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Rasmussen encephalitis in childhood
M Topçu1, G Turanli, F M Aynaci
1Department of Pediatric Neurology, Hacettepe University, Faculty of Medicine, Ankara, Turkey. mtopcu@genetic.gen.hun.edu.tr
Insights
Early surgical intervention for Rasmussen encephalitis in children can lead to effective seizure control and aid in diagnosis. This pediatric neurology study highlights the potential benefits of timely surgical treatment for intractable epilepsy.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Epileptology
Background:
- Rasmussen encephalitis is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere, leading to progressive neurological deficits and intractable epilepsy.
- Intractable epilepsy in pediatric patients often necessitates exploring advanced treatment modalities beyond conventional anticonvulsant therapies.
Observation:
- Six pediatric patients diagnosed with Rasmussen encephalitis underwent surgical treatment for drug-resistant epilepsy.
- Diagnostic evaluations included MRI, SPECT, WADA tests, and PCR for viral studies in relevant cases.
- Seizures, often presenting as epilepsia partialis continua, were refractory to multiple anticonvulsant drugs and showed only temporary response to intravenous immunoglobulin therapy in some cases.
Findings:
- Resective surgery with electrocorticography was the chosen surgical approach.
- One patient, who underwent surgery three months after seizure onset, achieved complete seizure control.
- The mean postsurgical follow-up was 32.3 months, indicating the need for long-term monitoring.
Implications:
- Early surgical intervention in Rasmussen encephalitis may offer both diagnostic confirmation and effective seizure management.
- This approach could potentially halt disease progression and improve long-term neurological outcomes in affected children.
- Further research into optimal timing and surgical techniques for Rasmussen encephalitis is warranted.
Abstract:
Six patients admitted to the Department of Pediatric Neurology at Hacettepe University Children's Hospital between 1992 and 1997 with a clinical diagnosis of Rasmussen encephalitis received surgical treatment for their intractable epilepsy. MRI, SPECT and WADA tests were performed in patients with an epileptic focus demonstrated on routine or long-term video EEG monitoring. Viral studies using the PCR methodology were performed in cases with histopathological evidence of Rasmussen encephalitis. The ages of these patients ranged between 7 and 16 years, and the mean age at onset of seizures was 7.1+/-2.2 years. In four patients seizures presented as epilepsia partialis continua and were refractory to anticonvulsive drug therapy. In three cases intravenous immunoglobulin therapy yielded temporary and partial improvement in seizure control. The mean presurgical follow-up duration was 2.04+1.74 years, and early surgical intervention for epilepsy was performed in one case. The surgical approach selected for the treatment of epilepsy was resective surgery with electrocorticography. The mean postoperative follow-up duration was 32.3+17.2 months. Seizures were fully controlled in one patient, in whom surgery was performed 3 months after the seizures first started. Early surgical intervention may provide histopathological evidence for diagnosis as well as effective seizure control.