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  1. Article ; Online: The ClC-1 chloride channel inhibitor NMD670 improves skeletal muscle function in rat models and patients with myasthenia gravis.

    Skov, Martin / Ruijs, Titia Q / Grønnebæk, Thomas S / Skals, Marianne / Riisager, Anders / Winther, Jeppe Blichfeldt / Dybdahl, Kamilla Løhde Tordrup / Findsen, Anders / Morgen, Jeanette J / Huus, Nete / Broch-Lips, Martin / Nielsen, Ole B / de Cuba, Catherine M K E / Heuberger, Jules A A C / de Kam, Marieke L / Tannemaat, Martijn / Verschuuren, Jan J G M / Knutsen, Lars J S / Kelly, Nicholas M /
    Jensen, Klaus G / Arnold, William D / Burghes, Arthur H / Olesen, Claus / Bold, Jane / Petersen, Thomas K / Quiroz, Jorge A / Hutchison, John / Chin, Eva R / Groeneveld, Geert J / Pedersen, Thomas H

    Science translational medicine

    2024  Volume 16, Issue 739, Page(s) eadk9109

    Abstract: Myasthenia gravis (MG) is a neuromuscular disease that results in compromised transmission of electrical signals at the neuromuscular junction (NMJ) from motor neurons to skeletal muscle fibers. As a result, patients with MG have reduced skeletal muscle ... ...

    Abstract Myasthenia gravis (MG) is a neuromuscular disease that results in compromised transmission of electrical signals at the neuromuscular junction (NMJ) from motor neurons to skeletal muscle fibers. As a result, patients with MG have reduced skeletal muscle function and present with symptoms of severe muscle weakness and fatigue. ClC-1 is a skeletal muscle specific chloride (Cl
    MeSH term(s) Humans ; Rats ; Animals ; Chlorides/therapeutic use ; Myasthenia Gravis/drug therapy ; Muscle, Skeletal/physiology ; Neuromuscular Junction ; Chloride Channels
    Chemical Substances Chlorides ; Chloride Channels
    Language English
    Publishing date 2024-03-20
    Publishing country United States
    Document type Randomized Controlled Trial ; Clinical Trial, Phase I ; Journal Article
    ZDB-ID 2518854-9
    ISSN 1946-6242 ; 1946-6234
    ISSN (online) 1946-6242
    ISSN 1946-6234
    DOI 10.1126/scitranslmed.adk9109
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Idiopathic delayed-onset edema surrounding deep brain stimulation leads: Insights from a case series and systematic literature review.

    de Cuba, Catherine M K E / Albanese, Alberto / Antonini, Angelo / Cossu, Giovanni / Deuschl, Günther / Eleopra, Roberto / Galati, Alejandro / Hoffmann, Carel F E / Knudsen, Karina / Landi, Andrea / Lanotte, Michele Maria R / Marcante, Andrea / Mosch, Arne / Pilleri, Manuela / Reich, Martin M / Ricchi, Valeria / Rinaldo, Sara / Romito, Luigi M / Saba, Felipe S /
    Sacristan, Horacio E / Schuurman, P Richard / Trezza, Andrea / van den Munckhof, Pepijn / Volkmann, Jens / Zibetti, Maurizio / Contarino, Maria Fiorella

    Parkinsonism & related disorders

    2016  Volume 32, Page(s) 108–115

    Abstract: Introduction: Deep brain stimulation (DBS) is effective for some neurological and psychiatric conditions. Idiopathic delayed-onset edema (IDE) surrounding the leads has been anecdotally reported. The etiology, predisposing factors and prognosis of this ... ...

    Abstract Introduction: Deep brain stimulation (DBS) is effective for some neurological and psychiatric conditions. Idiopathic delayed-onset edema (IDE) surrounding the leads has been anecdotally reported. The etiology, predisposing factors and prognosis of this complication are unknown. We present a multicenter case series of patients with IDE, and a systematic literature review, aimed at defining the pathophysiology and identifying appropriate treatment strategies.
    Methods: IDE was defined as edema along the DBS lead, occurring ≥72 h postoperatively, in absence of trauma, vascular events or infection. Information on patients with IDE was collected in a standardized way. A systematic search was performed in Pubmed.
    Results: Twelve new patients presenting with 14 episodes of IDE are described. From the literature, 38 patients were identified. No common surgical aspects or patient-related factors were identified as risk predictors for the onset of IDE. Symptoms included deterioration of the stimulation effect, seizures and focal neurological signs. Although the condition is self-limiting, with symptoms resolution in 28.5 days on average, three patients underwent surgical revision and seven received antibiotics.
    Conclusions: IDE is a rare complication of DBS procedures, presenting from few days to months after surgery. Symptoms can be mild and not-specific, and the condition is self-limiting. The diagnosis of IDE is made after exclusion of vascular events or infections. The pathophysiology is still unexplained. The recognition of this complication can help avoiding unnecessary surgical procedures (system explantation) and antibiotic treatment.
    Language English
    Publishing date 2016-11
    Publishing country England
    Document type Journal Article
    ZDB-ID 1311489-x
    ISSN 1873-5126 ; 1353-8020
    ISSN (online) 1873-5126
    ISSN 1353-8020
    DOI 10.1016/j.parkreldis.2016.09.007
    Database MEDical Literature Analysis and Retrieval System OnLINE

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