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Related Experiment Video

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Alternating flexed-extended posturing in progressive supranuclear palsy.

Nobuyuki Ishii1, Hitoshi Mochizuki1

  • 1Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, University of Miyazaki, Japan.

Neurology International
|October 4, 2019
PubMed
Summary

Late-stage progressive supranuclear palsy (PSP) can manifest unusual "alternating flexed-extended posturing." This complex motor symptom may indicate pathological progression in advanced PSP cases.

Keywords:
abnormal posturingdecerebrate statedecorticate statedystoniaprogressive supranuclear palsy

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Area of Science:

  • Neurology
  • Neuroscience
  • Geriatrics

Background:

  • Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease.
  • Late-stage PSP is characterized by severe motor impairments and cognitive decline.
  • Complex posturing is an atypical motor manifestation observed in some neurodegenerative conditions.

Purpose of the Study:

  • To describe a unique case of "alternating flexed-extended posturing" in a patient with advanced progressive supranuclear palsy (PSP).
  • To explore the potential mechanisms and significance of this complex posturing in the context of PSP.
  • To highlight this presentation as a potential phenotype of pathological progression in PSP.

Main Methods:

  • Case report of a 69-year-old male patient with a 5-year history of PSP.
  • Clinical examination noting neck dystonia and "alternating flexed-extended posturing."
  • Brain magnetic resonance imaging (MRI) to assess cerebral atrophy.

Main Results:

  • The patient presented with aspiration pneumonia and exhibited "alternating flexed-extended posturing" alongside neck dystonia.
  • Brain MRI revealed global cerebral atrophy, predominantly affecting the cortex and midbrain.
  • The observed posturing suggests a complex interplay of decortication, decerebration, and dystonia.

Conclusions:

  • "Alternating flexed-extended posturing" may represent a distinct phenotype in the pathological progression of late-stage PSP.
  • Understanding these complex motor phenomena is crucial for managing advanced PSP.
  • This case underscores the diverse and severe motor manifestations possible in PSP.