The clinical and cognitive spectrum of locked-in syndrome: 1-year follow-up of 100 patients

In patients with locked-in syndrome (LIS), it is not known exactly to what extent cognitive functions are preserved and it is not known exactly how much it has improved. We aimed to examine the clinical and cognitive features of LIS 1 year after stroke. One hundred patients with locked-in syndrome (LIS) were recruited between January 2008 and May 2019 among 8200 patients with ischemic stroke. Patients were classified into two groups as single pontine infarcts (n = 72), and pontine plus multiple ischemic lesions (PMIL) (n = 28). Since the patients had limited motor and verbal response, the cognitive status of the patients in the early and late stages was evaluated with the Short Neuropsychological Questionnaire for Disabled Patients (SNQDP) test. At the onset of stroke, orientation to time and place was normal in 43% of patients with a single infarct compared with 18% of those with PMIL (OR 3.48; 95% CI 1.10-10.18; P = 0.015). There was no sustained visual fixation or tracking in 53% of patients with a single pontine infarct and 82% of those with PMIL (OR 4.12; 95% CI 1.41-12.02; P = 0.005). After 1-year follow-up, there was significant difference between patients with a single infarct and those with PMIL regarding to perception and execution, especially complex command follow (P = 0.042), attention span and concentration (P = 0.30), intelligible verbalization (P = 0.022). There was relatively high incidence (24%) of patients with a "good outcome" (mRS = 3) in those with single infarct. Given as in our study that many patients can show significant improvement in LIS, we recommend aggressive supportive measures, intense physical, speech and cognitive therapy to facilitate interaction with others and the environment.