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Updated: Jul 23, 2026

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Locked-in syndrome revisited.

Laura Schnetzer1,2,3,4, Mark McCoy5, Jürgen Bergmann5

  • 1Department of Neurology, Neurological Intensive Care and Neurorehabilitation, Christian Doppler Medical Centre, Paracelsus Medical University, Ignaz-Harrer-Straße 79, A-5020 Salzburg, Austria.

Therapeutic Advances in Neurological Disorders
|April 3, 2023
PubMed
Summary

Locked-in syndrome (LiS) involves paralysis with preserved cognition. Despite patient-reported high quality of life, a shift in perception is needed to prioritize autonomy and dignity.

Keywords:
brainstemcognitive functionconsciousnessdifferential diagnosisethicslesionponsquadriplegiaquality of lifetreatment

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Area of Science:

  • Neurology
  • Neuroscience
  • Rehabilitation Medicine

Background:

  • Locked-in syndrome (LiS) presents as quadriplegia with intact consciousness and cognition, distinguished by preserved vertical eye movements.
  • Understanding the anatomical basis in the pons, mesencephalon, and thalamus is crucial for differentiating LiS subtypes and related conditions.
  • Differential diagnoses include cognitive motor dissociation and akinetic mutism, complicating clinical assessment.

Purpose of the Study:

  • To review the subcategorization, etiologies, and anatomical underpinnings of Locked-in syndrome.
  • To discuss current treatment strategies, emphasizing early, interdisciplinary, and aggressive rehabilitation.
  • To explore the quality of life and ethical considerations for LiS patients, contrasting patient perspectives with those of healthcare professionals.

Main Methods:

  • Literature review of LiS subcategorization, etiologies, and neuroanatomical correlates.
  • Analysis of treatment options, rehabilitation goals, and communication strategies.
  • Examination of quality of life reports and ethical implications, including patient-reported outcomes and professional perceptions.

Main Results:

  • LiS is categorized into classical, complete, incomplete, and locked-in plus syndromes based on neuroanatomical damage.
  • An early, interdisciplinary approach focusing on communication and psychological support is recommended for rehabilitation.
  • A significant disparity exists between LiS patients' high self-reported quality of life and the generally pessimistic views of medical professionals and caregivers.

Conclusions:

  • Prioritizing patient autonomy and dignity is essential, challenging negative perceptions of life with LiS.
  • Disseminating knowledge, accelerating diagnostics, and promoting technical support are vital for improving care.
  • Further research and increased awareness are necessary to ensure a fulfilling life for individuals with LiS.