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  1. Article: Editorial: Advances in Therapeutics for Hyperkinetic Movement Disorders.

    Klopstock, Thomas / Hall, Deborah / Frucht, Steven / Flamand-Roze, Emmanuel

    Frontiers in neurology

    2022  Volume 13, Page(s) 853084

    Language English
    Publishing date 2022-02-24
    Publishing country Switzerland
    Document type Editorial
    ZDB-ID 2564214-5
    ISSN 1664-2295
    ISSN 1664-2295
    DOI 10.3389/fneur.2022.853084
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Autoxidation Kinetics of Tetrahydrobiopterin-Giving Quinonoid Dihydrobiopterin the Consideration It Deserves.

    Boulghobra, Ayoub / Bonose, Myriam / Alhajji, Eskandar / Pallandre, Antoine / Flamand-Roze, Emmanuel / Baudin, Bruno / Menet, Marie-Claude / Moussa, Fathi

    Molecules (Basel, Switzerland)

    2023  Volume 28, Issue 3

    Abstract: In humans, tetrahydrobiopterin (H4Bip) is the cofactor of several essential hydroxylation reactions which dysfunction cause very serious diseases at any age. Hence, the determination of pterins in biological media is of outmost importance in the ... ...

    Abstract In humans, tetrahydrobiopterin (H4Bip) is the cofactor of several essential hydroxylation reactions which dysfunction cause very serious diseases at any age. Hence, the determination of pterins in biological media is of outmost importance in the diagnosis and monitoring of H4Bip deficiency. More than half a century after the discovery of the physiological role of H4Bip and the recent advent of gene therapy for dopamine and serotonin disorders linked to H4Bip deficiency, the quantification of quinonoid dihydrobiopterin (qH2Bip), the transient intermediate of H4Bip, has not been considered yet. This is mainly due to its short half-life, which goes from 0.9 to 5 min according to previous studies. Based on our recent disclosure of the specific MS/MS transition of qH2Bip, here, we developed an efficient HPLC-MS/MS method to achieve the separation of qH2Bip from H4Bip and other oxidation products in less than 3.5 min. The application of this method to the investigation of H4Bip autoxidation kinetics clearly shows that qH2Bip's half-life is much longer than previously reported, and mostly longer than that of H4Bip, irrespective of the considered experimental conditions. These findings definitely confirm that an accurate method of H4Bip analysis should include the quantification of qH2Bip.
    MeSH term(s) Humans ; Tandem Mass Spectrometry ; Biopterins/analysis ; Biopterins/metabolism ; Pterins ; Kinetics
    Chemical Substances sapropterin (EGX657432I) ; 7,8-dihydrobiopterin (6779-87-9) ; Biopterins ; Pterins
    Language English
    Publishing date 2023-01-28
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 1413402-0
    ISSN 1420-3049 ; 1431-5165 ; 1420-3049
    ISSN (online) 1420-3049
    ISSN 1431-5165 ; 1420-3049
    DOI 10.3390/molecules28031267
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.MO 81: Show issues

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  3. Article ; Online: Lessons from multitarget neurostimulation in isolated dystonia: Less is more?

    Cuartero, Marie-Charlotte / Grabli, David / Flamand-Roze, Emmanuel / Karachi, Carine / Rouaud, Tiphaine / Derkinderen, Pascal / Damier, Philippe / Raoul, Sylvie / Krack, Paul / Moro, Elena / Fraix, Valérie / Chabardès, Stéphan / Burbaud, Pierre / Guehl, Dominique / Cuny, Emmanuel / Pinto, Serge / Vidailhet, Marie

    Brain stimulation

    2024  Volume 17, Issue 1, Page(s) 104–106

    MeSH term(s) Humans ; Dystonia/therapy ; Dystonic Disorders/therapy ; Treatment Outcome ; Deep Brain Stimulation
    Language English
    Publishing date 2024-01-03
    Publishing country United States
    Document type Letter ; Research Support, Non-U.S. Gov't
    ZDB-ID 2394410-9
    ISSN 1876-4754 ; 1935-861X
    ISSN (online) 1876-4754
    ISSN 1935-861X
    DOI 10.1016/j.brs.2023.12.012
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 6740: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  4. Article: Les aidants familiaux de jeunes patients atteints de maladies neurologiques chroniques lors de la transition entre secteur pédiatrique et prise en charge en milieu adulte : étude observationnelle de l?aidant familial principal.

    Manzato Sadki, Elodie / Grabli, David / Flamand Roze, Emmanuel / Baulac, Michel / Bourmaleau, Julie / Lefebvre, Sandrine

    Recherche en soins infirmiers

    2018  , Issue 133, Page(s) 93–98

    Abstract: Introduction / Context: The transition of young patients from pediatric to adult departments is a critical period with high risks of interruption of the care circuits, thus justifying the implementation of transition programs. This period is also ... ...

    Title translation Family caregivers of young patients suffering from chronic neurological diseases during the transition from neuropediatrics to adult neurology: An observational study of the burden felt by the primary family caregiver.
    Abstract Introduction / Context: The transition of young patients from pediatric to adult departments is a critical period with high risks of interruption of the care circuits, thus justifying the implementation of transition programs. This period is also difficult for caregivers, more particularly the main family caregiver. This study addresses the impact of this transition upon the family caregivers of young adults suffering from chronic neurological diseases.
    Objectives: To identify the main family caregivers, their profile, and to evaluate their implication and feelings in terms of burden at the time of the transition.
    Methods: A questionnaire, which included a modified version of the Zarit Burden Interview, was sent to the families of young patients who had recently moved to the adult neurology department.
    Results: Twenty-nine of the forty families contacted replied: the main caregiver is usually the mother (86.6%), the mean age is 51.8, 65% had kept their professional occupation, and 21% had quit. The burden scale showed that 65.5% felt little or no burden.
    Discussion and conclusion: This limited feeling of burden may be explained by the fact that the majority of patients did not have a motor/intellectual disability. The burden scale we used was originally created for caregivers of elderly patients (often their children), and may not be suitable for assessing children's parents. More specific scales should be considered.
    MeSH term(s) Caregivers/psychology ; Caregivers/statistics & numerical data ; Chronic Disease ; Emotions ; Female ; Humans ; Male ; Nervous System Diseases/therapy ; Neurology ; Pediatrics ; Surveys and Questionnaires ; Transition to Adult Care ; Young Adult
    Language French
    Publishing date 2018-07-31
    Publishing country France
    Document type Journal Article ; Observational Study
    ISSN 0297-2964
    ISSN 0297-2964
    DOI 10.3917/rsi.133.0093
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Intraputaminal Gene Delivery in Two Patients with Aromatic L-Amino Acid Decarboxylase Deficiency.

    François-Heude, Marie-Céline / Poulen, Gaetan / Flamand Roze, Emmanuel / Nguyen Morel, Marie-Ange / Gras, Domitille / Roch-Toreilles, Isabelle / Quintard, Adeline / Baroux, Gaelle / Meyer, Pierre / Coubes, Philippe / Milesi, Christophe / Cambonie, Gilles / Baleine, Julien / Sola, Chrystelle / Delye, Bénédicte / Dimopoulou, Evgenia / Sanchez, Stéphanie / Gasnier, Mathieu / Touati, Souad /
    Zamora, Alberto / Pontal, Daniel / Leboucq, Nicolas / Kouyoumdjian, Virginie / Lebasnier, Adrien / Sanquer, Sylvia / Mariano-Goulart, Denis / Roujeau, Thomas / Roubertie, Agathe

    Movement disorders clinical practice

    2023  Volume 10, Issue 5, Page(s) 811–818

    Abstract: Background: Aromatic l-amino acid decarboxylase deficiency (AADCD) is a rare, early-onset, dyskinetic encephalopathy mostly reflecting a defective synthesis of brain dopamine and serotonin. Intracerebral gene delivery (GD) provided a significant ... ...

    Abstract Background: Aromatic l-amino acid decarboxylase deficiency (AADCD) is a rare, early-onset, dyskinetic encephalopathy mostly reflecting a defective synthesis of brain dopamine and serotonin. Intracerebral gene delivery (GD) provided a significant improvement among AADCD patients (mean age, ≤6 years).
    Objective: We describe the clinical, biological, and imaging evolution of two AADCD patients ages >10 years after GD.
    Methods: Eladocagene exuparvovec, a recombinant adeno-associated virus containing the human complimentary DNA encoding the AADC enzyme, was administered into bilateral putamen by stereotactic surgery.
    Results: Eighteen months after GD, patients showed improvement in motor, cognitive and behavioral function, and in quality of life. Cerebral l-6-[
    Conclusion: Two patients with a severe form of AADCD had an objective motor and non-motor benefit from eladocagene exuparvovec injection even when treated after the age of 10 years, as in the seminal study.
    Language English
    Publishing date 2023-02-24
    Publishing country United States
    Document type Journal Article
    ISSN 2330-1619
    ISSN (online) 2330-1619
    DOI 10.1002/mdc3.13685
    Database MEDical Literature Analysis and Retrieval System OnLINE

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