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Growing bilateral occipital calcifications and epilepsy.
1Centro Angeli Custodi, Istituti Ospedalieri, Trento, Italy.
Brain & Development
|January 1, 1990
Summary
Atypical Sturge-Weber disease cases show growing bilateral occipital calcifications. Despite progression, pediatric patients remain well with controlled seizures and stable EEGs, suggesting a potentially better prognosis.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimaging
Background:
- Increasing cases of bilateral occipital calcifications with epilepsy/migraine are observed.
- These cases are often linked to atypical Sturge-Weber disease variants lacking facial nevus flammeus.
Observation:
- Two pediatric patients with bilateral occipital calcifications and neurological symptoms were studied.
- Patients exhibited typical cortico-subcortical bilateral occipital growing calcifications, despite electroclinical variations.
Findings:
- Unlike typical presentations, these patients have remained clinically well.
- Seizures are effectively managed with anti-epileptic drugs (AEDs).
- Electroencephalogram (EEG) results have remained stable, showing no worsening despite calcification growth.
Implications:
- This suggests a potentially more favorable prognosis for certain pediatric cases of Sturge-Weber disease.
- Further research is needed to understand the long-term outcomes and management of these atypical presentations.
- Findings challenge the generally accepted view of progressive worsening in Sturge-Weber syndrome.