Rinshō shinkeigaku = Clinical neurology
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We presented a case of Bickerstaff's brainstem encephalitis. A 50-year-old woman developed semicoma, external ophthalmoplegia, hyporeflexia, extensor plantar responses. A high titer of anti-GQ1b IgG antibody was detected in her acute phase serum. ⋯ Although MRI and CSF showed no abnormality, one-and-a-half syndrome was observed during the clinical course, suggesting involvement of the pontine tegmentum. She received steroid pulse-therapy and symptoms disappeared completely. Our case suggested that anti-GQ1b IgG antibody might relate to the pathogenesis of intramedullary as well as extramedullary lesions.
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In many patients with neuromuscular diseases, respiratory failure is mainly caused by alveolar hypoventilation in their terminal stages. Malnutrition is one of the common and serious problems in patients with chronic respiratory failure. Energy consumption for breathing is remarkably high in respiratory compromised patients, causing subsequent increase of total energy expenditure. ⋯ Since NIPPV doesn't have a closed circuit, there are some difficulties in respiratory management, such as air leakage to stomach and mouth, and airway obstruction. Failure to optimize NIPPV setting against increased CO2 load might cause hypercapnia in this patient. These suggest that evaluation of energy expenditure and design of nutritional program are essential to avoid hypercapnia due to nutritional support.