Factors influencing response to Botulinum toxin type A in patients with idiopathic cervical dystonia: results from an international observational study

Objectives Real-life data on response to Botulinum toxin A (BoNT-A) in cervical dystonia (CD) are sparse. An expert group of neurologists was convened with the overall aim of developing a definition of treatment response, which could be applied in a non-interventional study of BoNT-A-treated subjects with CD. Design International, multicentre, prospective, observational study of a single injection cycle of BoNT-A as part of normal clinical practice. Setting 38 centres across Australia, Belgium, Czech Republic, France, Germany, The Netherlands, Portugal, Russia and the UK. Participants 404 adult subjects with idiopathic CD. Most subjects were women, aged 41–60 years and had previously received BoNT-A. Outcome measures Patients were classified as responders if they met all the following four criteria: magnitude of effect (≥25% improvement Toronto Western Spasmodic Torticollis Rating Scale), duration of effect (≥12-week interval between the BoNT-A injection day and subject-reported waning of treatment effect), tolerability (absence of severe related adverse event) and subject's positive Clinical Global Improvement (CGI). Results High rates of response were observed for magnitude of effect (73.6%), tolerability (97.5%) and subject's clinical global improvement (69.8%). The subjective duration of effect criterion was achieved by 49.3% of subjects; 28.6% of subjects achieved the responder definition. Factors most strongly associated with response were age (<40 years; OR 3.9, p<0.05) and absence of baseline head tremor (OR 1.5; not significant). Conclusions Three of four criteria were met by most patients. The proposed multidimensional definition of response appears to be practical for routine practice. Unrealistically high patient expectation and subjectivity may influence the perception of a quick waning of effect, but highlights that this aspect may be a hurdle to response in some patients. Clinical registration number (NCT00833196; ClinicalTrials.gov)

Leucine-Rich Glioma-Inactivated 1 Encephalitis: Broadening the Sphere

Background: Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a rare entity. Its typical features are seizures, faciobrachial dystonic seizures (FBDS), cognitive impairment, and personality changes. Case report: We report the case of a 66-year-old man with an unusual presentation, consisting of two types of FBDS, one starting in the foot and the other consisting of asynchronous myoclonic and dystonic jerks of the face triggered by noise and chin stimulation. The patient displayed no personality changes or cognitive impairment. Discussion: LGI1 encephalitis is a heterogeneous disease. Many different forms of FBDS may be observed, and these seizures can be the only symptom. This type of encephalitis should be suspected in presenting very frequent episodic events with dystonic features, regardless of the part of the body affected

Méthodes non-invasives (PAS et rPAS) d'induction de plasticité corticale appliquées sur les muscles extenseurs du poignet chez les sujets sains et les patients hémiplégiques

TOULOUSE3-SCD-Bib. electronique (315559904) / SudocSudocFranceF

Effet de la levodopa sur la perception douloureuse dans la maladie de Parkinson

La douleur constitue une plainte fréquemment rencontrée dans la maladie de Parkinson. Cette douleur pourrait résulter d'une altération de la perception douloureuse secondaire au déficit dopaminergique central. La perception de la douleur chez les malades Parkinsoniens a fait l'objet de très peu d'études et les résultats sont hétérogènes. Dans cette étude randomisée, menée en ouvert 13 patients parkinsoniens et 10 volontaires sains ont été inclus. Le seuil nociceptif était évalué dans deux conditions différentes (avec ou sans L-dopa). Le seuil nociceptif était évalué grâce à deux modalités différentes de stimulation douloureuse : une stimulation thermique (au froid) et une stimulation électrique (estimation verbale et mesure du réflexe R III). Nous montrons que la L-dopa améliore significativement le seuil douloureux chez les patients parkinsoniens quelque soit la modalité testée. De plus les patients parkinsoniens présentent un seuil douloureux plus bas que les volontaires sains. En conclusion, nous confirmons une altération de la perception douloureuse chez les patients parkinsoniens. D'autre part la L-dopa semble normaliser la perception douloureuse chez ces mêmes patients parkinsoniens. Ce travail pilote nécessite d'être validé par un travail contre placebo en double aveugle.TOULOUSE3-BU Santé-Centrale (315552105) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

[Functional neuroimaging and the treatment of aphasia: speech therapy and repetitive transcranial magnetic stimulation]

International audienceFunctional imaging has provided new evidence of the neurobiological impact of the treatment of aphasia, including speech therapy, through the alteration of the activated language neural network. In such a way, speech therapy has proved its impact. The role of each hemisphere is still very unclear. Some of the authors link the left-lateralisation of activations to the therapeutic improvement of language and the right-activated network to a maladaptative strategy, whereas others consider the latter as a useful compensatory network for speech disorders. Repetitive trans-cranial magnetic stimulation (rTMS), first used to determine cortical activity, is now used to directly interfere with cerebral activity. In the years to come, rTMS should be developed as an adjuvant therapy for aphasia

Distinctive features of NREM parasomnia behaviors in parkinson's disease and multiple system atrophy.

To characterize parasomnia behaviors on arousal from NREM sleep in Parkinson's Disease (PD) and Multiple System Atrophy (MSA).From 30 patients with PD, Dementia with Lewy Bodies/Dementia associated with PD, or MSA undergoing nocturnal video-polysomnography for presumed dream enactment behavior, we were able to select 2 PD and 2 MSA patients featuring NREM Parasomnia Behviors (NPBs). We identified episodes during which the subjects seemed to enact dreams or presumed dream-like mentation (NPB arousals) versus episodes with physiological movements (no-NPB arousals). A time-frequency analysis (Morlet Wavelet Transform) of the scalp EEG signals around each NPB and no- NPB arousal onset was performed, and the amplitudes of the spectral frequencies were compared between NPB and no-NPB arousals.19 NPBs were identified, 12 of which consisting of 'elementary' NPBs while 7 resembling confusional arousals. With quantitative EEG analysis, we found an amplitude reduction in the 5-6 Hz band 40 seconds before NPBs arousal as compared to no-NPB arousals at F4 and C4 derivations (p<0.01).Many PD and MSA patients feature various NREM sleep-related behaviors, with clinical and electrophysiological differences and similarities with arousal parasomnias in the general population.This study help bring to attention an overlooked phenomenon in neurodegenerative diseases

Time–frequency chart of t-statistics comparing normalized EEG amplitude for the NPB arousals versus no-NPB arousals at F3, Fz, F4, C3, Cz, C4, Pz, O1, and O2.

<p>The arousal onset is the 0 of the time axis. Note a significant amplitude reduction in the 5–6 Hz range frequency (light blue horizontal line) in NPB compared to no-NPB arousals at F4 and C4 derivations, starting around 40 seconds before the arousal. The orange-and-yellow spot around the arousal is due to movement artefact and is not the expression of an increase in the low frequency band amplitude. The t-map was thresholded for display at p<0.01 uncorrected.</p

Electromagnetic articulography assessment of tongue function in non-dysarthric speakers with Parkinson’s disease

Objective: To compare the levels of lipid peroxidation products between Parkinson’s disease (PD) and study controls, and the relationship of these products in relation to clinical progression in PD. Background: Oxidative stress may be important in the pathogenesis of Parkinson’s disease (PD). The extent of oxidative stress damage was assessed using markers of lipid peroxidation in a cohort of PD patients and study controls. Methods: A total of 62 PD patients and 86 age-gender matched study controls (25 ischemic stroke and 61 community-based healthy controls) participated in this study. Their demographic and clinical characteristics were obtained using a standardized questionnaire. In PD patients, the clinical severity of their disease was assessed using the Hoehn-Yahr scale and the Unified Parkinson’s Disease Rating scales (UPDRS), and their cumulative exposure to levodopa calculated. Markers of lipid peroxidation (F2-isoprostanes, F2-IsoPs; hydroxyeicosatetraenoic acid, HETEs; and cholesterol oxidation products, COPs), were assessed in the plasma and urine samples using the gas chromatography-mass spectrometry method. Results: The mean (standard deviation) age was 64 (8) years and there were no differences in the gender, ethnicity and medical history in the PD and study control groups (p>0.05). Higher levels of plasma esterified F2-IsoPs, plasma free HETEs and COPs were observed in PD patients as compared to stroke and healthy controls (p<0.05, t-test). No significant correlation was observed between F2- IsoPs, HETEs and COPs in relation to the cumulative dosage of levodopa (r5 -0.15 to 0.21). A significant decrease in plasma free F2-IsoPs was observed with clinical progression of PD, according to the Hoehn-Yahr (p-trend<0.05) and UPDRS severity scales (r5 -0.372, p50.004). After adjusting for age, gender and cumulative levodopa dosage, lower plasma levels of free F2-IsoPs independently predicted UPDRS scores (OR -292, 95% CI -527 to -56). Conclusions: We identified certain markers of lipid peroxidation and COPs to be elevated in PD and suggested the use of plasma free F2-IsoPs as a potential marker of clinical progression

Flow chart explaining the selection procedure of the patients and on the NREM Parasomnia Behaviors (NPBs) on which subsequent analyses were conducted.

<p>Flow chart explaining the selection procedure of the patients and on the NREM Parasomnia Behaviors (NPBs) on which subsequent analyses were conducted.</p