Locked-In Syndrome: A Systematic Review of Long-Term Management and Prognosis

Abstract

Locked-in syndrome (LIS) is a neurological disorder in which there is damage to the ventral pons and caudal midbrain. An ischemic cause, such as basilar artery occlusion, can often lead to LIS. LIS has three subtypes: classical, partial, and total. There is loss of motion in the four extremities in classical LIS, loss of horizontal gaze, and aphasia. In partial LIS, the patient still has some motor function. Complete LIS has the worst outcome because patients cannot blink or have vertical gaze, thus rendering them incapable of communicating. Most cases of LIS occur due to ischemic infarcts. These patients require a great deal of physical rehabilitation to regain partial motor ability and a means to communicate. While the clinical features and pathophysiology are known, the prognosis and long-term treatment remain unknown.We conducted a systematic review using the Meta-Analysis Of Observational Studies in Epidemiology (MOOSE) protocol. We use an advanced PubMed strategy using the inclusion criteria of observational studies or clinical trials conducted in the last 20 years, written in English, and conducted on humans. We excluded systematic reviews, literature reviews, metanalysis, and studies that did not meet the outcomes of our objectives.The prognosis of LIS is not good, and most patients remain locked in, with poor quality of life, especially motor functions. Respiratory failure and depression are big comorbidities. In the acute setting, patients benefit from rapid intervention. The subacute treatment needs to manage aggressively to improve functional scores best. The long-term treatment focus is on the quality of life and managing comorbidities.

Locked In Syndrome, a Rare Clinical Presentation: A Case Report

“…Corticobulbar tracts passing through the midbrain and pons contain upper motor neurons for 5, 7 and 9-12 cranial nerves. 5 Pontine respiratory centers are also affected leading to apnea and respiratory distress. RAS responsible for consciousness lies dorsally and does not affect by ventral pontine lesion.…”

Understanding the Psychological Well-being of Patients With Locked-in Syndrome: A Scoping Review

“…However, most research has been based on small sample sizes due to the rarity of the condition, and studies have varied in the measures and approach used for QoL assessment as well as the etiology of LiS targeted for examination. In a recent systematic review on the prognosis and management of LiS patients, Halan et al described that LiS patients had poor QoL, but noted this to be due to motor function disability which may be separate from depressive and psychiatric ailments of LiS [ 7 ]. Given the lack of cumulative knowledge on the psychological well-being of LiS patients, there is a need to summarize what is known based on current evidence from the scientific literature, including an understanding of the types of psychological and QoL instruments and measures used across LiS patients of varying etiology.…”

Locked-in syndrome responding to endovascular treatment

“…Several case studies have reported the prognosis of this illness, the largest of which showed a severely disabled state or death. 5 However, there have been several reports of patients with substantial clinical improvement, which suggests that early stroke treatment, such as endovascular mechanical thrombectomy, intravenous thrombolysis, and intra-arterial thrombolysis with tissue plasminogen activator, may enhance the possibility of a greater recovery for patients with LiS secondary to acute basilar artery occlusion (ABAO). 6 7 To our knowledge, information on rapid endovascular treatment (EVT) for patients with LiS is also scarce, especially regarding the short-and long-term prognoses for these patients.…”

“…Although the clinical features, diagnosis, and classification of LiS have been established, its treatment and prognosis remain largely unknown. Several case studies have reported the prognosis of this illness, the largest of which showed a severely disabled state or death 5. However, there have been several reports of patients with substantial clinical improvement, which suggests that early stroke treatment, such as endovascular mechanical thrombectomy, intravenous thrombolysis, and intra-arterial thrombolysis with tissue plasminogen activator, may enhance the possibility of a greater recovery for patients with LiS secondary to acute basilar artery occlusion (ABAO) 6 7…”