We describe an identical syndrome of cystic leukoencephalopathy in three Turkish children, including two siblings. The neurological findings were noted within the first months of life and include severe intellectual impairment, motor retardation, and spasticity. Magnetic resonance imaging of the brain showed extensive cysts within the anterior temporal lobes, ventricular enlargement and white matter disease. The signal intensities of the cysts' content were identical to those of the cerebrospinal fluid. The patients' screening for known inborn errors of metabolism, especially those characterised by white matter involvement, did not reveal any abnormality. The clinical picture and the magnetic resonance imaging characteristics are unique diagnostic features of a new disease entity so far not described in the literature.

Objectives The aim of this study was to examine motor performance and trainability in youths with cystic fibrosis (CF). Methods Twenty‐two children and adolescents (11 f/11 m), age range 6–17 years (11.3 ± 3.3 years), mean FEV1 91.0 ± 21.7% pred.finished the partially monitored 12‐months exercise program. Patients performed the Deutsche Motorik Test (DMT) to assess flexibility, balance, strength, power and totalmotor performance. An incremental ergometer cycle test was used to assess maximal exercise capacity (Wpeak). All tests were performed before (T1), after 6 months of monitored exercise training (T3) and another 6 months without monitoring (T4). Results Motor Competence in total and test‐items of the DMT (except foreward bend) improved to T3 (p < .05). No further improvement could be observed after the end of the monitoring (T3). However, the values remained stable at the improved level (T4). Girls scored lower in test items depending on strength/power but scored higher in balancing compared to boys (p > .05). Wpeak and FEV1 were not influenced by the training program. From T3 to T4 a slight decrease was observed (p ≤ .05). Conclusions The findings demonstrate benefits of an individualizedmonitored long‐term exercise intervention on motor performance in CF with improvements of test‐tasks to predicted normal. Monitoringseems to be a facilitator in maintaining motivation toward physical activity as no further increase in motor performance was observed after stopping supervision. The results suggest that an individually tailored monitoredregular exercise program should include all aspects of physical fitness with a variety of movement experiences.

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BackgroundIn patients with severe neurological impairment, recurrent respiratory tract infections frequently occur as a result of impaired clearance of airway secretions and microbial airway colonisation. We hypothesised that inhaled antibiotic therapy may improve the morbidity of these patients.MethodsA retrospective data analysis of 20 patients (11 nontracheotomised and nine tracheotomised) with neurological impairment and microbial airway colonisation was carried out at a children's university hospital. Two questionnaires that asked about the number of respiratory tract infections, antibiotic therapies and hospitalisations were distributed to the patients/caregivers: a first questionnaire representing the 12 months prior to the initiation of inhaled antibiotics and a second questionnaire describing the first 12 months under therapy.ResultsDuring the first 12 months of therapy, the frequency of respiratory tract infections among all participants was reduced from a mean of 6.8 episodes (median (interquartile range (IQR)) 6.0 (4.0–10.0) episodes) to a mean of 2.5 episodes (median (IQR) 2.0 (1.0–3.0) episodes; p<0.001). In addition, a significant decrease of systemic antibiotic therapies (mean 7.7, median (IQR) 6.0 (4.0–10.0)versus2.5, 2.5 (0.0–3.75) episodes; p<0.001) and hospitalisations (mean 3.9, median (IQR) 3.5 (1.0–5.0)versus0.9, 0.0 (0.0–1.0) episodes; p<0.001) was noted. This significant therapeutic effect could be demonstrated in a subgroup analysis in both tracheotomised and nontracheotomised subjects. The reduction of respiratory tract infections and systemic antibiotic therapies (and thus the therapeutic success) was significantly greater in the nontracheotomised group compared with the tracheotomised group.ConclusionsThe presented data suggest that inhaled antibiotics might play a role in treating recurrent respiratory tract infections in neuromuscular diseases.