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[Etiology and clinical features of epilepsia partialis continua: an analysis of six cases]
Mu Zhang1, Zhen-Li Tang, Li-Wen Wu
1Department of Emergency, Xiangya Hospital, Central South University, Changsha 410008, China. 985882728@qq.com.
Insights
Epilepsia partialis continua (EPC) in children is often caused by immune inflammation or metabolic disorders. Treatment and outcomes for pediatric EPC depend heavily on the underlying cause, with varied prognoses.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroscience
Background:
- Epilepsia partialis continua (EPC) is a rare and distinct form of status epilepticus characterized by continuous focal seizures.
- Understanding the diverse etiologies and clinical presentations of EPC in children is crucial for effective management.
Purpose of the Study:
- To investigate the causes and clinical characteristics of epilepsia partialis continua (EPC) in pediatric patients.
- To compare clinical and laboratory features, as well as prognosis, based on different etiologies of EPC.
Main Methods:
- Retrospective analysis of six pediatric cases diagnosed with EPC.
- Review of clinical features, diagnostic findings (MRI, PET/CT, EEG), treatment strategies, and patient outcomes.
- Comparison of outcomes across various identified etiologies.
Main Results:
- Etiologies included Rasmussen encephalitis, focal cortical dysplasia, POLG gene mutation-related Alpers syndrome, Angelman syndrome, and tuberculous meningitis.
- Clinical manifestations included focal seizures, automatism, tonic seizures, and autonomic symptoms; EEG and MRI showed characteristic abnormalities.
- Treatment response varied significantly, with surgical intervention successful in some cases and poor prognosis in others, particularly Alpers syndrome.
Conclusions:
- Immune inflammation and metabolic disorders are primary drivers of EPC in children.
- The specific etiology of pediatric EPC dictates the appropriate treatment approach, therapeutic success, and overall prognosis.
- EPC is a unique epileptic condition where etiology is the key determinant of patient outcomes.
Objective:
To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children.
Methods:
A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies.
Results:
There were five girls and one boy, with an onset age ranging from one year and seven months to nine years. Two were diagnosed with Rasmussen encephalitis, one was diagnosed with focal cortical dysplasia, one was diagnosed with Alpers syndrome caused by POLG gene mutation, one was diagnosed with Angelman syndrome, and one was diagnosed with tuberculous meningitis. The latter two children had the predisposing factors for acute encephalopathy induced by status epilepticus and craniocerebral operation during the onset of EPC, while the other four children had natural progression of EPC. All the children had focal seizures except EPC, and symptoms included automatism, bilateral asymmetric tonic seizure, deflection, complex motor, and autonomic symptoms, with disturbance of consciousness in some children. EPC often lasted for several days or even several months. All children had abnormalities on head MRI, including local abnormal signal, cortex swelling, diffusive brain atrophy or brain atrophy at one side, local cortex thickening, and cortical necrosis. Head PET/CT scan was performed for three children and found local hypermetabolism or co-existence of hypermetabolism and hypometabolism. All the children had abnormalities on electroencephalography (EEG), with cerebral, hemispheric, or diffusive distribution of abnormal electrical activities, and during the onset of EPC, some EEG changes were recognizable and some were difficult to identify. All the children with EPC were not sensitive to antiepileptic drugs. EPC was relatively self-limiting in the child with Angelman syndrome. The child with focal cortical dysplasia underwent resection of epileptic foci and had good postoperative control, without neurological dysfunction. The child with Rasmussen encephalitis underwent functional hemispherectomy and had no attack after surgery, with neurological dysfunction. The child with Alpers syndrome had the worst prognosis.
Conclusions:
EPC is a special type of epileptic seizures. Immune inflammation and metabolic etiologies are the main causes of EPC in children, and the selection of treatment regimens, treatment outcome, and prognosis depend on etiology.
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