Stiff person syndrome and other immune-mediated movement disorders - new insights

PURPOSE OF REVIEW: This review highlights the recent developments in immune-mediated movement disorders and how they reflect on clinical practice and our understanding of the underlying pathophysiological mechanisms. RECENT FINDINGS: The antibody spectrum associated with stiff person syndrome and related disorders (SPSD) has broadened and, apart from the classic glutamic acid decarboxylase (GAD)- and amphiphysin-antibodies, includes now also antibodies against dipeptidyl-peptidase-like protein-6 (DPPX), gamma-aminobutyric acid type A receptor (GABAAR), glycine receptor (GlyR) and glycine transporter 2 (GlyT2). The field of movement disorders with neuronal antibodies keeps expanding with the discovery for example of antibodies against leucine rich glioma inactivated protein 1 (LGI1) and contactin associated protein 2 (Caspr2) in chorea, or antibodies targeting ARHGAP26- or Na+/K+ ATPase alpha 3 subunit (ATP1A3) in cerebellar ataxia. Moreover, neuronal antibodies may partly account for movement disorders attributed for example to Sydenham's chorea, coeliac disease, or steroid responsive encephalopathy with thyroid antibodies. Lastly, there is an interface of immunology, genetics and neurodegeneration, e.g. in Aicardi–Goutières syndrome or the tauopathy with IgLON5-antibodies. SUMMARY: Clinicians should be aware of new antibodies such as dipeptidyl-peptidase-like protein-6, gamma-aminobutyric acid type A receptor and glycine transporter 2 in stiff person syndrome and related disorders, as well as of the expanding spectrum of immune-mediated movement disorders

Microstructural and Corrosion Properties of Cold Rolled Laser Welded UNS S32750 Duplex Stainless Steel

The main goal of this work was to study the effect of plastic deformation on weldability of duplex stainless steel (DSS). It is well known that plastic deformation prior to thermal cycles can enhance secondary phase precipitation in DSS which can lead to significant change of the ferrite-austenite phase ratio. From this point of view one of the most important phase transformation in DSS is the eutectoid decomposition of ferrite. Duplex stainless steels (DSSs) are a category of stainless steels which are employed in all kinds of applications where high strength and excellent corrosion resistance are both required. This favorable combination of properties is provided by their biphasic microstructure, consisting of ferrite and austenite in approximately equal volume fractions. Nevertheless, these materials may suffer from several microstructural transformations if they undergo heat treatments, welding processes or thermal cycles. These transformations modify the balanced phase ratio, compromising the corrosion and mechanical properties of the material. In this paper, the microstructural stability as a consequence of heat history due to welding processes has been investigated for a super duplex stainless steel (SDSS) UNS S32750. During this work, the effects of laser beam welding on cold rolled UNS S32750 SDSS have been investigated. Samples have been cold rolled at different thickness reduction (\u3b5 = 9.6%, 21.1%, 29.6%, 39.4%, 49.5%, and 60.3%) and then welded using Nd:YAG laser. Optical and electronical microscopy, eddy\u2019s current tests, microhardness tests, and critical pitting temperature tests have been performed on the welded samples to analyze the microstructure, ferrite content, hardness, and corrosion resistance. Results show that laser welded joints had a strongly unbalanced microstructure, mostly consisting of ferritic phase (~60%). Ferrite content decreases with increasing distance from the middle of the joint. The heat-affected zone (HAZ) was almost undetectable and no defects or secondary phases have been observed. Both hardness and corrosion susceptibility of the joints increase. Plastic deformation had no effects on microstructure, hardness or corrosion resistance of the joints, but resulted in higher hardness of the base material. Cold rolling process instead, influences the corrosion resistance of the base material

Tardive syndromes

Dopamine receptor-blocking antipsychotics, first introduced into clinical practice in 1952, were hailed as a panacea in the treatment of a number of psychiatric disorders. However, within 5 years, this notion was to be shattered by the recognition of both acute and chronic drug-induced movement disorders which can accompany their administration. Tardive syndromes, denoting the delayed onset of movement disorders following administration of dopamine receptor-blocking (and also other) drugs, have diverse manifestations ranging from the classic oro-bucco-lingual dyskinesia, through dystonic craniocervical and trunk posturing, to abnormal breathing patterns. Although tardive syndromes have been an important part of movement disorder clinical practice for over 60 years, their pathophysiologic basis remains poorly understood and the optimal treatment approach remains unclear. This review summarises the current knowledge relating to these syndromes and provides clinicians with pragmatic, clinically focused guidance to their management

The Dynamics of Interethnic Friendships and Negative Ties in Secondary School:The Role of Peer-Perceived Ethnicity

This study examines ethnic integration in secondary school. Social identity theory suggests that perception of relevant individual attributes plays a crucial role in defining ingroups and outgroups, contributing to befriending, and disliking others. Therefore, we analyze the role of peer-perceived ethnicity in social ties. Networks of friendship, dislike, and perceived ethnicity were modeled together using dynamic stochastic actor-oriented models, separating the effect of perceived ethnicity on social ties from that of social ties on perceived ethnicity. Data came from a Hungarian sample of 12 school classes with one minority group: the Roma. Treating friendship and dislike as mutually exclusive and comparing them to neutral relations, we found evidence for the role of perceived ethnicity in dislike-majority students disliked those they perceived as minority peers. However, we saw no direct effect of ethnicity on the friendship network. Implications of the joint modeling of mutually exclusive relationship aspects are discussed

Parkinsonism and dystonia: Clinical spectrum and diagnostic clues

The links between the two archetypical basal ganglia disorders, dystonia and parkinsonism, are manifold and stem from clinical observations, imaging studies, animal models and genetics. The combination of both, i.e. the syndrome of dystonia-parkinsonism, is not uncommonly seen in movement disorders clinics and has a myriad of different underlying aetiologies, upon which treatment and prognosis depend. Based on a comprehensive literature review, we delineate the clinical spectrum of disorders presenting with dystonia-parkinsonism. The clinical approach depends primarily on the age at onset, associated neurological or systemic symptoms and neuroimaging. The tempo of disease progression, and the response to L-dopa are further important clues to tailor diagnostic approaches that may encompass dopamine transporter imaging, CSF analysis and, last but not least, genetic testing. Later in life, sporadic neurodegenerative conditions are the most frequent cause, but the younger the patient, the more likely the cause is unravelled by the recent advances of molecular genetics that are focus of this review. Here, knowledge of the associated phenotypic spectrum is key to guide genetic testing and interpretation of test results. This article is part of the Special Issue "Parkinsonism across the spectrum of movement disorders and beyond" edited by Joseph Jankovic, Daniel D. Truong and Matteo Bologna

Elastodynamic image forces on dislocations.

The elastodynamic image forces on edge and screw dislocations in the presence of a planar-free surface are derived. The explicit form of the elastodynamic fields of an injected, quiescent screw dislocation are also derived. The resulting image forces are affected by retardation effects: the dislocations experience no image force for a period of time defined by the arrival and reflection at the free surface of the dislocation fields. For the case of injected, stationary dislocations, it is shown that the elastodynamic image force tends asymptotically to the elastotatic prediction. For the case of injected, moving dislocations, it is shown that the elastodynamic image force on both the edge and the screw dislocations is magnified by inertial effects, and becomes increasingly divergent with time; this additional effect, missing in the elastostatic description, is shown to be substantial even for slow moving dislocations. Finally, it is shown that the elastodynamic image force of an edge dislocation moving towards the surface at the Rayleigh wave speed becomes repulsive, rather than attractive; this is suggestive of instabilities at the core of the dislocation, and likely resonances with the free surface.EPSRC via the EPSRC Doctoral Prize Fellowship progra

Tremor in motor neuron disease may be central rather than peripheral in origin

BACKGROUND AND PURPOSE: Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post-mortem observations have revealed considerable variability in the phenotype. Rhythmic involuntary oscillations of the hands during action, resembling tremor, can occur in MND, but their pathophysiology has not yet been investigated. METHODS: A total of 120 consecutive patients with MND were screened for tremor. Twelve patients with action tremor and no other movement disorders were found. Ten took part in the study. Tremor was recorded bilaterally using surface electromyography (EMG) and triaxial accelerometer, with and without a variable weight load. Power spectra of rectified EMG and accelerometric signal were calculated. To investigate a possible cerebellar involvement, eyeblink classic conditioning was performed in five patients. RESULTS: Action tremor was present in about 10% of our population. All patients showed distal postural tremor of low amplitude and constant frequency, bilateral with a small degree of asymmetry. Two also showed simple kinetic tremor. A peak at the EMG and accelerometric recordings ranging from 4 to 12 Hz was found in all patients. Loading did not change peak frequency in either the electromyographic or accelerometric power spectra. Compared with healthy volunteers, patients had a smaller number of conditioned responses during eyeblink classic conditioning. CONCLUSIONS: Our data suggest that patients with MND can present with action tremor of a central origin, possibly due to a cerebellar dysfunction. This evidence supports the novel idea of MND as a multisystem neurodegenerative disease and that action tremor can be part of this condition

Benign tremulous parkinsonism of the young-consider Parkin

Benign tremulous parkinsonism is generally considered a disease of the elderly, characterised by dominance of tremor over other motor manifestations, and by slower disease progression. Herein, we draw attention to a different clinical syndrome, benign tremulous parkinsonism of the young, which we have observed in Parkin disease

A dynamic discrete dislocation plasticity method for the simulation of plastic relaxation under shock loading

In this article, it is demonstrated that current methods of modelling plasticity as the collective motion of discrete dislocations, such as two-dimensional discrete dislocation plasticity (DDP), are unsuitable for the simulation of very high strain rate processes (106 s-1 or more) such as plastic relaxation during shock loading. Current DDP models treat dislocations quasi-statically, ignoring the time-dependent nature of the elastic fields of dislocations. It is shown that this assumption introduces unphysical artefacts into the system when simulating plasticity resulting from shock loading. This deficiency can be overcome only by formulating a fully time-dependent elastodynamic description of the elastic fields of discrete dislocations. Building on the work of Markenscoff & Clifton, the fundamental time-dependent solutions for the injection and non-uniform motion of straight edge dislocations are presented. The numerical implementation of these solutions for a single moving dislocation and for two annihilating dislocations in an infinite plane are presented. The application of these solutions in a two-dimensional model of timedependent plasticity during shock loading is outlined here and will be presented in detail elsewhere. © 2013 The Author(s) Published by the Royal Society. All rights reserved