSA-P) from Parkinson's disease (PD)? PATIENTS AND METHODS Seventy-two Parkinsonian patients diagnosed as probable PD or MSA-P. RESULTS PD (n = 58, 80.6%) and MSA (n = 14, 19.4%) patients were of similar age and had motor symptoms for similar duration. P...

Current clinical diagnostic tools are limited in their ability to accurately differentiate idiopathic Parkinson's disease (PD) from multiple system atrophy (MSA) and other parkinsonian disorders early in the disease course, but eye movements may stand as objective and sensitive markers of disease differentiation and progression. To assess the use of eye movement performance for uniquely charact...

BACKGROUND Multiple system atrophy (MSA) is diverse in clinical phenotype, disease progression, and prognosis. Sudden death is a leading cause of death in patients with MSA. OBJECTIVE To determine what clinical factors affect the progression and survival prognosis of those with MSA. DESIGN A retrospective review of the medical records of 49 consecutive Japanese patients with pathologically ...

BACKGROUND Formal laboratory testing of autonomic function is reported to distinguish between patients with Parkinson's disease and those with multiple system atrophy (MSA), but such studies segregate patients according to clinical criteria that select those with autonomic dysfunction for the MSA category. OBJECTIVE To characterise the profiles of autonomic disturbances in patients in whom th...

The clinical features of progressive supranuclear palsy (PSP) overlap with other parkinsonian syndromes, including multiple system atrophy (MSA). Autonomic dysfunction is a characteristic of MSA, but has also been described in PSP. We therefore report results from a series of physiological studies of cardiovascular autonomic function in 35 PSP and 20 MSA subjects, and 26 age-matched healthy con...

OBJECTIVE To determine ways to improve diagnostic accuracy of multiple system atrophy (MSA), we assessed the diagnostic process in patients who came to autopsy with antemortem diagnosis of MSA by comparing clinical and pathologic features between those who proved to have MSA and those who did not. We focus on likely explanations for misdiagnosis. METHODS This is a retrospective review of 134 ...

BACKGROUND External anal sphincter (EAS) electromyography (EMG) abnormalities can distinguish multiple system atrophy (MSA) from Parkinson's disease in the first five years after disease onset. However, the prevalence of the abnormalities in the early stages of MSA is unknown. OBJECTIVES To present EAS-EMG data in the various stages of MSA. METHODS 84 patients with "probable" MSA were recru...

Multiple system atrophy (MSA) is a rare, yet fatal neurodegenerative disease that presents clinically with autonomic failure in combination with parkinsonism or cerebellar ataxia. MSA impacts on the autonomic nervous system affecting blood pressure, heart rate and bladder function, and the motor system affecting balance and muscle movement. The cause of MSA is unknown, no definitive risk factor...

Hot cross bun sign refers to the cruciform-shaped hyperintensity on T2W axial magnetic resonance images (MRI) in multisystem atrophy due to the selective loss of myelinated transverse pontocerebellar fibers and neurons in the pontine raphe and sparing of the pontine tegmentum and corticospinal tracts [Figure 1, Figure 2].[1] The name derives from a sweet spiced bun baked by the Christian church...