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Review
. 2023 Mar 29:16:17562864231160873.
doi: 10.1177/17562864231160873. eCollection 2023.

Locked-in syndrome revisited

Laura Schnetzer  1   2   3   4 , Mark McCoy  5 , Jürgen Bergmann  5 , Alexander Kunz  5   3 , Stefan Leis  5 , Eugen Trinka  5   2   3
Affiliations
Review

Locked-in syndrome revisited

Laura Schnetzer et al. Ther Adv Neurol Disord. .

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.

Keywords: brainstem; cognitive function; consciousness; differential diagnosis; ethics; lesion; pons; quadriplegia; quality of life; treatment.

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Conflict of interest statement

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
MRI of a patient with locked-in syndrome due to basilar thrombosis showing lesions involving the ventral pons (arrow).
Figure 2.
Figure 2.
Regions of the brainstem and thalamus which can be lesioned in classical LiS, complete LiS and LiPS, including the most important structures causing the symptoms and function losses. ARAS, ascending reticular activating system; LiPS, locked-in plus syndrome; LiS, locked-in syndrome; MLF, medial longitudinal fasciculus; PPRF, paramedian pontine reticular formation.
Figure 3.
Figure 3.
MRI of a patient with locked-in plus syndrome due to basilar artery thrombosis, the arrows showing the lesions involving the brainstem (pons and mesencephalon) and thalamus.
See this image and copyright information in PMC

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