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Eye movements in children with opsoclonus-polymyoclonus
F S Shawkat1, C M Harris, J Wilson
1Department of Ophthalmology, Hospital for Sick Children, London, U.K.
Neuropediatrics
|August 1, 1993
Summary
The study suggests the cerebellum, specifically the fastigial nuclei, may cause opsoclonus-polymyoclonus ("Dancing Eye Syndrome") in children. Eye movement analysis revealed consistent saccadic overshoot dysmetria, indicating a potential cerebellar origin.
Area of Science:
- Neuroscience
- Ophthalmology
- Pediatrics
Background:
- Opsoclonus-polymyoclonus, or "Dancing Eye Syndrome," is a rare neurological disorder.
- The precise anatomical origin of this syndrome remains uncertain, with both brainstem and cerebellar involvement hypothesized.
Purpose of the Study:
- To investigate the anatomical localization of the underlying abnormality in children with opsoclonus-polymyoclonus.
- To characterize the specific eye movement abnormalities associated with the syndrome.
Main Methods:
- Electrooculography (EOG) was used to record and analyze eye movements in five children diagnosed with opsoclonus-polymyoclonus.
- Assessment included saccades, smooth pursuit, optokinetic nystagmus, and vestibulo-ocular reflex.
Main Results:
- All five children exhibited saccadic overshoot dysmetria, a consistent finding.
- Smooth pursuit and optokinetic nystagmus were normal, despite superimposed opsoclonus.
- The vestibulo-ocular reflex was normal in most cases, with one showing a prolonged time-constant, similar to experimental monkey lesions.
Conclusions:
- Saccadic overshoot dysmetria strongly suggests a cerebellar origin for the oculomotor deficits in opsoclonus-polymyoclonus.
- Preserved smooth pursuit and absence of certain nystagmus types imply sparing of the flocculus and paraflocculus.
- The cerebellar fastigial nuclei are proposed as the potential origin of these oculomotor deficits.