Locked In Syndrome: Looking Inside It, A Case Report

Authors

  • Asmell Ramos Cabrera Associate Professor AIUWA, Senior Internal Medicine Consultant EFSTH, Medicare ltd., Gambia.
  • Humberto Acosta Pupo
  • Kehinde O Oduntan General Surgeon, Medicare ltd., Gambia.
  • Rainier Paulino General Physician, Medicare Ltd., Gambia.
  • Mayelin Martinez
  • Jennifer Shallop
  • Onyia Onyema

Keywords:

Locked in syndrome, Asmell Ramos,, Rare diseases, Cerebrovascular diseases, Gambia

Abstract

Locked-in syndrome (LIS) is well known to the public, since the masterful description of Alexandre Dumas. Additionally, Émile Zola reflected this condition in one of his characters, and later, others did as well [1]. In medical practice it is considered a rare neurological disease, with an incidence and a prevalence that is difficult to determine. It affects both sexes and is more common in adults, basically as a complication of cerebrovascular diseases (CVD). Patients with classic LIS develop quadriplegia, but normally remain conscious and can communicate by blinking, constituting the most dramatic presentation of brain stem involvement. To diagnose it is challenging because of its similarity to conditions such as akinetic mutism, coma, persistent vegetative state, psychogenic pseudocoma, or brain death. There have been cases reported that have taken months and even years to establish a definitive diagnosis. The CVD incidence rate in Africa is among the highest incidence in the world, with West Africa (WA) being the largest. Paradoxically, no reports of LIS associated with CVD have been found in the subregion. Our goal is to document the first case in WA of a patient with classic-type locked-in syndrome, secondary to acute pontine ischemic infarction, associated to severe vitamin B deficiency, and accompanying with acute osmotic demyelination syndrome. Which was a result of the intensive correction of severe hyponatremia. A case that is difficult to manage and complex to diagnose, which makes it especially interesting for our professionals. Recognizing the disease will avoid delays in diagnosis and facilitate early intervention, reducing associated complications and mortality, which remains high in the initial phases. Then, together with a multidisciplinary team, achieve the best possible social reintegration of patients, knowing in advance the serious limitations they will face in their future

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Published

2024-05-01

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Articles