Malnutrition-related hyperammonemic encephalopathy presenting with burst suppression: a case report.

BACKGROUND: Hyperammonemia is a common cause of metabolic encephalopathy, mainly related to hepatic cirrhosis. Numerous nonhepatic etiologies exist but they are infrequent and not well known, thus, leading to misdiagnosis and inadequate care. Electroencephalography has a proven diagnostic and prognostic role in comatose patients. Burst suppression is a preterminal pattern found in deep coma states and is rarely associated with metabolic causes. CASE PRESENTATION: We report the case of an 81-year-old Caucasian man presenting with rapidly progressive somnolence and mutism. Soon after his arrival in our hospital, he developed profound coma. A comprehensive diagnostic workup was unremarkable except for admission electroencephalography showing diffuse slowing of cerebral activity with an intermittent pattern of burst suppression. He was admitted to our intensive care unit for supportive care where malnutrition-related hyperammonemia was diagnosed. His clinical course was spontaneously favorable and follow-up electroencephalography demonstrated normal cerebral activity. CONCLUSIONS: Nonhepatic hyperammonemia is a rare and potentially reversible cause of encephalopathy. Ammonia level measurement should be part of the diagnostic workup in patients with unexplained coma, particularly in the setting of nutritional deficiencies or nutritional supply. Detection of diffuse and nonspecific mild to moderate slowing of cerebral activity (theta-delta ranges) on electroencephalography is common. In contrast, to the best of our knowledge, burst suppression has never been described in association with hyperammonemia.

[Creating a stroke unit in a community hospital improves the management of stroke patients]. / Création d'une « stroke unit ¼ dans un hôpital de zone : une optimisation de la prise en charge des patients AVC.

Dramatic advances in recent years have changed the care of stroke patients. Creation of stroke hubs with centers and units achieved a significant reduction in mortality and morbidity. The indications for acute therapies such as intravenous thrombolysis and endovascular treatment target an increasing proportion of stroke patients. Early rehabilitation and multidisciplinary approaches have become part of the gold standard in stroke care. The creation of additional stroke units and strengthening local synergies should continue in order to propose the best medical treatments for all stroke patients.

La prise en charge des patients souffrant d'accident vasculaire cérébral (AVC) a connu des avancées spectaculaires dans les dernières années. Le développement de réseaux de « stroke units ¼ et « stroke centers ¼ a permis une nette diminution de mortalité et morbidité. Les indications aux traitements aigus, tels que la thrombolyse intraveineuse et le traitement endovasculaire, visent une proportion croissante de patients. La rééducation précoce et la prise en charge multidisciplinaire font désormais partie des traitements de référence. Les efforts de création d'unités spécialisées et le renforcement des synergies locales devront être poursuivis afin de proposer les meilleurs traitements disponibles à l'ensemble de la population.

Death after cessation of treatment by cystic fibrosis patients: An international survey of clinicians.

BACKGROUND: Little is known about cystic fibrosis patients, who are not considered to be terminally ill, and who die after voluntary cessation of treatment. AIM: This study was undertaken to provide an international snapshot of this issue. DESIGN: An online survey was distributed across three continents. SETTING: Distribution to the medical directors of the cystic fibrosis centres affiliated with the US Cystic Fibrosis Foundation, Cystic Fibrosis Australia (inclusion of New Zealand) and to every clinician member of the European Cystic Fibrosis Society. RESULTS: More than 200 cystic fibrosis patients not considered to be terminally ill and, who voluntarily ceased treatment, were reported by the clinicians surveyed. Detailed data were reported in 102 patients (4 children, 25 adolescents and 73 adults). Only one child, six adolescents and one adult were judged by clinicians not to be competent to make the decision to stop treatment. Time-consuming and low immediate-impact therapies, such as respiratory physiotherapy, were most frequently discontinued. Resignation was the main reported reason for discontinuing treatment, followed by reactive depression and lack of familial support. A total of 69% of the patients received palliative care and 72% died in the 6 months following cessation of treatment. CONCLUSION: Death of cystic fibrosis patients, not considered to be terminally ill, is reported in Europe, the United States and Australia due to voluntary cessation of treatment.