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Periodic alternating gaze deviation in infancy.
R H Legge1, H S Weiss, T R Hedges
1Department of Ophthalmology, New England Medical Center, Tufts University, Boston, MA.
Neurology
|September 1, 1992
Summary
Periodic alternating gaze, a rare condition, was observed in two infants with early onset. This eye movement disorder is linked to congenital defects in the hindbrain, specifically the inferior cerebellar vermis.
Area of Science:
- Neurology
- Pediatrics
- Neuroscience
Background:
- Periodic alternating gaze (PAG) is a rare nystagmus variant.
- Its etiology and associated pathologies are not well-understood.
- Early-onset forms are exceptionally uncommon.
Observation:
- Two cases of congenital periodic alternating gaze are presented.
- Onset was noted at birth and in infancy.
- Magnetic Resonance Imaging (MRI) revealed significant congenital defects in the posterior cranial fossa in both infants.
Findings:
- A key shared finding was the absence of normal structures in the inferior cerebellar vermis region.
- These hindbrain abnormalities were consistently observed.
- The findings suggest a strong correlation between cerebellar vermis malformations and early-onset PAG.
Implications:
- Periodic alternating gaze may serve as a clinical indicator of underlying hindbrain pathology.
- Further research into cerebellar vermis development and its role in ocular motor control is warranted.
- Understanding these associations can aid in earlier diagnosis and management of related neurological conditions.