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000285470 1001_ $$00009-0001-4221-7131$$aYogeshwar, Selina M$$b0
000285470 245__ $$aBrain atrophy patterns in anti-IgLON5 disease.
000285470 260__ $$aOxford$$bOxford Univ. Press$$c2026
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000285470 520__ $$aAnti-IgLON5 disease is an autoimmune encephalitis that presents with a heterogenous clinical phenotype, including sleep disorders, movement abnormalities and bulbar involvement. It is characterized by autoantibodies against IgLON5, 85% association with HLA-DQB1*05:∼ and a brainstem-dominant tauopathy. Cellular and murine models report pathogenic effects of the autoantibodies, and neurodegenerative factors suggest progressive atrophy as a common sequela. However, evidence from in vivo patient data and long-term follow-up is limited, and the degree of progression remains elusive. In this multicentre study, clinical and brain MRI data were collected from 127 patients across 12 countries to investigate the relationships between clinical presentations and the development of distinct brain atrophy patterns. Our data show that most patients develop a complex multisystem phenotype as the disease progresses; however, neuromuscular manifestations rarely emerge at later disease stages. By comparison to healthy controls, this disease presents with severe substructure-specific atrophy, especially affecting the hypothalamus, brainstem, accumbens and basal ganglia, which, in age-independent analyses, show significant ventricular enlargement and also suggest progression of brainstem atrophy over the disease course. Moreover, the focality of atrophy was functionally linked to specific symptoms, with more severe involvement of the basal ganglia in patients with movement disorders, and greater atrophy in the hippocampus and thalamus in patients with cognitive impairment. Taken together, our results provide evidence of distinct atrophy patterns in anti-IgLON5 disease, which closely mirror sites of pathophysiologic processes, including autoantibody binding and tau deposition. Our data emphasize the brainstem as the pathophysiological hub of the disease and provide normative data for the incorporation of atrophy measurements into routine clinical assessments and future treatment studies to monitor disease trajectory and evaluate future treatment strategies.
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000285470 650_7 $$2Other$$aIgLON5
000285470 650_7 $$2Other$$aMRI
000285470 650_7 $$2Other$$aatrophy
000285470 650_7 $$2Other$$aautoimmune encephalitis
000285470 650_7 $$2NLM Chemicals$$aIgLON5 protein, human
000285470 650_7 $$2NLM Chemicals$$aAutoantibodies
000285470 650_7 $$2NLM Chemicals$$aCell Adhesion Molecules, Neuronal
000285470 650_2 $$2MeSH$$aHumans
000285470 650_2 $$2MeSH$$aMale
000285470 650_2 $$2MeSH$$aAtrophy: pathology
000285470 650_2 $$2MeSH$$aFemale
000285470 650_2 $$2MeSH$$aMiddle Aged
000285470 650_2 $$2MeSH$$aBrain: pathology
000285470 650_2 $$2MeSH$$aBrain: diagnostic imaging
000285470 650_2 $$2MeSH$$aAged
000285470 650_2 $$2MeSH$$aMagnetic Resonance Imaging
000285470 650_2 $$2MeSH$$aAdult
000285470 650_2 $$2MeSH$$aAutoantibodies: immunology
000285470 650_2 $$2MeSH$$aCell Adhesion Molecules, Neuronal: immunology
000285470 650_2 $$2MeSH$$aDisease Progression
000285470 650_2 $$2MeSH$$aAutoimmune Diseases of the Nervous System: pathology
000285470 650_2 $$2MeSH$$aAutoimmune Diseases of the Nervous System: diagnostic imaging
000285470 650_2 $$2MeSH$$aAutoimmune Diseases of the Nervous System: immunology
000285470 650_2 $$2MeSH$$aEncephalitis: pathology
000285470 650_2 $$2MeSH$$aEncephalitis: immunology
000285470 650_2 $$2MeSH$$aEncephalitis: diagnostic imaging
000285470 7001_ $$00000-0003-3523-4520$$aBartels, Frederik$$b1
000285470 7001_ $$00000-0001-8927-9818$$aGrüter, Thomas$$b2
000285470 7001_ $$00000-0001-5958-3288$$aMuñiz-Castrillo, Sergio$$b3
000285470 7001_ $$aPicard, Géraldine$$b4
000285470 7001_ $$aCrijnen, Yvette S$$b5
000285470 7001_ $$aBernard, Emilien$$b6
000285470 7001_ $$aHeidbreder, Anna$$b7
000285470 7001_ $$aZekeridou, Anastasia$$b8
000285470 7001_ $$aRingelstein, Marius$$b9
000285470 7001_ $$aKraft, Andrea$$b10
000285470 7001_ $$aKovac, Stjepana$$b11
000285470 7001_ $$aWandinger, Klaus-Peter$$b12
000285470 7001_ $$ade Vries, Juna M$$b13
000285470 7001_ $$aBoon, Agnita J W$$b14
000285470 7001_ $$aVeenbergen, Sharon$$b15
000285470 7001_ $$aGeis, Christian$$b16
000285470 7001_ $$aPenner, Loana$$b17
000285470 7001_ $$00000-0002-2420-701X$$aMelzer, Nico$$b18
000285470 7001_ $$00000-0002-8972-515X$$aLeypoldt, Frank$$b19
000285470 7001_ $$aBlaabjerg, Morten$$b20
000285470 7001_ $$00000-0002-6140-5584$$aPittock, Sean J$$b21
000285470 7001_ $$aGaig, Carles$$b22
000285470 7001_ $$aSabater, Lidia$$b23
000285470 7001_ $$aSantamaria, Joan$$b24
000285470 7001_ $$aGraus, Francesc$$b25
000285470 7001_ $$aDalmau, Josep$$b26
000285470 7001_ $$0P:(DE-2719)2810931$$aPrüss, Harald$$b27$$udzne
000285470 7001_ $$00000-0002-5769-1100$$aHöftberger, Romana$$b28
000285470 7001_ $$aSchreiner, Bettina$$b29
000285470 7001_ $$00000-0002-3889-8739$$aMcKeon, Andrew$$b30
000285470 7001_ $$aLewerenz, Jan$$b31
000285470 7001_ $$00000-0002-7667-9748$$aIrani, Sarosh$$b32
000285470 7001_ $$00000-0002-6928-5310$$aMignot, Emmanuel$$b33
000285470 7001_ $$aTitulaer, Maarten J$$b34
000285470 7001_ $$aAyzenberg, Ilya$$b35
000285470 7001_ $$00000-0002-4721-5952$$aHonnorat, Jérôme$$b36
000285470 7001_ $$0P:(DE-2719)9001141$$aFinke, Carsten$$b37
000285470 7001_ $$aConsortium, IgLON5 Imaging$$b38$$eCollaboration Author
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