Case report: Neuronal intranuclear inclusion disease presenting with acute encephalopathy

Neuronal intranuclear inclusion disease (NIID), a neurodegenerative disease previously thought to be rare, is increasingly recognized despite heterogeneous clinical presentations. NIID is pathologically characterized by ubiquitin and p-62 positive intranuclear eosinophilic inclusions that affect multiple organ systems, including the brain, skin, and other tissues. Although the diagnosis of NIID is challenging due to phenotypic heterogeneity, a greater understanding of the clinical and imaging presentations can improve accurate and early diagnosis. Here, we present three cases of pathologically proven adult-onset NIID, all presenting with episodes of acute encephalopathy with protracted workups and lengthy time between symptom onset and diagnosis. Case 1 highlights challenges in the diagnosis of NIID when MRI does not reveal classic abnormalities and provides a striking example of hyperperfusion in the setting of acute encephalopathy, as well as unique pathology with neuronal central chromatolysis, which has not been previously described. Case 2 highlights the progression of MRI findings associated with multiple NIID-related encephalopathic episodes over an extended time period, as well as the utility of skin biopsy for antemortem diagnosis

Amygdala sign, a FDG-PET signature of dementia with Lewy Bodies

BackgroundBiomarkers are being used increasingly to support the diagnosis of dementia with Lewy bodies (DLB). Novel biomarkers that increase diagnostic specificity of DLB are needed. We assessed previously known FDG-PET occipital cortex hypometabolism, and cingulate island sign biomarkers of DLB against a novel amygdala signature.MethodsRetrospective analysis of 49 patients evaluated at one tertiary memory clinic. All had a FDG-PET brain scan performed as part of their diagnostic work up evaluating three common neurodegenerative etiologies: Alzheimer dementia (AD), Frontotemporal dementia (FTD) and DLB. A consensus diagnosis of dementia was made based on accepted clinical criteria for AD, FTD and DLB. FDG-PET regional metabolism was delineated by automatic segmentation as well as manual tracing of amygdala and posterior cingulate volumes of interest. Mean normalized values calculated for regional FDG-PET signatures of DLB: occipital cortex hypometabolism and preservation of posterior cingulate and amygdala metabolism relative to whole brain metabolism were evaluated.ResultsSignificant overlap between DLB and AD patients (occipital, parietal, temporal and frontal hypometabolism) and between DLB and FTD (frontal hypometabolism and the posterior cingulate sign) were identified. Right amygdala (p = 0.028) and right posterior cingulate (p = 0.035) mean normalized regional metabolism levels were preserved in DLB compared to AD. Among subjects at less advanced stages of dementia (MoCA>10), relative preservation of regional metabolism was notable across both left (p = 0.006) and right (p = 0.020) amygdala.ConclusionRelative preservation of amygdala metabolism could complement previously described FDG-PET findings in earlier stages of DLB

Performances of III-P/Si based photoelectrodes for solar hydrogen production

National audienc

MBE-grown GaAs on a low-cost Si substrate for solar hydrogen production

International audienc

High stability of a MBE grown GaAs/Si photocathode for solar H2 production.

International audienc

Performance of epitaxial GaAs/Si vs GaAs photocathodes for solar hydrogen production.

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Photoelectrode/Electrolyte interfacial band lineup engineering with alloyed III-V thin films grown on Si substrate.

International audienceIn this work, we demonstrate how the classical concept of band gap engineering usually used in III–V semiconductor devices can be extended to the engineering of the band lineup between semiconducting photoelectrodes and electrolytes. The performances of photoelectrodes made of GaP1−xAsx epilayers in the full compositional range and grown on low-cost Si substrates were studied and compared with those of photoelectrodes grown on GaAs and GaP substrates. We first show that the changes of incident photon to current conversion efficiency (IPCE) with the As content in GaP1−xAsx alloys are related to the band gap nature (direct or indirect) and band gap energy variations. Then, from flat band potential measurements during Mott–Schottky experiments, valence and conduction band energies of GaP1−xAsx alloys are positioned versus the reversible hydrogen potential. A weak change of conduction band energies and a large evolution of valence band energies are obtained, in good agreement with expected theoretical trends. Such results show that both band gaps and semiconductor/electrolyte band lineups can be engineered through alloying of III–V semiconductors deposited on silicon substrates. This band lineup engineering strategy is expected to be of great interest to address specific redox reactions in the electrolyte, provided that suitable protecting or passivating layers can be used to limit surface/interface recombinations

Anticholinergic Drug Burden and Neurocognitive Performance in the Study of Latinos-Investigation of Neurocognitive Aging.

BackgroundStudies of cumulative anticholinergic drug burden on cognitive function and impairment are emerging, yet few for Hispanics/Latinos.ObjectiveTo examine associations between anticholinergic use and neurocognitive performance outcomes among diverse Hispanics/Latinos.MethodsThis prospective cohort study included diverse Hispanic/Latino participants, enrolled in the Study of Latinos-Investigation of Neurocognitive, from New York, Chicago, Miami, and San Diego (n = 6,249). Survey linear regression examined associations between anticholinergic use (measured during baseline [Visit 1] and average 7-year follow up [Visit 2]) with global cognition, episodic learning, memory, phonemic fluency, processing speed, executive functioning, and average 7-year change.ResultsAnticholinergic use was associated with lower cognitive global cognition (β= -0.21; 95% CI [-0.36; -0.05]), learning (β= -0.27; 95% CI [-0.47; -0.07]), memory (β= -0.22; 95% CI [-0.41; -0.03]), and executive functioning (β= -0.22; 95% CI [-0.40; -0.03]) scores, particularly among those who took anticholinergics at both visits. Anticholinergic use was associated with faster decline in global cognition, learning, and verbal fluency (β: -0.28 [95% CI: -0.55, -0.01]; β: -0.28 [95% CI: -0.55, -0.01]; β: -0.25, [95% CI -0.47, -0.04], respectively). Sex modified associations between anticholinergic use with global cognition, learning, and executive functioning (F3 = 3.59, F3 = 2.84, F3 = 3.88, respectively).ConclusionAnticholinergic use was associated with lower neurocognitive performance, especially among those who used anticholinergics at both visits, among a study population of diverse Hispanics/Latinos. Findings will support evidence-based decisions regarding anticholinergic prescriptions and efforts to minimize cognitive impact