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Environment-driven responses in progressive supranuclear palsy

J Ghika1, M Tennis, J Growdon

  • 1Massachusetts General Hospital, Boston 02114, USA.

Journal of the Neurological Sciences
|May 1, 1995
PubMed
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Patients with progressive supranuclear palsy (PSP) exhibit novel environmentally-driven behaviors due to fronto-striatal dysfunction. These include visual grasping and compulsive actions, indicating parietal lobe disinhibition.

Area of Science:

  • Neuroscience
  • Neurology
  • Gerontology

Background:

  • Fronto-striatal dysfunction in neurodegenerative diseases presents with incompletely described neurological signs and behaviors.
  • Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized by fronto-striatal and other brain region degeneration.

Observation:

  • Seven patients with PSP were observed for novel environmentally-driven behaviors.
  • Cognitive deficits, particularly in frontal lobe functions, were noted in all patients.
  • Reduced frontal lobe cerebral perfusion was observed in SPECT scans of 4 out of 5 patients.

Findings:

  • Patients displayed "visual grasping," becoming fixated on environmental stimuli (e.g., TV, mirror) with an inability to shift gaze.
  • Environmental cues, like removing a table or unbuckling a seatbelt, triggered actions (e.g., standing) that were impossible on command.

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  • Compulsive utilization behaviors (e.g., repetitive phone handling) and heightened reflexes (grasp, facial, eyelid opening apraxia) were observed.
  • Stimuli-oriented behaviors, including echolalia and echopraxia, were present.

    • Implications:

    • The observed behaviors suggest a potential parietal lobe disinhibition secondary to fronto-striatal dysfunction in PSP.
    • Understanding these novel behaviors can improve clinical assessment and patient care for fronto-striatal disorders.
    • This research highlights the complex interplay between environmental stimuli and neurological dysfunction in neurodegenerative diseases.