Super-Refractory Epilepsia Partialis Continua Unmasking Rasmussen Syndrome in a Filipino Adolescent: A Case Report
Sam Jeffrey B. Tiongco *
Saint Louis University, School of Medicine, Baguio City, Benguet, Philippines.
*Author to whom correspondence should be addressed.
Abstract
Aims: To report an adolescent with drug-resistant focal epilepsy who developed super-refractory epilepsia partialis continua (EPC), in the setting of suspected Rasmussen syndrome, highlighting diagnostic and management challenges in a resource-limited setting.
Case Presentation: A 14-year-old Filipino male with six years of focal motor epilepsy on phenobarbital, levetiracetam, and perampanel presented after focal seizures escalated over seven hours into generalised tonic-clonic clusters every 30 minutes. Examination showed left hemiparesis, hemisensory loss, facial asymmetry, and an NIHSS of 16. EEG showed asymmetric slowing with right temporoparietal sharp waves, and a brain MRI three years earlier had already shown focal right fronto-temporal encephalomalacia. Seizures progressed to status epilepticus and then EPC refractory to add-on topiramate, lacosamide, and continuous midazolam, propofol, and ketamine infusions, requiring 14 days of ventilation and treatment with pulse methylprednisolone. These laterality-concordant findings culminating in super-refractory EPC led to a presumptive diagnosis of Rasmussen syndrome. He was weaned off sedation, transitioned to oral antiseizure therapy, and discharged after 20 days with residual hemiparesis; he was referred to a comprehensive epilepsy centre for video-EEG, epilepsy-protocol imaging, and hemispherotomy evaluation.
Discussion: Rasmussen syndrome, a rare unihemispheric encephalitis, should be suspected when chronic drug-resistant focal epilepsy with concordant lateralising imaging and EEG abnormalities culminates in EPC; limited access to video-EEG, epilepsy-protocol MRI, and immunotherapy delays referral for hemispherotomy, the intervention most likely to achieve seizure freedom.
Conclusion: Progressive unihemispheric seizures, hemiparesis, and imaging evidence of atrophy in a child should prompt early consideration of Rasmussen syndrome and timely surgical referral, since medical therapy rarely controls the disease.
Keywords: Rasmussen syndrome, Rasmussen encephalitis, epilepsia partialis continua, drug-resistant epilepsy, hemispherotomy, status epilepticus