Locked-In Syndrome, also known as Enclaustration, Deseferentization, or Enclosure Syndrome, was first defined in 1966 by Plum and Posner. Causes can be grouped into vascular and non-vascular, the former being the most frequent. Clinically this syndrome is characterized by consciousness and the state of watch conserved, but there is quadriplegia, anartria, dysphagia and difficulty to coordinate the ventilatory mechanics, representing pulmonary complications the main cause of death. In most cases, the patient retains vertical eye mobility, so the only method of communication is through eye blinking and vertical eye movements. Next, a case presentation and review of the literature with the most relevant pathophysiological, clinical, diagnostic and therapeutic aspects is done.
Pons
Síndrome de Locked-In: Reporte de Caso y Revisión de Literatura. Locked-In Syndrome: Case Report And Review Of Literature.
Sindrome parkinsoniano “rígido-acinético” debido a mielinosis extrapontina y mielinosis pontina asintomática secundarios a corrección rápida de hiponatremia.
The osmotic demyelinating syndromes are not always restricted to the pons. The extrapontine myelinolysis is seen in 10% of patients with central pontine myelinolysis. We present a 62-year-old woman who developed hyponatremia due to repeated vomiting. An acute akinetic-rigid syndrome followed rapid correction of hyponatremia. Bilateral putaminal and caudate lesions were seen on MRI. There were no clinical features of pontine involvement even though a typical lesion was seen on MRI. She recovered well with only symptomatic treatment. Although the exact pathogenesis is unknown, the most widely accepted hypothesis is that cellular edema secondary to the fluctuating osmotic forces, with rapid correction of hyponatremia, results in fibre-tract compression and demyelination. In contrast to the generally expected poor outcome in this condition, patients do have good recovery with symptomatic treatment and good nursing care.
