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Postcallosotomy seizure outcome in hemiconvulsion-hemiatrophy-epilepsy syndrome
S Y Kwan1, T T Wong, K P Chang
1Section of Epilepsy, Neurological Institute, Taipei Veterans General Hospital, Taiwan, ROC.
Insights
Callosotomy significantly reduced seizures in patients with hemiconvulsion-hemiplegia-epilepsy syndrome, even with brain atrophy. This epilepsy surgery offers a viable alternative to hemispherectomy, improving patient outcomes.
Area of Science:
- Neurology
- Neurosurgery
- Epileptology
Background:
- Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) is a rare, severe epilepsy characterized by unilateral convulsions, hemiplegia, and intractable seizures.
- Callosotomy, a surgical procedure severing the corpus callosum, is considered for refractory epilepsy.
- This study evaluates long-term outcomes of callosotomy in HHE patients with significant brain hemisphere atrophy.
Observation:
- Three patients with right hemisphere atrophy, left hemiparesis, and intractable epilepsy underwent callosotomy.
- Pre-operative Wada tests in two patients indicated preserved motor function in the atrophic hemisphere, supporting callosotomy over hemispherectomy.
- Patients presented with diverse seizure types including generalized tonic, atypical absence, simple partial (motor and sensory), and complex partial seizures, some somatosensory-induced.
Findings:
- All three patients experienced a significant reduction in overall seizure frequency (>50%) post-callosotomy.
- Specific seizure types showed variable responses: generalized tonic seizures decreased by >90% in one patient, while simple partial seizures remained unchanged in others.
- New-onset simple partial motor seizures and brief generalized myoclonic jerks were observed post-surgery in one patient.
Implications:
- Callosotomy is an effective surgical option for managing intractable epilepsy in HHE syndrome, even in the presence of significant hemispheric atrophy.
- The procedure can lead to substantial seizure reduction, improving quality of life for affected individuals.
- Careful pre-operative assessment, including Wada testing, is crucial for selecting appropriate surgical candidates and predicting outcomes.
Abstract:
Recently, three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who underwent callosotomy were monitored for more than four years. All patients had atrophy of the right brain hemisphere with left hemiparesis and seizures. Two cases were probably the result of traumatic intracranial hemorrhage and one was due to an unknown cause. Wada tests were done in cases 1 and 2, which showed spared motor function of the atrophic hemisphere, supporting the choice of callosotomy instead of hemispherectomy. Patient 1 had atypical absence seizures and asymmetric generalized tonic seizures before surgery, the latter of which decreased by about 60% after callosotomy. Patient 2 had simple partial seizures of the motor type and complex partial seizures, the latter of which were also induced by touch (somatosensory-induced reflex epilepsy). This patient's complex partial seizures disappeared completely, but the simple partial seizures remained unchanged. Patient 3 had generalized tonic seizures, simple partial seizures of the sensory type and complex partial seizures. After surgery, the frequency of the generalized tonic seizures decreased more than 90%. The simple partial seizures of the sensory type remained unchanged. There were two new types of seizures after surgery, simple partial seizures of the motor type and brief generalized myoclonic jerks. All patients had significant reductions in numbers of seizures of more than 50%.