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Markedly asymmetric presentation in multiple system atrophy.

Amit Batla1, Maria Stamelou, Katerina Mensikova

  • 1Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, United Kingdom.

Parkinsonism & Related Disorders
|June 11, 2013
PubMed
Summary
This summary is machine-generated.

Multiple system atrophy with parkinsonism (MSA-P) can rarely mimic Corticobasal syndrome (CBS) by presenting with severe asymmetry and dystonic limb posturing. This highlights the importance of considering MSA-P in atypical parkinsonism cases.

Keywords:
AsymmetryAtypical parkinsonismCorticobasal degenerationCorticobasal syndromeMultiple system atrophyParkinsonism

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Area of Science:

  • Neurology
  • Neurodegenerative Diseases

Background:

  • Multiple system atrophy (MSA) typically features symmetrical parkinsonism unresponsive to levodopa, alongside autonomic, cerebellar, or corticospinal dysfunction.
  • Atypical parkinsonism with significant asymmetry often suggests alternative diagnoses, such as Corticobasal Syndrome (CBS).

Observation:

  • This study details five unique cases initially presenting with pronounced asymmetric parkinsonism and rigid, dystonic limb posturing.
  • These patients later exhibited clinical and/or radiological signs consistent with probable MSA with parkinsonism (MSA-P).

Findings:

  • Patients met the diagnostic criteria for probable MSA-P after a five-year disease progression.
  • Two cases showed characteristic MRI findings, and one case was pathologically confirmed, supporting the MSA-P diagnosis.

Implications:

  • These cases demonstrate that MSA-P can uncommonly manifest with severe asymmetry, dystonic limb movements, and myoclonic jerks.
  • Such presentations may initially lead to a misdiagnosis of CBS, underscoring the need for careful differential diagnosis in atypical parkinsonism.