Multiparametric neuromonitoring was performed in a 60-year-old patient after heart transplantation and respiratory insufficiency following encephalitis. Abnormal findings were seen in the brainstem auditory evoked potential interpeak latencies (IPL) at the level of the mid to upper brain stem (wave IV-V IPL), which were significantly increased on both sides. This neurophysiologic abnormality co...

conclusions in summary, there is a lack of consensus about the use of neuromuscular blockers in patients with ibm. the authors avoided these drugs and were able to easily secure the patient’s airway and maintain adequate muscle relaxation using a balanced sevoflurane-remifentanil anesthetic. clinical trials are necessary to define the pharmacology of neuromuscular blockers in patients with ibm ...

STUDY OBJECTIVES To evaluate the pulmonary manifestations and the course of acute respiratory insufficiency associated with lysinuric protein intolerance (LPI). DESIGN Retrospective review of clinical data and chest radiographs (total 225) obtained during the lifetime follow-up of 31 LPI patients. About half of the 25 patients without respiratory symptoms underwent high-resolution computed to...

AIM To investigate the efficacy of nasal high frequency ventilation (nHFV) in newborn infants with moderate respiratory insufficiency. METHOD Twenty one preterm and term neonates were treated with nHFV for respiratory insufficiency. Criteria for starting nHFV were: deterioration on nasal CPAP expressed by a median pH of 7.24 and pCO2 of 8.3 kPa, or increasing FIO2. nHFV was delivered using th...

MECP2 mutations mainly occur in females with Rett syndrome. Mutations have been described in 11 boys with progressive encephalopathy: seven of nine with affected sisters and two de novo. The authors report four de novo occurrences: three pathogenic and one potentially pathogenic. Common features include failure to thrive, respiratory insufficiency, microcephaly, and abnormal motor control. MECP...