LANCE-ADAMS SYNDROME AFTER PROLONGED HYPOXIA.CASE REPORT.

ANA LUIZA GUILHERMINO PEREIRA, ANA CAROLINA SAWARIS NETO, ISABELLY DE ARRUDA CARDOSO, FERNANDA NICOLI BROCH, LUCAS ALVES DE MOURA, ISABELE SILVEIRA SIERRA

Resumo


Introduction: The patients who survive long periods of brain anoxya, being that because of cardiopulmonary resuscitation, or for acute respiratory insufficiency, usually end with severe neurologic impairment. Lance Adams Syndrome (LAS) is a rare clinical condition that is classified as Chronic Posthypoxic Myoclonus (PHM), characterized by starting days and even weeks after an episode of cardiorespiratory arrest (CPR) and generally complete recovery of conscience state. The purpose of this report was to elucidate the clinical diagnosis aspects of LAS, and its correct treatment. Case description: A 32-year-old male patient was initially hospitalized for orthopedic services at the Santa Casa de Misericórdia Hospital, due to a fracture of the right hand, with half-amputation. During the surgical procedure, the patient presented a large volume of emesis despite having declared fasting, culminating with massive bronchoaspiration and lowering of consciousness level, maintaining hypoxemia even after orotracheal intubation and mechanical ventilation. As a result of such an event, it evolved with hypoxic encephalopathy, coma, reentrant seizures and later with difficult to control myoclonus, remaining in the Intensive Care Center (ICC) for 30 days with orotracheal intubation. Initially treated as a state of Epileptic Status, and currently in use of Levetiracetam, Valproic Acid and Clonazepam to control myoclonus. Discussion: The key areas inflicted in the physiopathology of LAS remain elusive, although it has been related to the loss of serotonin within the inferior olive nucleus, with partial response to serotonergic treatment and low cerebrospinal levels of serotonin metabolites. The electroencephalographic patterns suggest a cortical origin. Myoclonus is an abnormal involuntary movement, triggered by muscle activation or sensory stimuli, relieved during sleep or rest, usually affecting face and limbs, which significantly interferes and impairs the quality of life. Less than 150 cases have been reported in the worldwide medical literature and the failure to recognize it may result in inappropriate anticonvulsant therapy and delayed treatment. Differentiating LAS from other entities allows early diagnosis and properly management, which implies in an improved functional outcome, minimizing disabilities and improving the prognosis. Although little controlled evidence is available, levetiracetam, clonazepam or valproic acid, either alone or in combinations, are reasonably used and still requires scientific validation.

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