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1.  Ocular motor anatomy in a case of interrupted saccades 
Progress in brain research  2008;171:563-566.
Saccades normally place the eye on target with one smooth movement. In late-onset Tay—Sachs (LOTS), intrasaccadic transient decelerations occur that may result from (1) premature omnipause neuron (OPN) re-activation due to malfunction of the latch circuit that inhibits OPNs for the duration of the saccade or (2) premature inhibitory burst neuron (IBN) activation due to fastigial nucleus (FN) dysregulation by the dorsal cerebellar vermis. Neuroanatomic analysis of a LOTS brain was performed. Purkinje cells were absent and gliosis of the granular cell layer was present in the dorsal cerebellar vermis. Deep cerebellar nuclei contained large inclusions. IBNs were present with small inclusions. The sample did not contain the complete OPN region; however, neurons in the OPN region contained massive inclusions. Pathologic findings suggest that premature OPN re-activation and/or inappropriate firing of IBNs may be responsible for interrupted saccades in LOTS. Cerebellar clinical dysfunction, lack of saccadic slowing, and significant loss of cerebellar cells suggest that the second cause is more likely.
doi:10.1016/S0079-6123(08)00680-8
PMCID: PMC2752380  PMID: 18718354
fastigial nucleus; omnipause neurons; burst neurons; latch circuit; brainstem
2.  Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease 
Progress in brain research  2008;171:567-570.
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).
doi:10.1016/S0079-6123(08)00681-X
PMCID: PMC2750844  PMID: 18718355
fastigial nucleus; omnipause neurons; burst neurons; latch circuit

Results 1-2 (2)