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  1. Article ; Online: GAD65 Autoimmune Encephalitis: A Cause of Rapidly Evolving Frontotemporal Atrophy.

    Vanhoorne, Alexander / Van Langenhove, Tim / Miatton, Marijke / Laureys, Guy

    Alzheimer disease and associated disorders

    2021  Volume 36, Issue 1, Page(s) 80–82

    Abstract: We describe a patient who presented with subacute onset of short-memory impairment, disorientation, and gait instability, with progressive deterioration. Workup demonstrated glutamic acid decarboxylase antibody-related encephalitis. Aggressive ... ...

    Abstract We describe a patient who presented with subacute onset of short-memory impairment, disorientation, and gait instability, with progressive deterioration. Workup demonstrated glutamic acid decarboxylase antibody-related encephalitis. Aggressive immunotherapy with high-dose intravenous corticoids, followed by slow oral taper, plasmapheresis, rituximab, and cyclophosphamide did not halt disease progression. During follow-up, she developed a frontotemporal dementia phenotype. Serial imaging showed the appearance of marked atrophy of the frontal and anterior temporal regions. We conclude that glutamic acid decarboxylase antibody-related encephalitis may rarely present with a treatment-refractory frontotemporal phenotype.
    MeSH term(s) Atrophy ; Autoantibodies ; Encephalitis ; Female ; Glutamate Decarboxylase/therapeutic use ; Hashimoto Disease ; Humans
    Chemical Substances Autoantibodies ; Glutamate Decarboxylase (EC 4.1.1.15)
    Language English
    Publishing date 2021-06-16
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1002700-2
    ISSN 1546-4156 ; 0893-0341
    ISSN (online) 1546-4156
    ISSN 0893-0341
    DOI 10.1097/WAD.0000000000000463
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: R-tPA Resistance Is Specific for Platelet-Rich Stroke Thrombi and Can Be Overcome by Targeting Nonfibrin Components.

    Vandelanotte, Sarah / François, Olivier / Desender, Linda / Staessens, Senna / Vanhoorne, Alexander / Van Gool, Fréderick / Tersteeg, Claudia / Vanhoorelbeke, Karen / Vanacker, Peter / Andersson, Tommy / De Meyer, Simon F

    Stroke

    2024  Volume 55, Issue 5, Page(s) 1181–1190

    Abstract: Background: Resistance to r-tPA (recombinant tissue-type plasminogen activator) is a well-known but poorly understood phenomenon that hampers successful recanalization in patients with acute ischemic stroke. Using clinically relevant thrombi from ... ...

    Abstract Background: Resistance to r-tPA (recombinant tissue-type plasminogen activator) is a well-known but poorly understood phenomenon that hampers successful recanalization in patients with acute ischemic stroke. Using clinically relevant thrombi from patients with acute ischemic stroke, we investigated if and how thrombus composition impacts r-tPA-mediated lysis. In addition, we explored strategies to overcome r-tPA resistance.
    Methods: Thrombi were split into 2 parts, 1 of which was used for thrombolysis and the other for detailed histological analysis. Thrombolysis was performed in normal human plasma using r-tPA alone, using r-tPA in combination with DNase-1 or using r-tPA in combination with N,N'-diacetyl-l-cystine. Thrombus lysis was calculated as the percentage of residual thrombus weight compared with its initial weight and the degree of lysis was linked to thrombus composition determined via histology.
    Results: Interestingly, we found that the efficacy of r-tPA-mediated thrombolysis was strongly correlated with the composition of the thrombi. Thrombi containing high amounts of red blood cells and low amounts of DNA and von Willebrand Factor were efficiently degraded by r-tPA, whereas thrombi containing low amounts of red blood cells and higher amounts of DNA and von Willebrand Factor were resistant to r-tPA. Importantly, combination of r-tPA with DNase-1 or N,N'-diacetyl-l-cystine significantly and specifically improved the lysis of these r-tPA-resistant thrombi.
    Conclusions: Using patient thrombus material, our results for the first time show that the composition of stroke thrombi largely determines their susceptibility to r-tPA-mediated thrombolysis. Red blood cell-poor thrombi have a specific resistance to r-tPA, which can be overcome by targeting nonfibrin components using DNase-1 or N,N'-diacetyl-l-cystine.
    Language English
    Publishing date 2024-03-25
    Publishing country United States
    Document type Journal Article
    ZDB-ID 80381-9
    ISSN 1524-4628 ; 0039-2499 ; 0749-7954
    ISSN (online) 1524-4628
    ISSN 0039-2499 ; 0749-7954
    DOI 10.1161/STROKEAHA.123.045880
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort.

    Dekeyser, Cathérine / Vanhoorne, Alexander / Hemelsoet, Dimitri / Van Hijfte, Liesbeth / De Zaeytijd, Julie / Van Driessche, Veroniek / Van Hoecke, Helen / Miatton, Marijke / Van Vrekhem, Tineke / Maes, Leen / Laureys, Guy

    Journal of neuroimmunology

    2023  Volume 376, Page(s) 578032

    Abstract: Susac syndrome (SuS) is a rare immune-mediated endotheliopathy that affects the brain, retina and inner ear and is characterised by the variable clinical triad of encephalopathy, visual and vestibulocochlear dysfunction. Here, we present clinical and ... ...

    Abstract Susac syndrome (SuS) is a rare immune-mediated endotheliopathy that affects the brain, retina and inner ear and is characterised by the variable clinical triad of encephalopathy, visual and vestibulocochlear dysfunction. Here, we present clinical and paraclinical data of 19 SuS patients followed at Ghent University Hospital and highlight some atypical clinical and novel radiological findings. Our findings suggest that spinal involvement expands the clinical phenotype of SuS. We further introduce dark blood sequences as a more sensitive technique to detect radiological disease activity in SuS. Our data add to the current understanding of the diagnosis, monitoring and treatment of SuS.
    MeSH term(s) Humans ; Susac Syndrome/diagnosis ; Magnetic Resonance Imaging ; Brain ; Brain Diseases ; Retina
    Language English
    Publishing date 2023-01-21
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 8335-5
    ISSN 1872-8421 ; 0165-5728
    ISSN (online) 1872-8421
    ISSN 0165-5728
    DOI 10.1016/j.jneuroim.2023.578032
    Database MEDical Literature Analysis and Retrieval System OnLINE

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