New insight in spiral drawing analysis methods - Application to action tremor quantification.

Objective: Spiral drawing is one of the standard tests used to assess tremor severity for the clinical evaluation of medical treatments. Tremor severity is estimated through visual rating of the drawings by movement disorders experts. Different approaches based on the mathematical signal analysis of the recorded spiral drawings were proposed to replace this rater dependent estimate.

SCA13 causes dominantly inherited non-progressive myoclonus ataxia.

Introduction: Spinocerebellar ataxia 13 (SCA13) is a rare autosomal dominant cerebellar ataxia. To our knowledge, its association to movement disorders has never been described. We aimed at reporting 8 new SCA13 cases with a focus on movement disorders especially myoclonus.

Orthostatic tremor: a cerebellar pathology?

SEE MUTHURAMAN ET AL DOI101093/AWW164 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Primary orthostatic tremor is characterized by high frequency tremor affecting the legs and trunk during the standing position. Cerebellar defects were suggested in orthostatic tremor without direct evidence. We aimed to characterize the anatomo-functional defects of the cerebellar motor pathways in orthostatic tremor.

A randomized, controlled, double-blind, crossover trial of zonisamide in myoclonus-dystonia.

Objective: To evaluate the efficacy and safety of zonisamide in patients with myoclonus-dystonia.

Methods: We conducted a randomized, double-blind, placebo-controlled crossover trial of zonisamide (300 mg/d) in 24 patients with myoclonus-dystonia. Each treatment period consisted of a 6-week titration phase followed by a 3-week fixed-dose phase.

Intrinsic signature of essential tremor in the cerebello-frontal network.

Essential tremor is a movement disorder characterized by tremor during voluntary movements, mainly affecting the upper limbs. The cerebellum and its connections to the cortex are known to be involved in essential tremor, but no task-free intrinsic signatures of tremor related to structural cerebellar defects have so far been found in the cortical motor network. Here we used voxel-based morphometry, tractography and resting-state functional MRI at 3 T to compare structural and functional features in 19 patients with essential tremor and homogeneous symptoms in the upper limbs, and 19 age- and gender-matched healthy volunteers.

FXTAS: new insights and the need for revised diagnostic criteria.

Objective: Fragile X-associated tremor ataxia syndrome (FXTAS) is defined by FMR1 premutation, cerebellar ataxia, intentional tremor, and middle cerebellar peduncle (MCP) hyperintensities. We delineate the clinical, neurophysiologic, and morphologic characteristics of FXTAS.

Methods: Clinical, morphologic (brain MRI, (123)I-ioflupane SPECT), and neurophysiologic (tremor recording, nerve conduction studies) study in 22 patients with FXTAS, including 4 women.

Characterization of an endovascular prosthesis using the 3Bs rule (biocompatibility, biofunctionality and biodurability): a recommended protocol to investigate a device harvested at necropsy.

Numerous endovascular stent grafts to treat intrarenal aortic aneurysms are now commercially available, and many new concepts are currently in development worldwide. In order to objectively quantify their outcomes, we propose a detailed protocol to examine a reference device that was harvested from a patient who died a few hours after endovascular stent-graft deployment for an abdominal aortic aneurysm according to the 3Bs rule (biocompatibility, biofunctionality, and biodurability). Relevant patient history of this 63-year-old man included radiotherapy treatment for lung cancer.

Dopaminergic deficit is not the rule in orthostatic tremor.

Involvement of the dopaminergic system in orthostatic tremor is controversial. The aim of this study was to detect possible dopaminergic denervation in primary orthostatic tremor (OT). Twelve consecutive patients with a firm diagnosis of primary orthostatic tremor were compared with age-matched normal controls.

Analysis of sixty-two explanted Liotta bioprostheses: biocompatibility, biofunctionality, and biodurability issues.

Sixty-two explanted Liotta porcine bioprostheses were examined to review the issues related to their biocompatibility, biofunctionality, and biodurability. These bioprostheses were harvested from 56 patients with implantation times ranging from only a few hours to more than nine years of implantation. There were 10 acute and short-term (< 1 year), 20 midterm (1 < t < 5 years), and 32 long-term (> 5 years) cases.

Myoclonus or tremor in orthostatism: an under-recognized cause of unsteadiness in Parkinson's disease.

Patients with Parkinson's disease (PD) often complain of unsteadiness. This can occur as the result of various neurological dysfunctions, including changes in postural adjustments, loss of postural reflexes, axial akinesia and rigidity, freezing and/or postural hypotension. In some cases these symptoms remain unexplained, and rare cases of unsteadiness have been attributed to tremor on standing.

Newly developed hybrid suture without lubricant: noninvasive in vivo assessment of biocompatibility with multiparametric MR imaging.

Magnetic resonance imaging (MRI) and magnetic resonance (MR) relaxometry were used to assess noninvasively the tissue response of a new uncoated hybrid braided suture made from a combination of ultra-high-molecular-weight polyethylene (UHMWPE) and polyester (polyethylene terephthalate) (PET) yarns in comparison to a silicone impregnated braided 100% polyester (PET) control suture (Ticron). Both biomaterials were monitored for a period of 30 days following implantation in both incised and nonincised paravertebral rabbit muscles. In all cases, MR images and relaxometry demonstrated that the hybrid suture elicited either a milder or a similar tissue and cellular response compared to the control suture.

Head tremor in Parkinson's disease.

Head tremor is a typical feature of essential tremor. Patients with sporadic Parkinson's disease can have tremor of the tongue, lip, or chin, but classically do not have head tremor. We describe five patients with Parkinson's disease and head tremor in whom clinical and neurophysiological findings suggested that head tremor was a manifestation of Parkinson's disease.

Biocompatibility studies of the Anaconda stent-graft and observations of nitinol corrosion resistance.

Purpose: To validate the deployment, in vivo performance, biostability, and healing capacity of the Anaconda self-expanding endoprosthesis in a canine aortic aneurysm model.

Methods: Aneurysms were surgically created in 12 dogs by sewing a woven polyester patch onto the anterior side of the thoracic or abdominal aorta. Anaconda prostheses were implanted transfemorally for prescheduled periods (1 or 3 months).