Abstract
This oculomotor syndrome of childhood, originally termed “benign paroxysmal tonic upgaze of childhood” (Ouvrier and Billson, 1988) is reviewed from the Children’s Hospital of Westmead, Sydney, NSW, Australia.
Highlights
The authors hypothesize that patients with cortical triggers of GRS present regions of cortical hyperexcitability overlapping with the areas activated during sensory stimulation and cognitive or planned motor (praxis) activities
The EEG in pattern-sensitive epilepsies shows focal epileptiform discharges, whereas photosensitive epilepsies are usually accompanied by generalized polyspike-wave and spikewave complexes
The clinical features listed in the original cases were as follows: onset before 1 year of age; conjugate upward deviation of the eyes, with neck flexion; downbeating compensatory saccades; normal horizontal eye movements; fluctuation of symptoms during the daytime and relief in sleep; exacerbation during febrile illness; intermittent or persistent ataxia; otherwise normal neurologic exam; no deterioration and eventual improvement with long-term follow-up; normal EEG, CT, and CSF neurotransmitters
Summary
The authors hypothesize that patients with cortical triggers of GRS present regions of cortical hyperexcitability overlapping with the areas activated during sensory stimulation and cognitive or planned motor (praxis) activities. When these hyperexcitable areas are activated sufficiently, epileptic activity is produced that involves cortico-reticular or cortico-cortical pathways, resulting in a generalized reflex seizure. Genetic or acquired lesions may be responsible for the neuronal hyperexcitability.
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