Motor chronic inflammatory demyelinating polyneuropathy (CIDP) in 17 patients: Clinical characteristics, electrophysiological study, and response to treatment.

Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis.

Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.

Objective: To describe the main syndrome and clinical course in a large cohort of patients with anti-Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).

Methods: Twenty-year retrospective nationwide study and systematic review of the literature.

Results: Thirty-six patients with complete clinical information were identified (median age 66 years, range: 47-87 years).

Guillain-Barré Syndrome During Platinum-Based Chemotherapy: A Case Series and Review of the Literature.

Platinum-based chemotherapy is commonly associated with toxic sensory neuropathies, but also, although rarely, with Guillain-Barré syndrome (GBS). We describe five patients who developed GBS while receiving platinum-based chemotherapy for a solid tumor and report the five cases published so far. Most patients had received cumulative platinum doses below known neurotoxic levels, and all of them had an optimal outcome after platinum discontinuation, associated in most cases with administration of intravenous immunoglobulin.

Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors.

Objective: To report the induction of anti-Ma2 antibody-associated paraneoplastic neurologic syndrome (Ma2-PNS) in 6 patients after treatment with immune checkpoint inhibitors (ICIs). We also analyzed (1) patient clinical features compared with a cohort of 44 patients who developed Ma2-PNS without receiving ICI treatment and (2) the frequency of neuronal antibody detection before and after ICI implementation.

Methods: Retrospective nationwide study of all patients with Ma2-PNS developed during ICI treatment between 2017 and 2018.

Dystonia and dopamine: From phenomenology to pathophysiology.

A line of evidence suggests that the pathophysiology of dystonia involves the striatum, whose activity is modulated among other neurotransmitters, by the dopaminergic system. However, the link between dystonia and dopamine appears complex and remains unclear. Here, we propose a physiological approach to investigate the clinical and experimental data supporting a role of the dopaminergic system in the pathophysiology of dystonic syndromes.

Structural and functional brain biomarkers of clinical response to rTMS of medication-resistant auditory hallucinations in schizophrenia patients: study protocol for a randomized sham-controlled double-blind clinical trial.

Background: The potential of non-invasive repetitive transcranial magnetic stimulation (rTMS) to improve auditory verbal hallucinations (AVH) in schizophrenia patients has been increasingly explored over the past decade. Despite highly promising results, high inter-individual variability of clinical response and ineffective outcomes in a significant number of patients underscored the need to identify factors associated with the clinical response to rTMS. It should help improve the efficacy of rTMS in patients with medication-resistant AVH, and allow a better understanding of its neural impact.

Non convulsive status epilepticus in the elderly.

Non-convulsive status epilepticus (NCSE) is common in the elderly. It most often corresponds to prolonged focal seizures with impaired contact ("complex partial status epilepticus"). A form of de novo absence status epilepticus, much rarer, can also meet.

Does Bilateral Deep Brain Stimulation of the Subthalamic Nucleus Modify Ano-Rectal Motility in Parkinson's Disease? Results of a Randomized Cross-Over Study.

Background: Ano-rectal motility impairment is often observed during Parkinson's disease (PD), generating symptoms as constipation and/or incontinence with impaired quality of life. Subthalamic nuclei (STN) deep brain stimulation (DBS) improves motor symptoms of PD, but its effects on anorectal motility are unknown. This study aimed to assess the effects of STN-DBS on the anorectal motility in PD patients, in a randomized cross-over study.

Clinical and anatomical predictors for freezing of gait and falls after subthalamic deep brain stimulation in Parkinson's disease patients.

Introduction: Freezing of gait (FOG) and falls are the most disabling motor symptoms in Parkinson's disease (PD) patients. The effects of subthalamic deep-brain-stimulation (STN-DBS) on FOG and falls are still a matter of controversy, and factors contributing to their outcome have yet to be defined.

Methods: We examined the relationship between FOG and falls after STN-DBS and preoperative clinical features, MRI voxel-based-morphometry (VBM) analysis and statistical mapping of electrode locations.

Effect of short exposure to radiofrequency electromagnetic fields on saliva biomarkers: a study on the electrohypersensitive individuals.

Some individuals are reporting being sensitive to electromagnetic fields. They report some unspecific symptoms like headeache, sleep disruption, heart palpitations, itching, etc. The lack of scientific objective links between EHS symptoms and EMF exposure renders the diagnosis of EHS more complex.

Inhibition of Eph/ephrin interaction with the small molecule UniPR500 improves glucose tolerance in healthy and insulin-resistant mice.

Eph/ephrin interactions and their bidirectional signaling are integral part of the complex communication system between β-cells, essential for glucose homeostasis. Indeed, Eph/ephrin system was shown to be directly involved in the glucose-stimulated insulin secretion (GSIS) process occurring in the pancreatic islets. Here we tested the Eph antagonist UniPR500 as GSIS enhancer.

Glycosaminoglycans from Alzheimer's disease hippocampus have altered capacities to bind and regulate growth factors activities and to bind tau.

Glycosaminoglycans (GAGs), including heparan sulfates and chondroitin sulfates, are major components of the extracellular matrix. Upon interacting with heparin binding growth factors (HBGF), GAGs participate to the maintaintenance of tissue homeostasis and contribute to self-healing. Although several processes regulated by HBGF are altered in Alzheimer's disease, it is unknown whether the brain GAG capacities to bind and regulate the function of HBGF or of other heparin binding proteins, as tau, are modified in this disease.

The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study.

Unlabelled: Spinal muscular atrophy (SMA) type III and IV are autosomal recessive, slowly progressive lower motor neuron syndromes. Nevertheless, wider cerebral involvement has been consistently reported in mouse models. The objective of this study is the characterisation of spinal and cerebral pathology in adult forms of SMA using multimodal quantitative imaging.

Isolated seizures are a common early feature of paraneoplastic anti-GABA receptor encephalitis.

Objective: To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABA receptor antibodies (GABAR-Abs).

Methods: Retrospective clinical study of CSF-confirmed cases of GABAR-Abs encephalitis.

Results: We identified 22 patients (4 female) with GABAR-Abs, with a median age of 64 years (range 55-85).

Critical brain regions related to post-stroke aphasia severity identified by early diffusion imaging are not the same when predicting short- and long-term outcome.

Objectives: To identify the critical brain regions associated with 7-days, 3 and 6-months aphasia severity using diffusion-weighted imaging (DWI) in acute post-stroke patients.

Materials And Methods: We performed a voxel-based ADC (Apparent Diffusion Coefficient) analysis to identify the critical brain areas correlated with aphasia at the acute (7-days outcome) and chronic stages (3 and 6-months). The location of these areas was compared with the trajectory of the dorsal (the arcuate fasciculus) and the ventral language pathways (the inferior fronto-occipital and the uncinate fasciculi).

Beta burst coupling across the motor circuit in Parkinson's disease.

Exaggerated activity in the beta band (13-35 Hz) is a hallmark of basal ganglia signals in patients with Parkinson's disease (PD). Beta activity however is not constantly elevated, but comes in bursts. In previous work we showed that the longer beta bursts are maintained, the more the oscillatory synchronisation within the subthalamic nucleus (STN) increases, which is posited to limit the information coding capacity of local circuits.

Integrating EEG and MEG Signals to Improve Motor Imagery Classification in Brain-Computer Interface.

We adopted a fusion approach that combines features from simultaneously recorded electroencephalogram (EEG) and magnetoencephalogram (MEG) signals to improve classification performances in motor imagery-based brain-computer interfaces (BCIs). We applied our approach to a group of 15 healthy subjects and found a significant classification performance enhancement as compared to standard single-modality approaches in the alpha and beta bands. Taken together, our findings demonstrate the advantage of considering multimodal approaches as complementary tools for improving the impact of noninvasive BCIs.

Full sequencing and haplotype analysis of MAPT in Parkinson's disease and rapid eye movement sleep behavior disorder.

Background: MAPT haplotypes are associated with PD, but their association with rapid eye movement sleep behavior disorder is unclear.

Objective: To study the role of MAPT variants in rapid eye movement sleep behavior disorder.

Methods: Two cohorts were included: (A) PD (n = 600), rapid eye movement sleep behavior disorder (n = 613) patients, and controls (n = 981); (B) dementia with Lewy bodies patients with rapid eye movement sleep behavior disorder (n = 271) and controls (n = 950).

LRRK2 protective haplotype and full sequencing study in REM sleep behavior disorder.

Background: Individuals with rapid eye movement (REM)-sleep behavior disorder (RBD) are likely to progress to synucleinopathies, mainly Parkinson's disease (PD), dementia with Lewy-bodies (DLB) and multiple system atrophy (MSA). The genetics of RBD only partially overlaps with PD and DLB, and the role of LRRK2 variants in risk for RBD is still not clear.

Methods: The full coding sequence, exon-intron boundaries and 5' and 3' untranslated regions of LRRK2 were sequenced using targeted next-generation sequencing.