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Epilepsy surgery in Sturge-Weber syndrome
A M Bye1, J M Matheson, R A Mackenzie
1Department of Paediatric Nuerology, Prince of Wales Children's Hospital, Randwick, Australia.
Insights
Sturge-Weber syndrome patients with intractable seizures may benefit from surgical removal of the epileptogenic focus. This case demonstrates successful occipital lobe resection in a patient with bilateral central nervous system pathology.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Neurology
Background:
- Sturge-Weber syndrome is a rare congenital disorder characterized by a port-wine stain, neurological abnormalities, and often epilepsy.
- Intractable epilepsy in Sturge-Weber syndrome poses significant management challenges, particularly with bilateral central nervous system involvement.
Observation:
- A 13-year-old patient with Sturge-Weber syndrome presented with intractable complex partial seizures and visual symptoms.
- Electroencephalography revealed bilateral slow activity with right temporal epileptogenic focus. Imaging showed progressive bilateral occipital lesions with calcification, more pronounced on the right.
Findings:
- Surgical resection of the right occipital lobe was performed.
- One year post-surgery, the patient experienced a significant reduction in seizure frequency, with only seven brief seizures reported.
Implications:
- This case highlights the potential efficacy of surgical intervention for intractable epilepsy in selected patients with Sturge-Weber syndrome and bilateral central nervous system lesions.
- Early identification and surgical management of the epileptogenic focus can improve seizure control and quality of life in these complex cases.
Abstract:
A patient with Sturge-Weber syndrome developed seizures at the age of 4 years. At 13 years of age, she had intractable complex partial seizures with marked visual symptomatology. Interictal encephalograms showed bilateral slow activity, more marked over the right hemisphere with epileptogenic activity maximal in the right temporal region. Serial computerized axial tomography scans demonstrated evolution of bilateral occipital lesions with calcification and adjacent low density areas that were more marked on the right. Magnetic resonance imaging, angiography and neuropsychological evaluations were performed. An extensive resection of the right occipital lobe was achieved. One year after surgery, the patient has had seven brief seizures. Delineation of an epileptogenic focus and surgical removal of the lesion in patients with intractable seizures can now be considered in selected patients with bilateral central nervous system pathology.