Clinical Characteristics and Outcome in the Acute Phase of Ischemic Locked-In Syndrome: Case Series of Twenty Patients with Ischemic LIS

Abstract

Background: Locked-in syndrome (LIS) is a condition characterized by quadriplegia and anarthria. The most common cause is a ventral pontine lesion due to atherosclerotic basilar artery disease. Methods: Cases with LIS were prospectively identified among the patients with acute ischemic stroke over 3 years, between 2009 and 2011. Clinical characteristics, topographic localization of lesions, and outcome were determined during the first 6 months from onset of LIS. Results: Our case series consists of 20 patients (mean age 62 ± 10 years; range 46–82). Initially 16 patients had a reduced level of consciousness (mean 3 days; range 1–15). Respiratory disturbance, mainly as impairment of the breathing pattern, was noted in all cases. Five patients died within the first 10 days due to stroke progression or cardiac arrest. In the remaining cases the most frequent causes of death were pulmonary infections and sepsis. Overall mortality in the acute phase of LIS is 75%, and the median survival time is 42 days. There was a statistically significant association between the more extensive parenchymal brain stem lesions and observed mortality. Conclusions: Ischemic LIS is commonly caused by an acute complete occlusion of the basilar artery due to atherosclerotic lesions in intracranial vertebrobasilar vessels. Mortality remains high in the acute phase of the disease.

Posterior Circulation Ischemia or Occlusion in Five Adults With Failing Fontan Circulation

“…The preservation of consciousness with paralysis of all movement except for lateral gaze indicated injury to both corticospinal and corticobulbar tracts and the abducens nerves, consistent with a locked-in syndrome produced by injury in the ventral pons with preservation of function of the reticular activating system [14].…”

“…The outcome from posterior circulation strokes in adults is poor [14]. Since 1990 at our center, 139 Fontan conversions have been performed.…”

A Rare Sequela of Acute Disseminated Encephalomyelitis

“…Less frequently, basilar artery dissection, brainstem hemorrhage, central pontine myelinolysis, and primary or secondary malignant infiltration of the basis pontis can also cause LIS [8]. Other less common causes of LIS include brainstem encephalitis, polyneuritis, myasthenia gravis, poliomyelitis, EBV, pneumococcal meningitis, and West Nile viral infection [9].…”

Locked‐in syndrome caused by bilateral midbrain infarctions with occlusion of the intracranial vertebral artery

“…Locked‐in syndrome is a well‐known neurological status involving signs such as quadriplegia and supranuclear bulbar palsy without disturbance of consciousness. Most reported cases of locked‐in syndrome have been caused by ventral pontine infarction, although locked‐in syndrome with bilateral cerebral peduncle infarctions has also been described . Bilateral cerebral peduncle infarctions usually result from basilar artery (BA) occlusion (BAO) .…”