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Cerebellar pathology in motor neuron disease:neuroplasticity and neurodegeneration

Neural Regeneration Research 2022年第11期17卷 2335-2341页

作者： Rangariroyashe H.Chipika Grainne Mulkerrin pierre-françois pradat Aizuri Murad Fabrice Ango Cédric Raoul Peter Bede Computational Neuroimaging Group Biomedical Sciences InstituteTrinity College DublinDublinIreland Pitié-Salpêtrière University Hospital Sorbonne UniversityParisFrance The Neuroscience Institute of Montpellier(INM) INSERMCNRSMontpellierFrance

Amyotrophic lateral sclerosis is a relentlessly progressive multi-system *** clinical picture is dominated by upper and lower motor neuron degeneration,but extra-motor pathology is increasingly recognized,including cerebellar ***-mortem and neuroimaging studies primarily focus on the chara cterization of supratentorial disease,des pite emerging evidence of cerebellar degeneration in amyotrophic lateral *** clinical features of amyotrophic lateral sclerosis,such as dysarthria,dysphagia,cognitive and behavioral deficits,saccade abnormalities,gait impairment,respiratory weakness and pseudobulbar affect are likely to be exacerbated by co-existing cerebellar *** review summarizes in vivo and post mortem evidence for cerebellar degeneration in amyotrophic lateral scle *** imaging studies consistently capture cerebellar grey matter volume reductions,diffusivity studies readily detect both intra-cerebellar and cerebellar peduncle white matter alte rations and functional imaging studies commonly report increased functional connectivity with supratentorial *** functional connectivity is commonly interpreted as evidence of neuro plasticity representing compensatory processes despite the lack of post-mortem *** is a scarcity of post-mortem studies focusing on cerebellar alte rations,but these detect pTDP-43 in cerebellar *** rebellar pathology is an overloo ked facet of neurodegeneration in amyotrophic lateral sclerosis despite its contribution to a multitude of clinical symptoms,wides p read connectivity to spinal and supratentorial regions and putative role in compensating for the degeneration of primary motor regions.

关键词： amyotrophic lateral sclerosis ataxia cerebellum magnetic resonance imaging motor neuron disease neuroimaging neuroplasticity pathology primary lateral sclerosis pseudobulbar affect

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Neuroflament light and heterogeneity of disease progression in amyotrophic lateral sclerosis:development and validation of a prediction model to improve interventional trials

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Translational Neurodegeneration 2021年第3期10卷 400-411页

作者： Simon Witzel Felix Frauhammer† Petra Steinacker David Devos pierre‑françois pradat Vincent Meininger Stefen Halbgebauer Patrick Oeckl Joachim Schuster Simon Anders Johannes Dorst Markus Otto Albert C.Ludolph Department of Neurology University of UlmUlmGermany Center for Molecular Biology Heidelberg UniversityHeidelbergGermany Depart‑ment of Medical Pharmacology Expert center for ParkinsonCHU‑LilleLille Neuroscience and CognitionInsermUMR‑S1172LICENDNS‑Park NetworkUniversity of LilleLilleFrance Laboratoire D’Imagerie Biomédicale Sorbonne UniversitéCNRSINSERMParisFrance APHP Département de NeurologieHôpital Pitié-SalpêtrièreParisFrance German Centre for Neurodegenerative Diseases(DZNE)Site Ulm UlmGermany

Background:Interventional trials in amyotrophic lateral sclerosis(ALS)sufer from the heterogeneity of the disease as it considerably reduces statistical *** asked if blood neuroflament light chains(NfL)could be used to antici‑pate disease progression and increase trial ***:In 125 patients with ALS from three independent prospective studies-one observational study and two interventional trials-we developed and externally validated a multivariate linear model for predicting disease pro‑gression,measured by the monthly decrease of the ALS Functional Rating Scale Revised(ALSFRS-R)*** trained the prediction model in the observational study and tested the predictive value of the following parameters assessed at diagnosis:NfL levels,sex,age,site of onset,body mass index,disease duration,ALSFRS-R score,and monthly ALSFRS-R score decrease since disease *** then applied the resulting model in the other two study cohorts to assess the actual utility for interventional *** analyzed the impact on trial power in mixed-efects models and compared the performance of the NfL model with two currently used predictive approaches,which anticipate disease progression using the ALSFRS-R decrease during a three-month observational period(lead-in)or since disease onset(ΔFRS).Results:Among the parameters provided,the NfL levels(P<0.001)and the interaction with site of onset(P<0.01)contributed signifcantly to the prediction,forming a robust NfL prediction model(R=0.67).Model application in the trial cohorts confrmed its applicability and revealed superiority over lead-in andΔFRS-based *** NfL model improved statistical power by 61%and 22%(95%confdence intervals:54%-66%,7%-29%).Conclusion:The use of the NfL-based prediction model to compensate for clinical heterogeneity in ALS could signif‑cantly increase the trial ***00868166,registered March23,2009;NCT02306590,registered December 2,2014.

关键词： Neuroflament light Prediction model Disease progression Amyotrophic lateral sclerosis Interventional trials Statistical power

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Non-pharmacological intervention for posterior cortical atrophy

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World Journal of Clinical Cases 2016年第8期4卷 195-201页

作者： Agnès Weill--Chounlamountry Jorge Alves Pascale pradat--Diehl Département de Médecine Physique et de Réadaptation AP-HP H?pitaux Pitié-Salpêtrière Charles Foix GRC-UPMC Handicap Cognitif et Réadaptation Center for Evidence-Based Neuro Rehabilitation CEREBRO-Brain Health Center Laboratoire d’Imagerie Biomédicale (LIB) Inserm U1146 Sorbonne Universités UPMC UMCR2-CNRS UMR7371

Posterior cortical atrophy(PCA) is a rare neurodegene-rative condition characterized by progressive visual-perceptual deficits. Although the neurocognitive profile of PCA is a growing and relatively well-established field, non-pharmacological care remains understudied and to be widely established in clinical practice. In the present work we review the available literature on non-pharmacological approaches for PCA, such as cognitive rehabilitation including individual cognitive exercises and compensatory techniques to improve autonomy in daily life, and psycho-education aiming to inform people with PCA about the nature of their visual deficits and limits of cognitive rehabilitation. The reviewed studies represented a total of 7 patients. There is a scarcity of the number of studies, and mostly consisting of case studies. Results suggest non-pharmacological intervention to be a potentially beneficial approach for the partial compensation of deficits, improvement of daily functionality and improvement of quality of life. Clinical implications and future directions are also highlighted for the advancement of the field, in order to clarify the possible role of non-pharmacological interventions, and its extent, in PCA.

关键词： Cognitive rehabilitation Posterior cortical atrophy Alzheimer’s disease Non-pharmacological intervention Neuropsychological rehabilitation

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金刚烷胺三联疗法治疗无应答型丙型肝炎患者

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世界核心医学期刊文摘（胃肠病学分册） 2006年第8期2卷 53-54页

作者： Maynard M. pradat P. Bailly F. C. Trépo 孟欣颖(译) 王顺涛(校) 不详 Department of Hepato-gastroenterology HotelDieu 1 place de I'Hopital 69288 Lyon Cedex 02 France

Background/Aims: To determine whether addition of amantadine to pegylated interferon/ribavirin improved response rates among chronic hepatitis C patients, non-responders to interferon/ ribavirin and study the dynamic of response. Methods: In a double blind, multicenter, randomized trial, 200 non-responder patients received pegylated interferon 1.5 μ g/kg per week and ribavirin 800- 1200 mg/day, plus either amantadine 200 mg/day or placebo for 48 weeks. Endpoints were virological responses, ALT normalization, and histological benefit overtime. Results: Twenty percent of all patients achieved a sustained virological response (SVR). This rate was 8% higher in the triple therapy group (24% ) compared with the double therapy group (16% ) (P=0.22). A better virological response rate at week 24 was observed in the triple regimen group (43 vs 29% ; P=0.06), which was lost at week 48 suggesting viral escape. The biochemical response rate was also significantly higher with triple therapy at week 12 (63 vs 49% ; P=0.05) and week 24 (64 vs 49% ; P=0.03). Fibrosis stabilized or improved in 77% of all patients. Conclusions: Re-treatment of interferon/ ribavirin non-responder patients should be encouraged since a substantial proportion benefits from retreat-ment with pegylated interferon/ribavirin ± amantadine. In triple therapy involving amantadine, a time wise response and an increased SVR rate in subgroups less prone to viral breakthrough suggest clues for existing controversies.

关键词：丙型肝炎患者安慰剂治疗三联疗法金刚烷胺无应答聚乙二醇干扰素持续病毒学应答利巴韦林动力学特征随机试验

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