A pediatric case of locked-in-syndrome

Laura Wulleman, Magali De Roy, Sybille Andries, Christine Bonnier, Leslie Danvoye Published in the journal : April 2020 Category : Pediatrics

Summary :

The locked-in syndrome (LIS) is a rare neurologic disorder, especially in the pediatric population. It is defined by five clinical criteria: (1) persistence of eye opening and presence of vertical eye movements; (2) preserved superior cortical functions; (3) aphonia or severe hypophonia; (4) quadriplegia or quadriparesis; (5) initial communication mode with vertical eye movements or blinking. The LIS should be considered as part of the differential diagnoses appertaining to vegetative states and comas. The most common etiology is a pontine stroke, caused by vertebrobasilar artery thrombosis. We herein describe a pediatric clinical case of this pathology, which represents a challenge in terms of both diagnosis and management.

In this article, we present the etiological factors of ischemic pediatric stroke, a problem with multiple risk factors, with particular attention paid to post-varicella vasculopathy and its therapeutic management.

Key Words

Locked-in-syndrome, pediatric ischemic stroke, varicella

What is already known about the topic?

Rare neurological disorder, especially in the pediatric population Five clinical criteria Three clinical types Etiology: bilateral ventral pontine lesion Ischemic pediatric stroke: a problem with multiple risk factors

What does this article bring up for us?

Clinical description and review of the literature of a rare neurologic disorder, which generally represents a challenge in terms of diagnosis and therapeutic patient care. A reminder of the diagnosis and treatment of post-varicella vasculopathy in childhood, which is common etiology of post-varicella stroke.