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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.
Australian Paediatric Journal
|April 1, 1989
Summary
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.