Article Figures & Data
Figures
- Figure 1
- Some of the genetic causes reported to be associated with EPC.
Tables
Category Examples Structural abnormalities Migrational developmental disorders: FCD, heterotopia, TSC Brain tumors Gliomas and meningiomas Vascular Stroke, CSVT, vascular malformations, hemorrhage Trauma Traumatic brain injury Infections Meningitis, neurocysticercosis, HIV. HSV, COVID19, tick-borne encephalitis. Inflammation Rasmussen’s encephalitis, autoimmune encephalitis (NMDA, LGI1), SSPE, MS, anti-MOG antibodies, anti ANNA-1/anti-Hu antibodies Metabolic NKH, urea cycle disorders, mitochondrial disorders (MELAS, MERRF, Leigh disease), Menkes disease Genetic KCNA2, SCN1A, ADCK3 genes Electrolytes Diabetic ketoacidosis, hyponatremia Investigation Description and rationale Basic blood tests (glucose, electrolytes, liver function tests, renal function tests, ESR, CRP) To rule out electrolyte imbalances, liver or kidney dysfunction, or signs of infection. Electroencephalogram (EEG) and video EEG To characterize and confirm the origin of seizures and the concordance with imaging changes. Also, to gauge management outcome. Magnetic resonance imaging (MRI) brain To evaluate and follow brain structural changes. Magnetic resonance angiography (MRA) and magnetic resonance venography (MRV) In cases with suspected stroke, cerebral-venous sinuses thrombosis or suspected vasculitis. Magnetic resonance spectroscopy (MRS) In cases presenting with a clinical picture of mitochondrial disease Cerebrospinal fluid (CSF) (autoimmune markers) Autoimmune encephalitis panel of antibodies (e.g. NMDA, LGI1, GAD and GABA antibodies) Cerebrospinal fluid (CSF) (cultures) Routine CSF culture including HSV and consider measles antibody testing if clinically indicated Aminoacids, organic acids, lactate To screen for metabolic disorders Genetic (WES/WGS) Whole exome sequencing and whole genome sequencing in idiopathic EPC PET (positron emission tomography) brain or body To identify the abnormal functional focus if EEG and MRI data were non-concordant. Whole body Pet scan can be considered in cases of possible brain metastatic disease Single-photon emission computerized tomography (SPECT) To identify the abnormal functional focus if EEG and MRI data were non-concordant. Also, to confirm concordance with other investigations. Brain biopsy In rare cases, a brain biopsy may be necessary Treatment modality Description and rationale Antiseizure medications Most of the current ASMs can be tried in patients with EPC. Multiple mechanisms of action (rational polytherapy) can be effective. Immunotherapy Conventional immunotherapy like steroids, IVIG and plasma exchange or non-conventional options like rituximab and others like mycophenolate mofetil and azathioprine, have reported some efficacy in EPC due to autoimmune etiologies (like Rasmussen`s and anti-NMDA encephalitis-related EPC) Surgery Focal resection (hemispherectomy) is recommended in progressive Rasmussen`s encephalitis
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