Clinimetrics in Sjögrens syndrome / Clinimetría en el síndrome de Sjögren

ABSTRACT Introduction: Primary Sjögren's syndrome (pSS) is an autoimmune disease that can affect quality of life, cause disability, including progression to systemic complications in patients. In order to evaluate these components, several clinimetric scales have been used in pSS. Methods: In order to describe the most commonly used clinimetric scales in pSS, a systematic search of articles was carried out using Google Scholar, Scielo, Embase, Academic Search Ultimate, and Medline databases. Pubmed was used for the search in Medline, with the MeSH terms: 'Clinimetry'; 'Clinimetrics'; 'Quality of life'; 'Activity Index'; 'Scales'; 'Sjögren's syndrome'; linked with the Boolean connector AND. A total of 1081 articles published up to May 2018 were reduced to the 51 of the most relevant after application of inclusion criteria. Results: The most commonly used clinimetric scales in the evaluation of systemic involvement and quality of life in patients with pSS are described. Conclusion: Clinimetric methods are very useful from the point of view of follow-up, evaluation of response to treatment, perception of the disease by patients, and objective evaluation of clinical trials in pSS.

RESUMEN Introducción: El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune, cuyo compromiso se puede ver reflejado en la calidad de vida, incapacidad y progresión a complicaciones en los pacientes. Con el fin de evaluar estos componentes, diversas escalas clinimétricas se han utilizado en el SSp. Métodos: Para describir las escalas de clinimetría más utilizadas en el SSp, se realizó una búsqueda de artículos en las bases de datos Google Scholar, Scielo, Embase, Academic Search Ultimate y Medline. Se empleó Pubmed para la búsqueda en Medline, con los términos MeSH: «Clinimetry¼; «Clinimetrics¼; «Quality of life¼; « Activity index¼; «Scales¼; «Sjögren's syndrome¼; enlazados con el conector booleano AND. Se incluyeron 1.081 artículos publicados hasta mayo de 2018, que luego de la aplicación de los criterios de inclusión, se redujeron a 51 con la mayor relevancia. Resultados: Se describen las escalas de clinimetría más usadas en la evaluación del compromiso sistémico y de la calidad de vida en los pacientes con SSp. Conclusión: Los métodos clinimétricos tienen gran utilidad desde el punto de vista de seguimiento, evaluación de respuesta a tratamiento, percepción de la enfermedad por parte de los pacientes y evaluación objetiva de ensayos clínicos en el SSp.

Subcortical (thalamic) automated seizure detection: A new option for contingent therapy delivery.

The feasibility of automated detection of cortical-onset epileptic seizures from subcortical structures such as the thalamus was investigated via simultaneous recording of electroencephalography (EEG) and anterior and centromedian thalamic nuclei electrical signals (electrothalamography) in nine subjects with pharmacoresistant seizures admitted to an epilepsy monitoring unit after deep brain stimulating electrode implantation. Thalamic electrical signals were analyzed using a validated seizure detection algorithm, and times of seizure onset and termination were compared to those determined through visual analysis of video-EEG. Ictal activity was recorded from the scalp and thalamic nuclei in three subjects who had seizures during the 3-4-day recording period. In the majority of seizures, ictal activity in the thalamic nuclei preceded electrographic onset as determined from the EEG or clinical onset as determined from behavioral observations. Interictal epileptiform discharges were also recorded from the thalamus and in certain instances had no scalp representation. Subcortical/thalamic detection of cortical-onset seizures is feasible. This approach would enable contingent therapy delivery and may be particularly valuable for subjects with multiple or difficult-to-localize epileptogenic regions.

Challenges for emerging neurostimulation-based therapies for real-time seizure control.

In step with the worthwhile aim of this special issue, two junior investigators impart their insights on the therapeutic challenges imposed by pharmacoresistant epilepsies and offer viable approaches to improvement of treatment outcomes. Sunderam's comprehensive perspective addresses issues of critical importance for the design of efficacious therapies. Talathi delves into the thorny roles of so-called "interictal" spikes in ictio- and epileptogenesis, roles that are central to understanding the dynamics of these phenomena and implicitly of how to prevent them or abort them. First, however, Osorio and co-workers illustrate the complex behavior of the epileptogenic network and point to the importance of real-time intraindividual adaptation and optimization of therapies for seizures originating from the same epileptogenic network.

Electrical stimulation of the anterior nucleus of thalamus for treatment of refractory epilepsy.

PURPOSE: We report a multicenter, double-blind, randomized trial of bilateral stimulation of the anterior nuclei of the thalamus for localization-related epilepsy. METHODS: Participants were adults with medically refractory partial seizures, including secondarily generalized seizures. Half received stimulation and half no stimulation during a 3-month blinded phase; then all received unblinded stimulation. RESULTS: One hundred ten participants were randomized. Baseline monthly median seizure frequency was 19.5. In the last month of the blinded phase the stimulated group had a 29% greater reduction in seizures compared with the control group, as estimated by a generalized estimating equations (GEE) model (p = 0.002). Unadjusted median declines at the end of the blinded phase were 14.5% in the control group and 40.4% in the stimulated group. Complex partial and "most severe" seizures were significantly reduced by stimulation. By 2 years, there was a 56% median percent reduction in seizure frequency; 54% of patients had a seizure reduction of at least 50%, and 14 patients were seizure-free for at least 6 months. Five deaths occurred and none were from implantation or stimulation. No participant had symptomatic hemorrhage or brain infection. Two participants had acute, transient stimulation-associated seizures. Cognition and mood showed no group differences, but participants in the stimulated group were more likely to report depression or memory problems as adverse events. DISCUSSION: Bilateral stimulation of the anterior nuclei of the thalamus reduces seizures. Benefit persisted for 2 years of study. Complication rates were modest. Deep brain stimulation of the anterior thalamus is useful for some people with medically refractory partial and secondarily generalized seizures.

Seizure abatement with single dc pulses: is phase resetting at play?

Topological approaches for seizure abatement have received scarce attention. The ability to reset the phase of biological oscillations has been widely exploited in cardiology, as evidenced in part by the usefulness of implantable of defibrillators, but not in epileptology. The aim of this work is to investigate the feasibility of seizure blockage using single or brief monophasic (DC) pulse trains. Single DC or brief (0.1 s) pulse trains were delivered manually or automatically to generalized seizures, induced in rats with the convulsant 3-mercaptoprionic acid, a GABA inhibitor. Treatment outcome (blocked vs. not blocked seizures) was ascertained visually and correlated with the "rhythmicity index", an indirect estimate of neuronal synchrony level. Blockage using single or brief (0.1 s) DC pulses was consistently achieved for seizures with a rhythmicity index > 0.6, while seizures with levels <0.6 were not, although transient phase changes in their oscillations were effected. This work reveals that level of neuronal synchronization may be an important factor in determining the probability of seizure blockage. Seizure blockage using single or brief DC pulse trains and its effects on neural tissue merit further investigation. The clinical applicability of this therapeutic modality and means to enhance it are discussed.