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Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE. Case Presentation. An 8-year-old Caucasian boy presented with complete external ophthalmoplegia without ptosis, cerebellar ataxia, and a disturbance of consciousness. He had recently recovered from a confirmed Campylobacter jejuni infection. On subsequent laboratory testing he was anti-GQ1b antibody negative. He had a recurrence of diplopia at four-week follow-up. Conclusions. This patient's recurrence of diplopia was treated with a five-week course of oral corticosteroids which did not worsen his condition, and this may be a therapeutic option for similar patients. We will discuss the symptoms and treatment of reported pediatric cases of anti-GQ1b antibody negative cases of MFS and the variation between cases representing a spectrum of illness.

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Purpose The purpose of this study is to assess the intra‐ and interscan repeatability of free‐breathing phase‐resolved functional lung (PREFUL) MRI in stable pediatric cystic fibrosis (CF) lung disease in comparison to static breath‐hold hyperpolarized 129‐xenon MRI (Xe‐MRI) and pulmonary function tests. Methods Free‐breathing 1‐hydrogen MRI and Xe‐MRI were acquired from 15 stable pediatric CF patients and seven healthy age‐matched participants on two visits, 1 month apart. Same‐visit MRI scans were also performed on a subgroup of the CF patients. Following the PREFUL algorithm, regional ventilation (RVent) and regional flow volume loop cross‐correlation maps were determined from the free‐breathing data. Ventilation defect percentage (VDP) was determined from RVent maps (VDPRVent), regional flow volume loop cross‐correlation maps (VDPCC), VDPRVent ∪ VDPCC, and multi‐slice Xe‐MRI. Repeatability was evaluated using Bland–Altman analysis, coefficient of repeatability (CR), and intraclass correlation. Results Minimal bias and no significant differences were reported for all PREFUL MRI and Xe‐MRI VDP parameters between intra‐ and intervisits (all P > 0.05). Repeatability of VDPRVent, VDPCC, VDPRVent ∪ VDPCC, and multi‐slice Xe‐MRI were lower between the two‐visit scans (CR = 14.81%, 15.36%, 16.19%, and 9.32%, respectively) in comparison to the same‐day scans (CR = 3.38%, 2.90%, 1.90%, and 3.92%, respectively). pulmonary function tests showed high interscan repeatability relative to PREFUL MRI and Xe‐MRI. Conclusion PREFUL MRI, similar to Xe‐MRI, showed high intravisit repeatability but moderate intervisit repeatability in CF, which may be due to inherent disease instability, even in stable patients. Thus, PREFUL MRI may be considered a suitable outcome measure for future treatment response studies.

The mathematical model was successful in accommodating a wide range of breathing patterns and drug-device combinations. Furthermore, the model has demonstrated its effectiveness in predicting the amount of aerosol delivered to "normal" subjects. However, challenges remain in predicting deposition in obstructive lung disease.

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