Epilepsy and Autoimmunity: Basic Physiopathological Mechanisms

Summary Autoimmunity and inflammation have been linked with different neurological syndromes apart from epilepsy, but they are also increasingly observed in cases where epilepsy occurs alone as the dominant clinical feature. There is some evidence that inflammation and autoimmunity contribute to the etiology of epilepsy. On the other hand the basic physiopathological mechanisms are still unclear. Reviewing these basic mechanisms is important to understand the role of inflammation and autoimmunity on epilepsy to contribute to clinical diagnosis and future treatment options of epilepsy. The aim of this article is to elucidate these mechanisms on epileptogenesis

Epilepsy and Autoimmunity: Basic Physiopathological Mechanisms

Autoimmunity and inflammation have been linked with different neurological syndromes apart from epilepsy, but they are also increasingly observed in cases where epilepsy occurs alone as the dominant clinical feature. There is some evidence that inflammation and autoimmunity contribute to the etiology of epilepsy. On the other hand the basic physiopathological mechanisms are still unclear. Reviewing these basic mechanisms is important to understand the role of inflammation and autoimmunity on epilepsy to contribute to clinical diagnosis and future treatment options of epilepsy. The aim of this article is to elucidate these mechanisms on epileptogenesis

Do the neurologists recognize autoimmune epilepsy well enough? What is the effect of the pandemic on this matter?

Introduction The concept of "autoimmune epilepsy" (AE) has been emphasized more frequently through the recent increase in recognition of various autoantibodies specific to neuronal proteins

Idiopathic intracranial hypertension: Do we diagnose and manage it appropriately in the light of current data?

Background and objective: Idiopathic intracranial hypertension (IIH) is an increase of intracranial pressure without a known cause, which usually presented with headache. This study aimed to evaluate the changing diagnosis and management approaches of neurologists for IIH in light of recent data. Methods: An online questionnaire about IIH was developed covering 28 questions, and five sections: demographic data, diagnosis, examination, treatment, and follow-up. We compared the approach of neurologists with 1-9 years of experience (group-A) with that of neurologists with more than 10 years' experience (group-B). Results: A total of 517 neurologists (group A: n = 252, group B: n = 265) participated in the study. Responder rate of questionarre is 18.3%. The approach to IIH in diagnosis, examination, treatment, and follow-up processes was similar in both groups. The younger group (group A) recognized all neuro-radiologic findings, especially flattening of the posterior aspect of the globe (p = 0.001) and tortuosity of the optic nerve (p < 0.001) at higher rates compared with group B. The most commonly used medical treatment was acetazolamide (99%); corticosteroids were used more frequently by group B (p < 0.001). Optic nerve sheath fenestration (88.3%) was the first-line and ventriculo-peritoneal shunt (70.5%) was the second preferred surgical approach. It was observed that serial lumbar puncture applications (57.0%) were preferred more frequently than venous sinus stenting (19.0%) and bariatric surgery (10.0%). Conclusions: The changing information in the last decade about IIH was more closely followed by younger neurologists despite their lesser experience, but classic methods were preferred in surgical approaches in both groups. Our findings indicated that post-graduate education and guidelines should be disseminated for IIH

Long-term follow-up of a large cohort with focal epilepsy of unknown cause: deciphering their clinical and prognostic characteristics

Background and purpose Focal epilepsy of unknown cause (FEUC) is an under-investigated topic despite its remarkable frequency. We aimed to report the long-term follow-up findings along with the drug-response, 5 year remission rates and diagnostic changes to give an insight about the heterogeneous characteristics of FEUC. Methods Demographic, clinical, neurophysiological and imaging data of 196 patients diagnosed as FEUC according to ILAE criteria, with a minimum 5-year follow-up were evaluated in a tertiary epilepsy center. The drug resistance, 5 years of remission and relapse rates were investigated and the subgroups were compared statistically. Results The rate of drug resistance was 21.8% and status epilepticus (p < 0.001), abnormal neurological examination (p = 0.020), seizure onset before 10 years (p = 0.004) and a high initial seizure frequency (p = 0.006) were significant predictors of drug resistance. The rates of terminal 5-year remission, 5-year remission ever and relapse were 39.9%, 44.26% and 24.04%, respectively. There were 13 patients (6.6%) with a changed final diagnosis. Drug resistance (p = 0.004), pathological EEG (p = 0.034) and status epilepticus (p = 0.021) were negative variables for achieving remission. The lobar localization of seizures was not a predictor of remission or relapse. Onset after 10 years of age had a higher probability of achieving a 5-year remission according to Kaplan-Meier curves (p < 0.001). Conclusions Focal epilepsy of unknown cause has a benign electroclinical subgroup with favorable long-term course, lower drug resistance and higher 5 years of terminal remission and remission ever rates, when appropriately treated. Our findings might be valuable in terms of counseling and management of patients with FEUC at the first referral to epilepsy clinics

Investigation of Generalized EEG Paroxysms Accompanying Focal Epilepsies

Interictal focal EEG features were frequently observed in generalized, epilepsies, but there is limited information about interictal, epileptiform/nonepileptiform generalized paroxysms in focal epilepsies. We aimed to report the frequency and associated factors of generalized EEG discharges in focal epilepsy with unknown cause (FEUC) and mesial, temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). A total of 200 patients (FEUC in 90 patients; MTLE-HS in 110 patients) were included. Generalized epileptiform (spike/sharp waves simultaneously in all regions) and nonspecific generalized discharges (paroxysmal slow waves) were investigated. All clinical and laboratory findings of 2 groups were compared with each other and with remaining control group, without generalized paroxysms, statistically. Generalized EEG features were present in 22 (11%; 4 males) patients; 9 in the FEUC group (10%; 2) and 13 in the MTLE-HS group (11.8%). Female gender (P < .021), febrile seizure (P < .034), precipitant factors (P < .025), and parental consanguinity (P < .033) were significantly higher in the group with generalized EEG findings. Monotherapy rates were lower in the MTLE-HS group (P < .05). The relationship of generalized EEG features with female gender and parental consanguinity may point out to a genetic property among focal epilepsies, while the relationship with febrile seizures and precipitant factors may be a clue about mechanisms with more extensive involvement of the neuronal networks

Reflex epileptic features in patients with focal epilepsy of unknown cause

Objectives: There is a gap of knowledge regarding reflex seizures in patients with focal epilepsy of unknown cause (FEUC). We aimed to evaluate the prevalence, demographic and clinical characteristics of reflex seizures in patients with FEUC to provide an insight to the underlying ictogenic mechanisms and to draw attention to this important but under-investigated topic

Investigation of Magnetic Resonance Spectroscopy Findings in Patients with Absence Status Epilepticus

Objective: Absence status epilepticus (ASE) has been well recognized for many years, but its pathophysiology has not yet been illuminated and there are speculations about GABAergic mechanisms. We aimed to study the etiopathogenesis of ASE by using magnetic resonance-spectroscopy (MRS), which gives in vivo information about neuronal loss and/or dysfunction by correlating the results with age- and sex-matched normal healthy controls (HC)