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Stationary visual information has a stabilizing effect on posture, whereas moving visual information is destabilizing. We compared the influence of a stationary or moving fixation point to the influence of stationary or moving large-field stimulation, as well as the interaction between a fixation point and a large-field stimulus. We recorded body sway in 20(More)
We report seven unrelated families with mitochondrial tRNA(Ser(UCN)) gene mutations at three different loci. A novel G7497A mutation is found in two families, both of which present with progressive myopathy, ragged-red fibers, lactic acidosis, and deficiency of respiratory chain complexes I and IV. This mutation presumably affects the tertiary(More)
Fabry disease is an X-linked lysosomal storage disorder characterized by multi-organ dysfunction, including hearing loss - mainly sensorineural. The recent introduction of enzyme replacement therapy (ERT) has resulted in improvements in renal and cardiac function, pain and quality of life. One study has also suggested small improvements in high-frequency(More)
Dynamic Programming (DP) is a popular and efficient method for calculating disparity maps from stereo images. It allows for meeting real-time constraints even on low-cost hardware. Therefore, it is frequently used in real-world applications, although more accurate algorithms exist. We present a refined DP stereo processing algorithm which is based on a(More)
Alexander's law (AL) states that the slow-phase velocity of spontaneous nystagmus of peripheral vestibular origin is dependent on horizontal gaze position, with greater velocity when gaze is directed in the fast-phase direction. AL is thought to be a compensatory reaction resulting from adaptive changes in the horizontal ocular motor neural integrator.(More)
Visual input is highly relevant for safely stepping over obstacles. In this study, gaze-behaviour was investigated in elderly, middle-aged and young subjects as they walked on a treadmill repeatedly stepping over obstacles, which approached either on the right or left side. In between obstacle-steps, subjects visually fixated a target N or F located two or(More)
According to Alexander's law (AL), the slow phase velocity of nystagmus of vestibular origin is dependent on horizontal position, with lower velocity when gaze is directed in the slow compared with the fast phase direction. Adaptive changes in the velocity-to-position neural integrator are thought to cause AL. Although these changes have been described for(More)
In placebo-controlled trials, the overall incidence of nonconvulsive status epilepticus was no higher in the tiagabine-treated group than in the placebo-group. Case reports of nonconvulsive status epilepticus under tiagabine suggested a specific role of dose levels, since in these patients symptoms occurred mostly at 40 mg/day or higher. We report a case of(More)
Distal renal tubular acidosis (dRTA) is characterized by the inability to excrete acid in the renal collecting ducts resulting in inappropriately alkaline urine and hyperchloremic (normal anion gap) metabolic acidosis in the context of a normal (or near-normal) glomerular filtration rate. Inborn dRTA can be due to autosomal dominant or recessive gene(More)
Hearing loss is a common symptom in Fabry disease, but neither its natural course nor its aetiology has been defined precisely. The aim of this study was to provide a detailed epidemiological description of hearing impairment in patients in the Fabry Outcome Survey (FOS), which is the largest available database of Fabry patients. Questionnaires were(More)