Factors underlying the development of chronic temporal lobe epilepsy in autoimmune encephalitis

• Published in: Journal of the neurological sciences Vol. 396; pp. 102 - 107
• Main Authors: Casciato, Sara, Morano, Alessandra, Fattouch, Jinane, Fanella, Martina, Avorio, Federica, Albini, Mariarita, Basili, Luca Manfredi, Cerulli Irelli, Emanuele, Viganò, Alessandro, De Risi, Marco, Grammaldo, Liliana G., D'Aniello, Alfredo, Mascia, Addolorata, Manfredi, Mario, Quarato, Pierpaolo, Giallonardo, Anna Teresa, Di Gennaro, Giancarlo, Di Bonaventura, Carlo
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15.01.2019
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Autoimmune encephalopathies; Chronic focal epilepsy; Cognition Disorders - etiology; Cohort Studies; Disease Progression; Electroencephalography; Encephalitis - complications; Encephalitis - psychology; Encephalitis - therapy; Epilepsy, Temporal Lobe - diagnostic imaging; Epilepsy, Temporal Lobe - etiology; Female; Hashimoto Disease - complications; Hashimoto Disease - psychology; Hashimoto Disease - therapy; Humans; Immunotherapy - methods; Limbic encephalitis; Magnetic Resonance Imaging; Male; Middle Aged; Neurology; Seizures; Young Adult; Autoimmune encephalopathies; Chronic focal epilepsy; Limbic encephalitis; Seizures
• ISSN: 0022-510X; 1878-5883; 1878-5883
• DOI: 10.1016/j.jns.2018.10.026

Limbic encephalitis (LE) is an autoimmune condition characterized by amnestic syndrome, psychiatric features and seizures. Early diagnosis and prompt treatment are crucial to avoid long-term sequelae, including psycho-cognitive deficits and persisting seizures. The aim of our study was to analyze the characteristics of 33 LE patients in order to identify possible prognostic factors associated with the development of chronic epilepsy. This is a retrospective cohort study including adult patients diagnosed with LE in the period 2010–2017 and followed up for ≥12 months. Demographics, seizure semiology, EEG pattern, MRI features, CSF/serum findings were reviewed. All 33 LE patients (19 M/14F, mean age 61.2 years) presented seizures. Thirty subjects had memory deficits; 22 presented behavioural/mood disorders. Serum and/or CSF auto-antibodies were detected in 12 patients. In 31 subjects brain MRI at onset showed typical alterations involving temporal lobes. All patients received immunotherapy. At follow-up, 13/33 had developed chronic epilepsy; predisposing factors included delay in diagnosis (p = .009), low seizure frequency at onset (p = .02), absence of amnestic syndrome (p = .02) and absence/rarity of inter-ictal epileptic discharges on EEG (p = .06). LE with paucisymptomatic electro-clinical presentation seemed to be associated to chronic epilepsy more than LE presenting with definite and severe “limbic syndrome”. •LE is a potentially treatable, often overlooked, immune-mediated condition•In acute phase its clinical spectrum includes seizures and psycho-cognitive disorders•Chronic epilepsy with predominant temporal seizures can represent a long term sequela•Some specific factors are associated with the development of chronic epilepsy•These factors include diagnostic delay and mild/uncomplete phenotype at onset