Abstract
Creutzfeld-Jakob disease, is a rare neurodegenerative entity, is the most common form of prion diseases recognized in humans, compromises the cortical area of the brain, more specifically the cortex of the frontal, parietal, temporal and hippocampus lobes; it has a rapidly progressive course of an incurable nature. Its low prevalence in the general population, makes it difficult to arrive at a certain and early diagnosis, which can lead the medical team to establish an interdisciplinary management plan with the goal of improves the quality of life of these patients and their family; there are a broad difficult-to-control symptom between them the delirium that physically and emotionally impairs the patient, their loved ones and caregivers is common. Case presentation: 60-year-old patient with prion disease with cognitive impairment, oral intolerance, malnutrition, dehydration, myoclonus and insomnia; presenting multifactorial etiology delirium, very difficult to control, requiring management with atypical and typical neuroleptics, reaching the maximum therapeutic dose without adequate control, in the face of refractory delirium it was necessary to manage with palliative sedation, achieving symptomatic control and death at home. Conclusions: In case of Creutzfeld-Jakob dementia with difficult-to-control symptomatology, in particular in the face of refractory delirium, palliative sedation is a management option that can be performed at home by a trained team, achieving adequate results.
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