Progressive myoclonic epilepsy

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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• 🟢 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.

• 🧠 Intellectual Rigour
  • Palliative intent is explicitly acknowledged.
  • Cites prior PME cases (mostly pediatric, non-DRPLA).
  • Lacks deeper comparison to alternatives (e.g. VNS, thalamic DBS).

★★★☆☆ 6/10 Interesting and rare case with clear illustrative value. However, it lacks broader context, comparative analysis, and rigorous outcome measurement.

Clinical context: In adult patients with Dentatorubral-pallidoluysian atrophy (DRPLA)-related progressive myoclonic epilepsy (PME) that is disabling and refractory to standard therapies, → a total corpus callosotomy may offer clinically meaningful palliation.

Key benefits observed:

• ✅ Rapid reduction in seizure frequency
• ✅ Resolution of desaturation episodes
• ✅ Improved quality of life (QOL) at 1-year follow-up

Safety:

• 🚫 No major additional neurological or systemic harm reported postoperatively

Bottom line: > This case supports considering total callosotomy as a palliative surgical option in select adult PME cases where conventional therapies have failed.

Total corpus callosotomy may be a reasonable and effective palliative option in adult-onset DRPLA-related PME, particularly when conventional treatments have failed.

• Case Report
• Epilepsy Surgery
• DRPLA
• Corpus Callosotomy

• PME
• DRPLA
• callosotomy
• adult epilepsy
• palliative neurosurgery
• case lessons