Abstract

Objective: Multiple system atrophy (MSA) is a neurodegenerative disorder manifesting as parkinsonism, cerebellar ataxia, and autonomic dysfunction. It is categorized into MSA with predominant parkinsonism (MSA-P) and into MSA with predominant cerebellar ataxia (MSA-C). The pathophysiology of motor control circuitry involvement in MSA subtype is unclear. Bereitschaftspotential (BP) is a feasible clinical tool to measure electroencephalographic activity prior to volitional motions. We recorded BP in patients with MSA-P and MSA-C to investigate their motor cortical preparation and activation for volitional movement.Methods: We included eight patients with MSA-P, eight patients with MSA-C, and eight age-matched healthy controls. BP was recorded during self-paced rapid wrist extension movements. The electroencephalographic epochs were time-locked to the electromyography onset of the voluntary wrist movements. The three groups were compared with respect to the mean amplitudes of early (1,500–500 ms before movement onset) and late (500–0 ms before movement onset) BP.Results: Mean early BP amplitude was non-significantly different between the three groups. Mean late BP amplitude in the two patient groups was significantly reduced in the parietal area contralateral to the movement side compared with that in the healthy control group. In addition, the late BP of the MSA-C group but not the MSA-P group was significantly reduced at the central parietal area compared with that of the healthy control group.Conclusions: Our findings suggest that patients with MSA exhibit motor cortical dysfunction in voluntary movement preparation and activation. The dysfunction can be practicably evaluated using late BP, which represents the cerebello-dentato-thalamo-cortical pathway.

Highlights

  • Multiple system atrophy (MSA) is an adult-onset and progressive neurodegenerative disease

  • It is classified based on the motor phenotypes of striatonigral degeneration (SND), currently designated as MSA-parkinsonism type (MSA-P), and olivopontocerebellar atrophy (OPCA), currently designated as MSA-cerebellar type (MSA-C) [3]

  • MSA is a synucleinopathy in which the misfolded alpha-synuclein accumulates in the cytoplasmic inclusions of oligodendrocytes; this is different from Parkinson’s disease (PD), in which alpha-synuclein aggregates in the central nervous system neurons [7,8,9]

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Summary

Introduction

Multiple system atrophy (MSA) is an adult-onset and progressive neurodegenerative disease. MSA is characterized by a combination of autonomic dysfunction, parkinsonism, cerebellar, and pyramidal features [1, 2]. It is classified based on the motor phenotypes of striatonigral degeneration (SND), currently designated as MSA-parkinsonism type (MSA-P), and olivopontocerebellar atrophy (OPCA), currently designated as MSA-cerebellar type (MSA-C) [3]. Despite sharing some parkinsonian features, MSA exhibits a distinct clinical course and symptom spectrum from PD [17, 18]. Whether and how the underlying pathophysiology of MSA leads to motor dysfunction remains to be investigated

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