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Related Experiment Videos

Growing bilateral occipital calcifications and epilepsy.

P DeMarco1, G Lorenzin

  • 1Centro Angeli Custodi, Istituti Ospedalieri, Trento, Italy.

Brain & Development
|January 1, 1990
PubMed
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.

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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.

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