Electro-Clinical Features and Functional Connectivity Analysis in SYN1-Related Epilepsy.

Objective: There is currently scarce data on the electroclinical characteristics of epilepsy associated with synapsin 1 (SYN1) pathogenic variations. We examined clinical and electro-encephalographic (EEG) features in patients with epilepsy and SYN1 variants, with the aim of identifying a distinctive electroclinical pattern.

Methods: In this retrospective multicenter study, we collected and reviewed demographic, genetic, and epilepsy data of 19 male patients with SYN1 variants.

Complex visual discrimination is impaired after right, but not left, anterior temporal lobectomy.

The prevailing view in human cognitive neuroscience associates the medial temporal lobes (MTLs) with declarative memory. Compelling experimental evidence has, however, demonstrated that these regions are specialized according to the representations processed, irrespective of the cognitive domain assessed. This account was supported by the study of patients with bilateral medial temporal amnesia, who exhibit impairments in perceptual tasks involving complex visual stimuli.

Benign Paroxysmal Vertigo of Childhood. Video Recordings of Episodes.

Objectives: The main objective was to describe the nystagmus observed during benign paroxysmal vertigo (BPV) of childhood, which is one of the criteria included in the three versions of the International Classification of Headache Disorders that has never been specified. The secondary objectives were to emphasize the usefulness of a mobile phone to record nystagmus and discuss the physiopathology of this nystagmus.

Patient: A 6-year-old boy complained of approximately 30 to 50 vertigo attacks, most of them lasting around 1 minute, during a 6-month period.

Safety and efficacy of prophylactic levetiracetam for prevention of epileptic seizures in the acute phase of intracerebral haemorrhage (PEACH): a randomised, double-blind, placebo-controlled, phase 3 trial.

Background: The incidence of early seizures (occurring within 7 days of stroke onset) after intracerebral haemorrhage reaches 30% when subclinical seizures are diagnosed by continuous EEG. Early seizures might be associated with haematoma expansion and worse neurological outcomes. Current guidelines do not recommend prophylactic antiseizure treatment in this setting.

Usefulness and prognostic value of diagnostic tests in patients with possible chronic inflammatory demyelinating polyradiculoneuropathy.

Introduction/aims: Recent guidelines define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and possible CIDP. The aims of our study were to evaluate the value of diagnostic tests to support the diagnosis of CIDP in patients with possible CIDP and to identify prognostic factors of therapeutic success.

Methods: We conducted an observational retrospective two-center study between 2014 and 2019.

Coffee consumption and seizure frequency in patients with drug-resistant focal epilepsy.

Objective: To assess the relation between coffee consumption and seizure frequency in patients with drug-resistant focal epilepsy.

Methods: Cross-sectional analysis of data collected in the SAVE study, which included patients with drug-resistant focal epilepsy during long-term EEG monitoring. Patients in whom both coffee consumption and data about seizure frequency, including focal to bilateral tonic-clonic seizures (FBTCS), were available were selected.

Clinical Features and Management of Drop Attacks in Menière's Disease. Special Emphasis on the Possible Occurrence of Vertigo After the Drop Attacks.

Introduction: Tumarkin first described drop attacks (DA) in patients with a peripheral vestibular syndrome and speculated the role of a mechanical deformation of the otolith organs. We emphasized on the possible occurrence of vertigo/dizziness after a DA. In the light of the oculomotor examination of one patient right after the DA, we discussed on the mechanisms.

Cardiac Autonomic Dysfunction and Risk of Sudden Unexpected Death in Epilepsy.

Objective: We aimed to test whether patients who died of sudden unexpected death in epilepsy (SUDEP) had an abnormal cardiac autonomic response to sympathetic stimulation by hyperventilation.

Methods: We conducted a retrospective, observational, case-control study of a group of patients who died of SUDEP and controls who were matched to the patients for epilepsy type, drug resistance, sex, age at EEG recording, age at onset of epilepsy, and duration of epilepsy. We analyzed the heart rate (HR) and HR variability (HRV) at rest and during and after hyperventilation performed during the patient's last EEG recording before SUDEP.

A hidden mesencephalic variant of central pain.

Background: Central post-stroke pain (CPSP) can arise after lesions anywhere in the central somatosensory pathways, essentially within the spinothalamic system (STS). Although the STS can be selectively injured in the mesencephalon, CPSP has not been described in pure midbrain infarcts.

Methods: Of more than 300 CPSP consecutive cases, we describe five patients who developed definite neuropathic pain following lesions circumscribed to the postero-lateral mesencephalon.

Autoimmune episodic ataxia in patients with anti-CASPR2 antibody-associated encephalitis.

Objective: To report paroxysmal episodes of cerebellar ataxia in a patient with anti-contactin-associated protein-like 2 (CASPR2) antibody-related autoimmune encephalitis and to search for similar paroxysmal ataxia in a cohort of patients with anti-CASPR2 antibody-associated autoimmune encephalitis.

Methods: We report a patient with paroxysmal episodes of cerebellar ataxia observed during autoimmune encephalitis with anti-CASPR2 antibodies. In addition, clinical analysis was performed in a retrospective cohort of 37 patients with anti-CASPR2 antibodies to search for transient episodes of ataxia.

Efficacy of naloxone in reducing postictal central respiratory dysfunction in patients with epilepsy: study protocol for a double-blind, randomized, placebo-controlled trial.

Background: Generalized tonic-clonic seizures (GTCSs) are the main risk factor for sudden unexpected death in epilepsy (SUDEP). Experimental and clinical data strongly suggest that the majority of SUDEP results from a postictal respiratory dysfunction progressing to terminal apnea. Postictal apnea could partly derive from a seizure-induced massive release of endogenous opioids.

Thalamic pain: anatomical and physiological indices of prediction.

Thalamic pain is a severe and treatment-resistant type of central pain that may develop after thalamic stroke. Lesions within the ventrocaudal regions of the thalamus carry the highest risk to develop pain, but its emergence in individual patients remains impossible to predict. Because damage to the spino-thalamo-cortical system is a crucial factor in the development of central pain, in this study we combined detailed anatomical atlas-based mapping of thalamic lesions and assessment of spinothalamic integrity using quantitative sensory analysis and laser-evoked potentials in 42 thalamic stroke patients, of whom 31 had developed thalamic pain.

New practical definitions for the diagnosis of autosomal recessive spastic ataxia of Charlevoix-Saguenay.

Objective: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by mutations in the SACS gene. SACS encodes sacsin, a protein whose function remains unknown, despite the description of numerous protein domains and the recent focus on its potential role in the regulation of mitochondrial physiology. This study aimed to identify new mutations in a large population of ataxic patients and to functionally analyze their cellular effects in the mitochondrial compartment.

Effects of aging on laser evoked potentials.

Introduction: Aging has been reported to reduce the amplitude of laser evoked potentials. However, it is unknown whether this effect depends on the length of the sensory fibers. This is an important issue, because most painful neuropathies are length-dependent.

Emergence of hyper empathy after right amygdalohippocampectomy.

We report a fascinating case of a patient with a hyper empathy that appeared after resective epilepsy surgery. This behavioral modification has remained unchanged since the surgery took place 13 years ago. Recent neuropsychological objective assessments confirmed hyper empathy in a self-report questionnaire, and revealed higher affective theory of mind than controls in a "Reading the Mind in the Eyes Task.

Angelman syndrome and pseudo-hypsarrhythmia: a diagnostic pitfall.

Angelman syndrome is a rare genetic disorder scarcely diagnosed before the age of two years. We report the case of an eight-month-old female presenting with severe hypotonia, myoclonus, suspected spasms and an electroencephalogram with hypsarrhythmic-like features. She was initially treated with vigabatrin which resulted in worsening of myoclonic jerks.

Explicit and implicit emotional processing modifications in pharmacoresistant left temporal lobe epilepsy and anxiodepressive disorders.

We examined whether anxiodepressive patients with left temporal lobe epilepsy could be differentiated from those with depression but without epilepsy on tasks that investigate attentional bias toward and explicit judgment of emotional stimuli. Eight depressive patients, eight anxiodepressive patients with epilepsy, and eight controls participated in the present study. Anxiodepressive with epilepsy and depressive patients had comparable depression scores and the same cognitive profile.

How a clock can change your pain? The illusion of duration and pain perception.

The intensity of experimental pain is known to be dependent on stimulation duration. However, it remains unknown whether this effect arises largely from the actual stimulus duration or is substantially influenced by the subject's perception of the stimulus duration. In the present study, we questioned this issue by misleading the perception of the duration of pain in a population of 36 healthy volunteers stimulated with a thermode.

Operculo-insular pain (parasylvian pain): a distinct central pain syndrome.

Central pain with dissociated thermoalgesic sensory loss is common in spinal and brainstem syndromes but not in cortical lesions. Out of a series of 270 patients investigated because of somatosensory abnormalities, we identified five subjects presenting with central pain and pure thermoalgesic sensory loss contralateral to cortical stroke. All of the patients had involvement of the posterior insula and inner parietal operculum.

[Advantages and limitations of electroneuromyography for analysis of upper limb pain].

Objective: To study the utility of electroneuromyography in analysis of upper limb pain as a function of the existence of a diagnostic hypothesis.

Methods: We retrospectively compared the consecutive electroneuromyographic examinations performed between 1 January and 30 September 2004. All recordings were performed by the same examiner in the neurophysiology clinic in the department of neurology of Saint-Etienne university hospital UHC at the request of hospital specialists or surgeon and private general practitioners.

Thalamic thermo-algesic transmission: ventral posterior (VP) complex versus VMpo in the light of a thalamic infarct with central pain.

The respective roles of the ventral posterior complex (VP) and of the more recently described VMpo (posterior part of the ventral medial nucleus) as thalamic relays for pain and temperature pathways have recently been the subject of controversy. Data we obtained in one patient after a limited left thalamic infarct bring some new insights into this debate. This patient presented sudden right-sided hypesthesia for both lemniscal (touch, vibration, joint position) and spinothalamic (pain and temperature) modalities.

Laser-evoked potential abnormalities in central pain patients: the influence of spontaneous and provoked pain.

We recorded laser-evoked cortical potentials (LEPs) in 54 consecutive patients presenting with unilateral neuropathic central pain (n = 42) or with lateralized pain of non-organic origin (n = 12). A number of cases in each group had superimposed hyperalgesia or allodynia. In patients with central pain, LEPs were significantly attenuated after stimulation over the painful territory, relative to stimulation of the homologous normal territory.