case lessons

Total corpus callosotomy for an adult patient with progressive myoclonic epilepsy associated with dentatorubral-pallidoluysian atrophy: illustrative case

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In a single-patient illustrative case, Mine et al., from Kyushu University, Fukuoka, Japan, published in the Journal of Neurosurgery Case Lessons, report the first adult case of Dentatorubral-pallidoluysian atrophy (DRPLA)-associated progressive myoclonic epilepsy (PME) treated with a total corpus callosotomy (CC) for refractory seizures.

→ Outcome: Total CC led to a marked reduction in seizure frequency. Tonic seizures and FBTCSs with desaturation resolved by 1 year, with notable improvement in quality of life (QOL).

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🔎 Critical Appraisal

  • 🟢 Strengths
    • Novelty: First reported adult DRPLA-PME corpus callosotomy.
    • Clearly disabling epilepsy: Myoclonus, tonic seizures, desaturation.
    • Clear outcome: Sustained seizure reduction at 1-year.
  • 🟡 Limitations
    • Single case → low external validity.
    • No comparator: No data vs. anterior CC or other therapies.
    • Cognitive effects unquantified.
    • DRPLA is a diffuse neurodegenerative disease; CC does not target focus directly.

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