Parasagittal hemispherotomy in hemispheric polymicrogyria with electrical status epilepticus during slow sleep:
Martine Fohlen1, Georg Dorfmüller1, Sarah Ferrand-Sorbets1
1Pediatric Neurosurgery Unit, Rothschild Foundation Hospital, 29 rue Manin, 75019, Paris, France.
Purpose:
Polymicrogyria (PMG), although the most common brain malformation, represents a low percentage among patients operated on for epilepsy. In cases of hemispheric PMG, electrical status epilepticus during slow sleep (ESESS) may occur leading to an aggravation of the neurological condition and a risk of drug resistance. In such cases, surgical treatment can be offered.
Methods:
From a population of 230 children who underwent hemispherotomy for epilepsy, we retrospectively reviewed the patients with unilateral PMG and drug-resistant ESESS focusing on clinical charts, electrophysiological data and post-surgical outcome.
Results:
Eighteen patients were operated on at a mean age of 7.2 years. The average age was 2 years at seizure onset and 4.4 years at diagnosis of ESESS. All the patients preoperatively had some degree of developmental delay associated with a hemiparesis. During ESESS all of them evidenced a cognitive decline and eight experienced a worsening of the hemiparesis; ESESS was resistant to at least three antiepileptic drugs. The outcome of epilepsy, with a mean follow-up of 12.8 years showed that ESESS disappeared in all patients while 16 of 18 became seizure-free. An improvement of behavior and cognitive condition was observed in all.
Conclusion:
Hemispherotomy can be helpful in patients with drug-resistant ESESS and hemispheric PMG while keeping in mind that more often an accurate medical treatment can be sufficient. The main benefit of surgery is to definitively stop the seizures and to withdraw the medical treatment while keeping in mind the risk of motor aggravation.
Insights
Hemispherotomy surgery effectively treated drug-resistant electrical status epilepticus during slow sleep (ESESS) in children with hemispheric polymicrogyria (PMG), leading to seizure freedom and cognitive improvement.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Epileptology
Background:
- Polymicrogyria (PMG) is the most common brain malformation, but rarely requires epilepsy surgery.
- Hemispheric PMG can lead to electrical status epilepticus during slow sleep (ESESS), causing neurological decline and drug resistance.
Purpose of the Study:
- To evaluate the efficacy of hemispherotomy in children with drug-resistant ESESS and unilateral PMG.
- To assess post-surgical outcomes, including seizure control, cognitive function, and motor status.
Main Methods:
- Retrospective review of 18 children who underwent hemispherotomy for epilepsy due to unilateral PMG and drug-resistant ESESS.
- Analysis of clinical data, electrophysiological findings, and long-term post-surgical outcomes.
Main Results:
- All 18 patients experienced resolution of ESESS and 16 became seizure-free after a mean follow-up of 12.8 years.
- Significant improvements in behavior and cognitive function were observed in all patients.
- Preoperative developmental delay and hemiparesis were noted, with a risk of motor aggravation post-surgery.
Conclusions:
- Hemispherotomy is a viable surgical option for drug-resistant ESESS in hemispheric PMG, offering seizure cessation and cognitive benefits.
- While medical treatment may suffice in some cases, surgery provides definitive seizure control.
- Potential risks, including motor function aggravation, should be considered alongside the benefits.
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