Neurol Neuroimmunol Neuroinflamm
September 2024
Objectives: Encephalitis with anti-N-methyl-d-aspartate receptor antibodies (anti-NMDARe) is a rare disorder characterized by cognitive impairment, psychosis, seizures, and abnormal movements. Abnormal behaviors during REM sleep have not been described in anti-NMDARe.
Methods: Patients were monitored by video-polysomnography on a first night followed by multiple sleep latency tests and 18 hours of bed rest.
J Neurol Neurosurg Psychiatry
December 2023
Introduction: Autoimmune encephalitis (AE) diagnosis and follow-up remain challenging. Brain F-fluoro-deoxy-glucose positron emission tomography (FDG PET) has shown promising results in AE. Our aim was to investigate FDG PET alterations in AE, according to antibody subtype.
View Article and Find Full Text PDFHandb Clin Neurol
August 2022
Temporal lobe epilepsy (TLE) is the most common type of focal epilepsy. First descriptions of TLE date back in time and detailed portraits of epileptic seizures of temporal origin can be found in early medical reports as well as in the works of various artists and dramatists. Depending on the seizure onset zone, several subtypes of TLE have been identified, each one associated with peculiar ictal semiology.
View Article and Find Full Text PDFPurpose: Human neuronal activity, recorded in vivo from microelectrodes, may offer valuable insights into physiological mechanisms underlying human cognition and pathophysiological mechanisms of brain diseases, in particular epilepsy. Continuous and long-term recordings are necessary to monitor non predictable pathological and physiological activities like seizures or sleep. Because of their high impedance, microelectrodes are more sensitive to noise than macroelectrodes.
View Article and Find Full Text PDFAutoimmune encephalitis associated with antibodies directed against the leucine-rich glioma inactivated 1 (LGI1) protein is responsible for specific tonic-dystonic motor seizures. Although dysfunctions in neuronal excitability have been associated with anti-LGI1 autoantibodies, their relation to seizures remain inconclusive. We developed a new in vivo experimental rat model to determine whether inhibition of Kv1.
View Article and Find Full Text PDFFocal neuronal lipofuscinosis (FNL) is an uncommon epileptic disorder related to an excess of lipofuscin accumulation within dysmorphic-appearing neurons (DANs), whose epileptogenic mechanisms are still poorly understood. It shares some clinical and neuroimaging similarities with focal cortical dysplasia of type IIb (FCDIIb), but it represents a different pathological entity. Here, we identified two patients with FNL among a 10-year cohort of 323 patients who underwent neurosurgery for a focal pharmacoresistant epilepsy.
View Article and Find Full Text PDFCell Mol Life Sci
December 2021
Leucine-rich, glioma inactivated 1 (LGI1) is a secreted glycoprotein, mainly expressed in the brain, and involved in central nervous system development and physiology. Mutations of LGI1 have been linked to autosomal dominant lateral temporal lobe epilepsy (ADLTE). Recently auto-antibodies against LGI1 have been described as the basis for an autoimmune encephalitis, associated with specific motor and limbic epileptic seizures.
View Article and Find Full Text PDFBackground And Purpose: Seizures represent a core symptom of autoimmune encephalitides with specific therapeutic issues. To date, patients with new-onset seizures or established epilepsy are not systematically tested for autoimmune antibodies. We aimed to identify clinical and paraclinical criterion that could help to select patients requiring additional autoimmune antibodies serum and cerebrospinal fluid (CSF) detection.
View Article and Find Full Text PDFObjective: To highlight specific characteristics of seizure semiology and EEG features associated with different subtypes of autoimmune encephalitis (AE).
Methods: We systematically reviewed the seizure semiology and all the EEG recordings from patients with AE managed in a tertiary referral centre for epilepsy and a neuro-intensive care unit. Each characteristic across the different subtypes of AE was compared by post hoc analysis.
Objectives: To describe the characteristics, evolution and risk factors for long-term persistence of olfactory and gustatory dysfunctions (OGD) in COVID-19 outpatients.
Patients And Methods: We conducted a prospective study in SARS-CoV-2 infected outpatients with OGD. Weekly phone interviews were set up starting from COVID-19 onset symptoms and over the course of 60 days, using standardized questionnaires that included a detailed description of general symptoms and OGD.
How and when to stop antiepileptic drugs? Antiepileptic drugs withdrawal can be considered in seizure-free patients with a 2-year minimum complete remission. Risk of seizure recurrence, which is related both to the epilepsy syndrome and individual characteristics, must be evaluated and may rely on the use of a computer-based risk calculator. Identifying epileptic abnormalities on EEG and cortical lesions on brain MRI could lead to reconsider drug discontinuation.
View Article and Find Full Text PDFA reliable identification of a high-risk state for upcoming seizures may allow for preemptive treatment and improve the quality of patients' lives. We evaluated the ability of prodromal symptoms to predict preictal states using a machine learning (ML) approach. Twenty-four patients with drug-resistant epilepsy were admitted for continuous video-electroencephalographic monitoring and filled out a daily four-point questionnaire on prodromal symptoms.
View Article and Find Full Text PDFObjective: To perform a systematic analysis and scoring of brain MRI white matter hyperintensities (WMH) in adult-onset Krabbe disease.
Methods: We retrospectively collected basic clinical data and the first available brain MRI from patients with confirmed Krabbe disease with first clinical manifestations beyond 10 years of age. Data were obtained from our reference center for lysosomal diseases (n = 6) and from contacted authors of published articles describing patients with adult-onset Krabbe disease (n = 15).