Deep brain stimulation for severe dystonia associated with Wilson disease: A prospective multicenter meta-analysis of an N-of-1 trial.

Background And Purpose: Disabling dystonia despite optimal medical treatment is common in Wilson disease (WD). No controlled study has evaluated the effect of deep brain stimulation (DBS) on dystonia related to WD. This study was undertaken to evaluate the efficacy of DBS on dystonia related to WD.

Kidney involvement in Wilson's disease: a review of the literature.

Wilson's disease (WD) is a rare inherited disease due to the mutation of the ATP7B gene, resulting in impaired hepatic copper excretion and its pathological accumulation in various organs such as the liver, the nervous system, or the kidneys. Whereas liver failure and neuropsychiatric disorders are the most common features, less is known about the renal complications. We conducted a review of the literature to define the characteristics and pathophysiology of kidney involvement during WD.

Can Patients with Wilson's Disease Develop Copper Deficiency?

Background: Wilson's disease (WD) is a rare genetic condition characterized by a copper overload in organs secondary to mutation in ATP7B gene. Lifelong decoppering treatments are the keystone of the treatment but must be regularly adapted to obtain a correct copper balance and could lead to copper deficiency (CD).

Objectives: Study the characteristics of CD in WD patients.

Clinical, imaging, and management features of symptomatic carotid web: Insight from CAROWEB registry.

Background: Although carotid web (CaW) is increasingly diagnosed as a cause of cryptogenic stroke, data are still limited to monocentric small sample cohort. To broaden knowledge on symptomatic CaW, CAROWEB registry has been recently implemented.

Aims: In a large cohort of symptomatic CaW patients, we described epidemiologic characteristics, admission clinical and imaging features, and the current management including the secondary preventive strategy choice made in comprehensive French Stroke Units.

Diagnosis and Outcomes of Late-Onset Wilson's Disease: A National Registry-Based Study.

Background: Wilson's disease (WD) is usually diagnosed in children and young adults; limited data exist on late-onset forms.

Objective: The aim was to characterize the clinical and paraclinical presentations, therapeutic management, and outcomes in patients with late-onset WD.

Methods: Patients diagnosed with WD after age 40 years were identified from the French Wilson's Disease Registry (FWDR).

Experience on switching trientine formulations in Wilson disease: Efficacy and safety after initiation of TETA 4HCl as substitute for TETA 2HCl.

Background And Aim: This retrospective, multicenter study aims to assess the efficacy and safety in Wilson disease (WD) patients treated with trientine tetrahydrochloride (TETA 4HCl) after switch from trientine dihydrochloride (TETA 2HCl).

Methods: In total, 68 WD patients with stable copper metabolism were identified to receive TETA 4HCl (Cuprior™) after previous treatment with TETA 2HCl. We analyzed biochemical markers such as urinary copper, serum copper, non-coeruloplasmin bound copper (NCC), and transaminases as well as clinical scores (APRI; FIB-4 score) at baseline with a follow-up (FU) of 12 months.

Efficacy and Safety of Two Salts of Trientine in the Treatment of Wilson's Disease.

Background: Wilson's disease (WD) is one of the few genetic disorders that can be successfully treated with pharmacological agents. Copper-chelating agents (D-penicillamine and Trientine salts) and zinc salts have been demonstrated to be effective. There are two salts of trientine.

Cerebello-Motor Paired Associative Stimulation and Motor Recovery in Stroke: a Randomized, Sham-Controlled, Double-Blind Pilot Trial.

Cerebellum is a key structure for functional motor recovery after stroke. Enhancing the cerebello-motor pathway by paired associative stimulation (PAS) might improve upper limb function. Here, we conducted a randomized, double-blind, sham-controlled pilot trial investigating the efficacy of a 5-day treatment of cerebello-motor PAS coupled with physiotherapy for promoting upper limb motor function compared to sham stimulation.

Embolus Retriever with Interlinked Cages (ERIC) versus conventional stent retrievers for thrombectomy: a propensity score-based analysis.

Background: The Embolus Retriever with Interlinked Cages (ERIC) is one of the latest devices for thrombectomies. It has several architectural features that are supposed to enhance its ability to remove clots and prevent distal emboli. We aimed to compare ERIC with standard stent retrievers (SRs) using propensity score (PS) matching.

Elucidating the Structural and Functional Correlates of Upper-Limb Poststroke Motor Impairment.

Background and Purpose- Many studies have attempted to bring to light the neural correlates of poststroke motor impairment, but few have used multimodal approach to explain it. The aim of this study was to elucidate neural structural and functional correlates of upper limb motor impairment by combining electrophysiological, anatomic, and functional neuroimaging data. Methods- Forty ischemic stroke patients (median [min-max] age: 63 [33-82] years, time poststroke: 3.

Anterior cerebral artery embolism during thrombectomy increases disability and mortality.

Objective: During thrombectomy, thromboembolic migration in previously unaffected territory may occur and is not systematically notified. We report our data on the incidence, predictors, and clinical outcome of anterior cerebral artery emboli (ACAE).

Methods: From a prospectively collected thrombectomy database of consecutive patients with anterior circulation stroke between January 2012 and December 2016, 690 angiographic images were analyzed to assess ACAE.

Specific impairments in visual processing following lesion side in hemianopic patients.

Introduction: Following unilateral damage of the primary visual cortex one of the most common visual field defects observed is Homonymous Hemianopia (HH), a loss of vision of the contralesional hemifield in each eye. The ipsilesional ("intact") part of the central visual field is often used to compensate for difficulties encountered in the peripheral hemianopic visual field. However, the quality of vision within the central visual field is not well-known.