Locked-in syndrome revisited

Author:

Schnetzer Laura1234ORCID,McCoy Mark5,Bergmann Jürgen5,Kunz Alexander53,Leis Stefan5,Trinka Eugen523

Affiliation:

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

2. MRI Research Unit, Neuroscience Institute, Christian Doppler Medical Centre, Paracelsus Medical University, Salzburg, Austria

3. Karl Landsteiner Institute of Neurorehabilitation and Space Neurology, Salzburg, Austria

4. Spinal Cord Injury and Tissue Regeneration Centre, Paracelsus Medical University, Salzburg, Austria

5. Department of Neurology, Neurological Intensive Care and Neurorehabilitation, Christian Doppler Medical Centre, Paracelsus Medical University, Salzburg, Austria

Abstract

The locked-in syndrome (LiS) is characterized by quadriplegia with preserved vertical eye and eyelid movements and retained cognitive abilities. Subcategorization, aetiologies and the anatomical foundation of LiS are discussed. The damage of different structures in the pons, mesencephalon and thalamus are attributed to symptoms of classical, complete and incomplete LiS and the locked-in plus syndrome, which is characterized by additional impairments of consciousness, making the clinical distinction to other chronic disorders of consciousness at times difficult. Other differential diagnoses are cognitive motor dissociation (CMD) and akinetic mutism. Treatment options are reviewed and an early, interdisciplinary and aggressive approach, including the provision of psychological support and coping strategies is favoured. The establishment of communication is a main goal of rehabilitation. Finally, the quality of life of LiS patients and ethical implications are considered. While patients with LiS report a high quality of life and well-being, medical professionals and caregivers have largely pessimistic perceptions. The negative view on life with LiS must be overthought and the autonomy and dignity of LiS patients prioritized. Knowledge has to be disseminated, diagnostics accelerated and technical support system development promoted. More well-designed research but also more awareness of the needs of LiS patients and their perception as individual persons is needed to enable a life with LiS that is worth living.

Funder

Paracelsus Medizinische Privatuniversität

Publisher

SAGE Publications

Subject

Neurology (clinical),Neurology,Pharmacology

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