Slurred, Blurred, and a Hard-to-Find Word: Acute Progressive Neurologic Changes in a Pediatric Patient

Background: Acute disseminated encephalomyelitis (ADEM) is typically a viral sequelae, with a 1-3% mortality rate. ADEM has been described as a consequence of acute SARS-CoV-2 infection. This case report describes a child diagnosed with ADEM in the setting of acute SARS-CoV-2 infection with the clinical course complicated by co-infection with Human herpesvirus 6 (HHV-6). HHV-6 positivity in the CSF is often considered an incidental finding, but it has been shown to cause encephalopathy, seizures, and demyelinating disease. This case is unique in that the dual infection with SARS-CoV-2 and HHV-6 may have contributed to the development of ADEM, poor clinical outcome, and resistance to treatment in this case. Case Presentation: A 9 y.o. male with ADHD presented with 12 hours of acute onset left-sided weakness, blurred vision with left-sided vision loss, expressive aphasia, and ataxia. Head CT was negative at an outside ED prior to transfer. On exam, he was responsive to commands and agitated with waxing and waning consciousness. Exam significant for decreased left peripheral and blurry vision, expressive aphasia, slurred speech, and decreased left grip strength, although full strength testing was limited. Patellar reflexes 3+ but symmetric, Babinski down, and no clonus. Evaluation was pertinent for positive respiratory panel for SARS-CoV-2. CSF was without pleocytosis, elevated protein, and positive for HHV6. Serum studies positive for GAD65 Ab. MRI brain and spine with T2 hyperintensities showing extensive punctate white matter lesions throughout the cerebral hemispheres, midbrain, and cerebellum, enhancing signal abnormality throughout the spinal cord, and dominant lesions in the frontal lobes, to be correlated with vasculitis or demyelinating disorders. Normal MRA/MRV head. The patient received high dose IV steroids followed by IVIG due to lack of response. He was started on IV Ganciclovir, Remdesivir, Cefepime, and Vancomycin. He developed status epilepticus (SE) resistant to multiple anti-epileptic drugs and diabetes insipidus (DI), progressing to cerebral edema and herniation, neurogenic shock, and brain death. Discussion: The combination of encephalopathy, recent viral illness, acute neurological changes, and multi-white matter lesions in the brain and spine supported a diagnosis of ADEM due to SARS-CoV-2 versus HHV- 6. One study reported that seizures occurred in 11% of children with ADEM. SE is a serious complication of ADEM and likely contributed to the development of cerebral edema, resulting DI, and the patient’s poor outcome. It is uncertain at this time if co-viral illnesses played a role in this patient’s rapid decline.https://digitalcommons.unmc.edu/chri_forum/1061/thumbnail.jp

Prominent effects and neural correlates of visual crowding in a neurodegenerative disease population.

Crowding is a breakdown in the ability to identify objects in clutter, and is a major constraint on object recognition. Crowding particularly impairs object perception in peripheral, amblyopic and possibly developing vision. Here we argue that crowding is also a critical factor limiting object perception in central vision of individuals with neurodegeneration of the occipital cortices. In the current study, individuals with posterior cortical atrophy (n=26), typical Alzheimer's disease (n=17) and healthy control subjects (n=14) completed centrally-presented tests of letter identification under six different flanking conditions (unflanked, and with letter, shape, number, same polarity and reverse polarity flankers) with two different target-flanker spacings (condensed, spaced). Patients with posterior cortical atrophy were significantly less accurate and slower to identify targets in the condensed than spaced condition even when the target letters were surrounded by flankers of a different category. Importantly, this spacing effect was observed for same, but not reverse, polarity flankers. The difference in accuracy between spaced and condensed stimuli was significantly associated with lower grey matter volume in the right collateral sulcus, in a region lying between the fusiform and lingual gyri. Detailed error analysis also revealed that similarity between the error response and the averaged target and flanker stimuli (but not individual target or flanker stimuli) was a significant predictor of error rate, more consistent with averaging than substitution accounts of crowding. Our findings suggest that crowding in posterior cortical atrophy can be regarded as a pre-attentive process that uses averaging to regularize the pathologically noisy representation of letter feature position in central vision. These results also help to clarify the cortical localization of feature integration components of crowding. More broadly, we suggest that posterior cortical atrophy provides a neurodegenerative disease model for exploring the basis of crowding. These data have significant implications for patients with, or who will go on to develop, dementia-related visual impairment, in whom acquired excessive crowding likely contributes to deficits in word, object, face and scene perception

Processing emotion from abstract art in frontotemporal lobar degeneration

Abstract art may signal emotions independently of a biological or social carrier: it might therefore constitute a test case for defining brain mechanisms of generic emotion decoding and the impact of disease states on those mechanisms. This is potentially of particular relevance to diseases in the frontotemporal lobar degeneration (FTLD) spectrum. These diseases are often led by emotional impairment despite retained or enhanced artistic interest in at least some patients. However, the processing of emotion from art has not been studied systematically in FTLD. Here we addressed this issue using a novel emotional valence matching task on abstract paintings in patients representing major syndromes of FTLD (behavioural variant frontotemporal dementia, n=11; sematic variant primary progressive aphasia (svPPA), n=7; nonfluent variant primary progressive aphasia (nfvPPA), n=6) relative to healthy older individuals (n=39). Performance on art emotion valence matching was compared between groups taking account of perceptual matching performance and assessed in relation to facial emotion matching using customised control tasks. Neuroanatomical correlates of art emotion processing were assessed using voxel-based morphometry of patients' brain MR images. All patient groups had a deficit of art emotion processing relative to healthy controls; there were no significant interactions between syndromic group and emotion modality. Poorer art emotion valence matching performance was associated with reduced grey matter volume in right lateral occopitotemporal cortex in proximity to regions previously implicated in the processing of dynamic visual signals. Our findings suggest that abstract art may be a useful model system for investigating mechanisms of generic emotion decoding and aesthetic processing in neurodegenerative diseases

Last Men Standing: Chlamydatus Portraits and Public Life in Late Antique Corinth

Notable among the marble sculptures excavated at Corinth are seven portraits of men wearing the long chlamys of Late Antique imperial office. This unusual costume, contemporary portrait heads, and inscribed statue bases all help confirm that new public statuary was created and erected at Corinth during the 4th and 5th centuries. These chlamydatus portraits, published together here for the first time, are likely to represent the Governor of Achaia in his capital city, in the company of local benefactors. Among the last works of the ancient sculptural tradition, they form a valuable source of information on public life in Late Antique Corinth

Planet Formation Imager (PFI): science vision and key requirements

The Planet Formation Imager (PFI) project aims to provide a strong scientific vision for ground-based optical astronomy beyond the upcoming generation of Extremely Large Telescopes. We make the case that a breakthrough in angular resolution imaging capabilities is required in order to unravel the processes involved in planet formation. PFI will be optimised to provide a complete census of the protoplanet population at all stellocentric radii and over the age range from 0.1 to ~100 Myr. Within this age period, planetary systems undergo dramatic changes and the final architecture of planetary systems is determined. Our goal is to study the planetary birth on the natural spatial scale where the material is assembled, which is the "Hill Sphere" of the forming planet, and to characterise the protoplanetary cores by measuring their masses and physical properties. Our science working group has investigated the observational characteristics of these young protoplanets as well as the migration mechanisms that might alter the system architecture. We simulated the imprints that the planets leave in the disk and study how PFI could revolutionise areas ranging from exoplanet to extragalactic science. In this contribution we outline the key science drivers of PFI and discuss the requirements that will guide the technology choices, the site selection, and potential science/technology tradeoffs.S.K. acknowledges support from an STFC Rutherford Fellowship (ST/J004030/1) and Philip Leverhulme Prize (PLP-2013-110). Part of this work was carried out at the Jet Propulsion Laboratory, California Institute of Technology, under a contract with the National Aeronautics and Space Administration

Studying the birth of exoplanetary systems with the Planet Formation Imager (PFI)

International audienceDespite recent advancements, many fundamental questions still surround the processes that are involved in planetary birth: Where in the protoplanetary disk do the planets form and how do they grow? What factors determine the final architecture of planetary systems? How are water and other volatiles delivered to the protoplanets and how does this affect the potential habitability of these worlds?As part of the "Planet Formation Imager" (PFI) project we develop the roadmap for a future infrared high-angular resolution imaging facility that aims to answer these questions by witnessing the planetary formation processes on the natural scales where the material is assembled, which is the Hill sphere of the forming planets. PFI will detect giant protoplanets on all stellocentric radii, image their interaction with the ambient disk material, and trace their dynamical evolution during the first 100 million years, thereby reveal the processes that determine the architecture of planetary systems.In this contribution we give an overview about the work of the PFI science and technical working group and present radiation-hydrodynamics simulations from which we derive preliminary specifications that guide the design of the facility. We will present a baseline PFI architecture that can achieve these goals, point at remaining technical challenges, and suggest activities today that will help make the Planet Formation Imager facility a reality