Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease.
In late-onset Tay-Sachs disease (LOTS), saccades are interrupted by one or more transient decelerations. Some saccades reaccelerate and continue on before eye velocity reaches zero, even in darkness. Intervals between successive decelerations are not regularly spaced. Peak decelerations of horizontal and vertical components of oblique saccades in LOTS is more synchronous than those in control subjects. We hypothesize that these decelerations are caused by dysregulation of the fastigial nuclei (FN) of the cerebellum, which fire brain stem inhibitory burst neurons (IBNs).
Optican
Lance M
LM
National Eye Institute, Bethesda, MD, USA. LanceOptican@nih.gov
Rucker
Janet C
JC
Keller
Edward L
EL
Leigh
R John
RJ
D002529:Cerebellar Nuclei / Q000473:pathology / Q000502:physiology / Q000503:physiopathology*; D006801:Humans; D010775:Photic Stimulation; D011689:Purkinje Cells / Q000502:physiology; D012438:Saccades / Q000502:physiology*; D013661:Tay-Sachs Disease / Q000473:pathology / Q000503:physiopathology*; D014795:Visual Pathways / Q000502:physiology