Seizures, Epilepsy, and NORSE Secondary to Autoimmune Encephalitis: A Practical Guide for Clinicians.
Alberto VogrigGian Luigi GigliAnnacarmen NiloGiada PaulettoMariarosaria ValentePublished in: Biomedicines (2022)
The most recent International League Against Epilepsy (ILAE) classification has included "immune etiology" along with other well-known causes of epilepsy. This was possible thanks to the progress in detection of pathogenic neural antibodies (Abs) in a subset of patients, and resulted in an increased interest in identifying potentially treatable causes of otherwise refractory seizures. Most autoimmune encephalitides (AE) present with seizures, but only a minority of cases evolve to long-term epilepsy. The risk of epilepsy is higher for patients harboring Abs targeting intracellular antigens (T cell-mediated and mostly paraneoplastic, such as Hu, CV2/CRMP5, Ma2, GAD65 Abs), compared with patients with neuronal surface Abs (antibody-mediated and less frequently paraneoplastic, such as NMDAR, GABAbR, LGI1, CASPR2 Abs). To consider these aspects, conceptual definitions for two entities were provided: acute symptomatic seizures secondary to AE, and autoimmune-associated epilepsy, which reflect the different pathophysiology and prognoses. Through this manuscript, we provide an up-to-date review on the current state of knowledge concerning diagnosis and management of patients with Ab-mediated encephalitis and associated epilepsy. Special emphasis is placed on clinical aspects, such as brain magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) specificities, electroencephalographic (EEG) findings, cancer screening and suggestions for a rational therapeutic approach.
Keyphrases
- magnetic resonance imaging
- temporal lobe epilepsy
- end stage renal disease
- ejection fraction
- chronic kidney disease
- multiple sclerosis
- cerebrospinal fluid
- newly diagnosed
- healthcare
- machine learning
- peritoneal dialysis
- computed tomography
- contrast enhanced
- deep learning
- patient reported outcomes
- dendritic cells
- white matter
- intensive care unit
- young adults
- cancer therapy
- reactive oxygen species
- lymph node metastasis
- diffusion weighted imaging
- high density