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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.
Annals of Neurology
|December 1, 1992
Summary
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.