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Chronic encephalitis (Rasmussen's syndrome) and ipsilateral uveitis
A S Harvey1, F Andermann, I J Hopkins
1Department of Neurology, Royal Children's Hospital, Melbourne, Australia.
Insights
Acute uveitis in children with Rasmussen's syndrome suggests a potential viral cause. The eye inflammation correlated with brain inflammation, hinting at ocular infection and neurotropic spread.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
Background:
- Rasmussen's syndrome is a rare, chronic inflammatory brain disease affecting children.
- It is characterized by progressive neurological decline, often unilateral.
Observation:
- Two pediatric cases of acute uveitis during the early stages of Rasmussen's syndrome were documented.
- Ocular inflammation was ipsilateral to the cerebral inflammation in both children.
- One child also exhibited milder contralateral ocular inflammatory changes.
Findings:
- The concurrent occurrence of uveitis and encephalitis in Rasmussen's syndrome was observed.
- A strong correlation between the laterality of eye and brain inflammation was noted.
Implications:
- This association supports the viral hypothesis for Rasmussen's syndrome.
- It suggests the possibility of primary ocular infection with subsequent neurotropic spread to the brain.
- Ophthalmological monitoring may be valuable in early-stage Rasmussen's syndrome.
Abstract:
Two children are reported with acute uveitis during the early progressive phase of chronic encephalitis (Rasmussen's syndrome). In both children, the side of the uveitis was ipsilateral to the side of cerebral inflammation, although in 1 child there were milder inflammatory changes in the contralateral eye. This association adds weight to the viral hypothesis of chronic encephalitis and raises the possibility of primary ocular infection and neurotropic spread to the brain.