Serial Assessment of Gray Matter Abnormalities after Sport-Related Concussion.

There is an urgent need to characterize the acute physiological effects of sport-related concussion (SRC). We investigated the effects of SRC on gray matter structure and diffusion metrics in collegiate athletes at 1.64 (T1; n = 33), 8.33 (T2; n = 30), and 32.15 days (T3; n = 36) post-concussion, with healthy collegiate contact-sport athletes serving as controls (HA; n = 46). Plasma levels of glial fibrillary acidic protein (GFAP) were assessed in a subset of athletes. We hypothesized that acute SRC would be associated with increased fractional anisotropy (FA), decreased mean diffusivity (MD), and increased GFAP relative to noninjured HA, without acute differences in gray matter volume or cortical thickness. Further, we hypothesized that neither diffusion nor structure would show longitudinal changes across the first month post-SRC. Finally, we hypothesized that gray matter diffusion metrics would correlate with plasma GFAP levels, as indicated by pre-clinical literature. Consistent with our hypothesis, acute SRC was associated with decreased MD in the left pallidum, increased FA in the right amygdala, and a significantly greater number and volume of subject-specific clusters with increased FA compared to HA. No differences in gray matter volume, cortical thickness, or GFAP were observed between groups. There were no longitudinal changes in any measure across the first month post-SRC. Finally, FA in the right amygdala was inversely correlated with GFAP at T2. These results suggest that gray matter diffusion metrics may be useful in determining the physiological effects of SRC.

Evaluación de la intensidad de señal en núcleo dentado, puente, globus pallidus y tálamo en pacientes con esclerosis múltiple: valoración de la retención de gadolinio / Evaluation of signal intensity in dentate nucleus, pons, globus pallidus and thalamus in patients with multiple sclerosis: assessment of gadolinium retention

Introduction: Recently, some studies have reported accumulation of gadolinium in the brain of patients with multiple administrations of gadolinium-based contrast. Patients with multiple sclerosis are subjected to multiple contrasting resonances and could become a population at risk. Objective: To determine whether repeated intravenous exposure to gadolinium is associated with more intensity in the thalamus, dentate nucleus, pons and the globus pallidus. Methods: A retrospective study of 60 patients with MS who had undergone two or more contrasted MRs between 2007 and 2015, was performed. The ratios calculated were: dentate nuclei-to-pons (DNP), thalamus-to-pons (TP), caudate nuclei-to-pons (CNP), globus pallidus-to-thalamus (GPT), globus pallidus-to-pons (GPP), by reviewing simple T1 axial sequences. Relative changes were calculated and compared with the number of contrasted MRs. The concordance between observers and the intraclass correlation coefficient was evaluated. Results: There was no evidence of increased signal intensity in T1 sequences (DNP 0.524, GPT 0.466, GPP 0.684, TP 0.771, CNP 0.352). As there were no differences, the Spearman coefficient showed no correlation between relative changes and the number of resonances performed. Inter-observer agreement was almost perfect (0.982) for all structures. Conclusion: Our study did not find a statistically significant increase in the T1 signal intensity in patients with multiple sclerosis. However, there are factors to consider, such as the type of gadolinium and the time lapse between administrations.

Introducción: Recientemente, algunos estudios han informado acumulación de gadolinio en el cerebro de los pacientes con múltiples administraciones de contraste basado en gadolinio. Los pacientes con esclerosis múltiple son sometidos a múltiples resonancias contrastadas y podrían convertirse en una población de riesgo. Objetivo: Determinar si la exposición repetida por vía intravenosa a gadolino se asocia con mayor intensidad en el tálamo, el dentado, el puente y el globo pálido. Métodos: Se realizó un estudio retrospectivo núcleo de 60 pacientes con EM que habían sido sometidos a dos o más RM contrastadas entre 2007 - 2015. Se calcularon las razones núcleo dentado - puente (DNP), tálamo-puente (TP), núcleo caudado- puente (CNP), globus pallidus - tálamo (GPT), globus pallidos - puente (GPP), revisando secuencias T1 axiales simples. Se calcularon los cambios relativos y se compararon con el número de RM contrastadas. Se evaluó la concordancia entre observadores con el coeficiente de correlación intraclase. Resultados: No hubo evidencia de aumento de la intensidad de la señal en secuencias T1 (DNP 0,524, 0,446 GPT, GPP 0,684, 0,771 PT, CNP 0.352). Al no existir diferencias, el coeficiente de Spearman no mostró correlación entre los cambios relativos y el número de resonancias realizadas. La concordancia interobservador fue casi perfecta (0.982) para todas las estructuras. Conclusión: Nuestro estudio no encontró un aumento estadísticamente significativo en la intensidad de la señal T1 en pacientes con esclerosis múltiple. Sin embargo, hay factores a considerar, tales como el tipo de gadolinio y el lapso de tiempo entre las administraciones.

The role of MR imaging in assessment of brain damage from carbon monoxide poisoning: a review of the literature.

SUMMARY: The aim of this article is to review how MR imaging and associated imaging modalities provide clinicopathologic information on brain damage after carbon monoxide poisoning. Initially, many authors documented typical findings of conventional MR imaging in the gray matter structures such as the globus pallidus and in various regions of cerebral white matter. The focus of investigation has since shifted to observation of cerebral white matter areas that are more frequently detected on MR imaging and are more responsible for chronic symptoms than the gray matter. DWI has dramatically contributed to the ability to quantitatively assess cerebral white matter damage. Subsequently, DTI has enabled more sensitive evaluation than DWI and can demonstrate progressive pathologic changes in the early stage, allowing prediction of chronic conditions. In addition, MR spectroscopy reveals changes in metabolite levels, offering quantitative clinicopathologic information on brain damage after carbon monoxide poisoning.

Eye-of-the-tiger-sign in a 48 year healthy adult.

We report a healthy adult male, who underwent, as a control subject, part of a Huntington's disease study, extensive testing during three visits in a two year follow-up, including clinical examination and 3.0 T MRI scans. The T2-weighted MRI sequences revealed the "eye-of-the-tiger-sign". No clinical abnormalities in either motor, cognitive or behavioural domains were observed. PKAN2 and FTL gene mutation analysis were negative. This finding implies that an eye-of-the-tiger sign, which is considered a pathognomonic feature of neurodegeneration with brain iron accumulation (NBIA), can occur without any clinical symptoms.

Clinical neuroimaging and electrophysiological assessment of three DYT6 dystonia families.

The purpose of the study was to delineate clinical and electrophysiological characteristics as well as laryngoscopical and transcranial ultrasound (TCS) findings in THAP1 mutation carriers (MutC). According to recent genetic studies, DYT6 (THAP1) gene mutations are an important cause of primary early-onset dystonia. In contrast to DYT1 mutations, THAP1 mutations are associated with primary early-onset segmental or generalised dystonia frequently involving the craniocervical region and the larynx. Blood samples from twelve individuals of three German families with DYT6 positive index cases were obtained to test for THAP1 mutations. Eight THAP1 MutC were identified. Of these, six (three symptomatic and three asymptomatic) THAP1 MutC could be clinically evaluated. Laryngoscopy was performed to evaluate laryngeal dysfunction in patients. Brainstem echogenicity was investigated in all MutC using TCS. Two of the patients had undergone bilateral pallidal DBS. In all three symptomatic MutC, early-onset laryngeal dystonia was a prominent feature. Laryngeal assessment demonstrated adductor-type dystonia in all of them. On clinical examination, the three asymptomatic MutC also showed subtle signs of focal or segmental dystonia. TCS revealed increased substantia nigra (SN) hyperechogenicity in all MutC. Intraoperative microelectrode recordings under general anesthesia in two of the patients showed no difference between THAP1 and previously operated DYT1 MutC. The presence of spasmodic dysphonia in patients with young-onset segmental or generalised dystonia is a hallmark of DYT6 dystonia. SN hyperechogenicity on TCS may represent an endophenotype in these patients. Pallidal DBS in two patients was unsatisfactory.

Encephalopathy assessment in children with extra-hepatic portal vein obstruction with MR, psychometry and critical flicker frequency.

BACKGROUND & AIMS: Mild cognitive and psychomotor deficit has been reported in patients with extra-hepatic portal vein obstruction. This prospective study was done to ascertain the presence of minimal hepatic encephalopathy by neuropsychological testing and its correlation with diffusion tensor imaging derived metrics, T1 signal intensity, brain metabolites in (1)H magnetic resonance spectroscopy, blood ammonia and critical flicker frequency in patients with extra-hepatic portal vein obstruction. METHODS: Neuropsychological tests, critical flicker frequency, blood ammonia, diffusion tensor imaging, T1 signal intensity and (1)H magnetic resonance spectroscopy were determined in 22 extra-hepatic portal vein obstruction and 17 healthy children. Bonferroni multiple comparison post hoc analysis was done to compare controls with patient groups. RESULTS: Based on neuropsychological tests, 7/22 patients had minimal hepatic encephalopathy, and significantly increased Glx/Cr ratio, blood ammonia, mean diffusivity and globus pallidus T1 signal intensity with decreased critical flicker frequency in comparison to controls and in those without minimal hepatic encephalopathy. Cho/Cr, mI/Cr ratio and fractional anisotropy were unchanged in patient groups compared to controls. A significant inverse correlation of neuropsychological test with mean diffusivity, Glx/Cr ratio and blood ammonia and a positive correlation among mean diffusivity, blood ammonia and Glx/Cr ratio was seen. CONCLUSIONS: Extra-hepatic portal vein obstruction is a true hyperammonia model with porto-systemic shunting and normal liver functions that results in minimal hepatic encephalopathy in one-third of these children. Hyperammonia results in generalized low grade cerebral edema and cognitive decline as evidenced by increased Glx/Cr ratio, mean diffusivity values and abnormal neuropsychological tests.

Constantin von Economo's contribution to the understanding of movement disorders.

Constantin von Economo's (CvE) main scientific achievements were his studies on the cytoarchitectonics of the cerebral cortex, sleep, and encephalitis lethargica (EL). He found a close relationship between motor symptoms and psychiatric and behavioral disorders in EL and postencephalitic Parkinsonism and identified the underlying neuropathology in the diencephalon and the brainstem. In agreement with Tretiakoff's findings in Parkinson's disease, CvE related postencephalitic Parkinsonism to neuronal loss in the substantia nigra. Several of CvE's early, less well-known publications also deal with the basal ganglia and movement disorders. He demonstrated in rabbits that the substantia nigra modulates automatization, coordination, and succession of masticatory movements and swallowing. In a study on the effects of experimental lesions of the cerebral peduncle in cats and monkeys, CvE hypothesized a corticotegmental pathway that maintains motor functions after pyramidal tract lesions. Recent studies have identified this pathway, which ends in the pedunculopontine nucleus. In a study on posthemiplegic chorea, CvE discussed various pathophysiological hypotheses that partly resemble modern concepts of chorea. In a clinicopathological study on Wilson's disease, CvE traced the striofugal fibers and visualized the basal ganglia outflow pathways. CvE was an outstanding multidisciplinary movement disorder specialist who contributed substantially to modern basal ganglia research.

Evaluation of MR signal index for the assessment of occupational manganese exposure of welders by measurement of local proton T1 relaxation time.

Magnetic resonance (MR) imaging has become a useful tool for the estimation of occupational exposure to manganese (Mn). However, validity of signal indices on T1-weighted imaging, a proxy measurement of T1 relaxation time shortening due to Mn deposition, needs to be evaluated in an occupationally exposed population. We performed MR imaging on 20 male welders and 10 age- and gender-matched, non-office workers in a shipyard. We measured the relative signal intensity ratio of the globus pallidus (GP) to the frontal white matter on T1-weighted MR images (pallidal index, PI). We also assessed the visual scale for the relative signal intensity of the GP, which was graded as 0-2 by a radiologist. We evaluated both signal index and visual scale in relation to the T1 relaxation time measured at the GP. Both PI and T1 relaxation time were significantly increased in the welders compared to the non-welders. T1 relaxation time began to decrease from visual scale 2, while PI began to increase from visual scale 1. Environmental Mn exposure indices correlated well with both T1 relaxation time and PI. However, blood Mn level correlated only with T1 relaxation time, and not with PI. T1 relaxation time correlated with PI only at the higher level of PI. These results indicate the possible inaccuracy of visual scoring and the discrepancy between T1 relaxation time and PI, especially at the lower level of signal intensity. Although both T1 and PI are good biomarkers for the environmental Mn exposure, non-linearity of the relationship between T1 and PI must be considered in the assessment of Mn exposure using MRI.

Firing patterns of pallidal cells in parkinsonian patients correlate with their pre-pallidotomy clinical scores.

It is unclear how the disordered activity of cells in the basal ganglia contributes to the symptoms of Parkinson's disease (PD). We recorded from single neurons extracellularly in 3 regions of the globus pallidus (GPe, GPie and GPii) in patients undergoing pallidotomy for PD. Movement-related cell firing patterns, analysed using hidden Markov models, were significantly correlated with patients' preoperative clinical scores (off drugs). Responses of cells in GPii correlated best with the scores for specific motor tasks, rather than general ones related to activities of daily living, but the reverse was true for responses from GPe. In both GPii and GPe, a higher score (i.e. greater parkinsonian severity) was associated with greater variability in cell firing rather than an increase in firing rate itself.