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Despite the many models of saccadic eye movements, little attention has been paid to the shape of saccade trajectories. Some investigators have argued that saccades are driven by a rectangular "bang-bang" neural control signal, whereas others have emphasized the similarity to fast arm movement trajectories, such as the "minimum jerk" profile. However,(More)
PURPOSE To determine a gene locus for a family with a dominantly inherited vestibulocerebellar disorder characterised by early onset, but not congenital nystagmus. DESIGN Observational and experimental study. METHODS We carried out a phenotypic study of a unique four generation family with nystagmus. We performed genetic linkage studies including a(More)
BACKGROUND/AIMS Abnormalities in the saccadic main sequence are an important finding and may indicate pathology of the ocular motor periphery or central neurological disorders. In young or uncooperative patients it can be difficult eliciting a sufficient number of saccades to measure the main sequence. It is often assumed that the quick phases of(More)
BACKGROUND The occurrence of monocular naso-to-temporal optokinetic nystagmus (OKN) asymmetry, as a reflection of the immature oculomotor system in infants, and its persistence with early onset monocular visual deprivation, is well known. This asymmetry has been linked with poor binocular function and attributed to disruption of the development of binocular(More)
Electronic article surveillance (EAS) is used in many applications throughout the world to prevent theft. EAS systems produce electromagnetic (EM) energy around exits to create an EM interrogation zone through which protected items must pass before leaving the establishment. Specially designed EAS tags are attached to these items and must either be(More)
Abnormal eye movements in the infant or voting child can be congenital or acquired. They may be a result of abnormal early visual development or a sign of underlying neurologic or neuromuscular disease. It is important to be able to detect these abnormalities and to distinguish them from normal but immature eye movements. The spectrum of disease in children(More)
Epileptic nystagmus (EN) is a rare form of nystagmus that occurs only during epileptic seizures. We report an infantile case in which EN was first noted at 10 days of age. Electronystagmography showed a right-beating nystagmus with predominantly linear slow phases that traversed the midline. Neuro-imaging revealed dysplasia of the left middle temporal gyrus(More)
Exogenous enzyme replacement therapy achieves satisfactory biomedical correction in Gaucher type 1 disease and may halt or reverse neurological progression in type 3, while it does not appear to influence the outcome in type 2. In view of the therapeutic possibilities, early detection and monitoring of type 3 Gaucher disease, as well as evaluation of the(More)
Gaucher disease (GD) is a lysosomal disorder involving the accumulation of glucocerebroside in the liver, spleen, bones and brain. Some patients exhibit only systemic disease (type I), but others have additional neurological signs which may lead to rapid neurodegeneration in infancy (type II) or take a more intermediate course (type III). Types II and III(More)